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A stress ulcer is a single or multiple mucosal defect which can become complicated by upper gastrointestinal bleeding or physiologic stress. Ordinary peptic ulcers are found commonly in the gastric antrum and the duodenum whereas stress ulcers are found commonly in fundic mucosa and can be located anywhere within the stomach and proximal duodenum. Signs and symptoms
Stress ulcers, as defined by overt bleeding and hemodynamic instability, decreased hemoglobin, and/or need for transfusion, were seen in 1.5% of patients in the 2252 patients in the Canadian Critical Care Trials group study. People with stress ulcers have a longer ICU length of stay (up to 8 days) and a higher mortality (up to 4 fold) than patients who do not have stress ulceration and bleeding. While the bleeding and transfusions associated with the stress ulcerations contribute to the increased mortality, the contribution of factors like low blood pressure, sepsis, and respiratory failure to the mortality independently of the stress ulceration cannot be ignored. Risk factors
Risk factors for stress ulcer formation that have been identified are numerous and varied. However, two landmark studies and one position paper exist that addresses the topic of risk factors for stress ulcer formation:
Non-critically ill medical patients with 2 or more of the following: respiratory failure, sepsis, heart failure, hepatic encephalopathy, jaundice, kidney failure, stroke, hypertension, previous gastrointestinal disease and treatment with corticosteroids, NSAIDS, heparin, or warfarin. In surgical critically ill patients, only those patients who are on a mechanical ventilator for more than 48 hours and/or those with a coagulopathy. | History
Early development
The 2014 Ebola outbreak killed more than 11,300 people. Regeneron used its VelociGene, VelocImmune and VelociMab antibody discovery and production technologies and coordinated with the U.S. governments Biomedical Advanced Research and Development Authority (BARDA). The therapy was developed in six months and a Phase 1 trial in healthy humans was completed in 2015. PALM trial
During the 2018 Équateur province Ebola outbreak, a similar monoclonal antibody treatment, mAb114, was requested by the Democratic Republic of Congo (DRC) Ministry of Public Health. mAb114 was approved for compassionate use by the World Health Organization MEURI ethical protocol and at DRC ethics board. mAb114 was sent along with other therapeutic agents to the outbreak sites. However, the outbreak came to a conclusion before any therapeutic agents were given to patients.Approximately one month following the conclusion of the Équateur province outbreak, a distinct outbreak was noted in Kivu in the DRC (2018–20 Kivu Ebola outbreak). Once again, mAb114 received approval for compassionate use by WHO MEURI and DRC ethic boards and has been given to many patients under these protocols. In November 2018, the Pamoja Tulinde Maisha (PALM [together save lives]) open-label randomized clinical control trial was begun at multiple treatment units testing mAb114, REGN-EB3 and remdesivir to ZMapp. Despite the difficulty of running a clinical trial in a conflict zone, investigators have enrolled 681 patients towards their goal of 725. | 0-1
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Differential
It may not be possible to tell the difference between beta blocker toxicity and calcium channel blocker overdose based on signs and symptoms. Management
The medical management of CCB toxicity may be difficult. It may not improve with the usual treatments used for a low blood pressure and a slow heart rate. In those who have no symptoms or signs six hours following taking an immediate release formulation and 24 hours after taking an extended release formulation need no further medical treatment. Detoxification
Activated charcoal is recommended if it can be given within an hour or two of taking the calcium channel blockers. In those who have taken an extended release formulation of a CCB but are otherwise doing fine, whole bowel irrigation with polyethylene glycol may be useful. Causing vomiting by the use of medications such as ipecac is not recommended. Insulin
High doses of intravenous insulin with glucose may be useful and are a first line treatment in overdoses. As this treatment may cause a drop in blood sugar and blood potassium levels, these should be monitored closely. Other
Intravenous calcium gluconate or calcium chloride is considered a specific antidotes. Slow heart rate can be treated with atropine and sympathomimetics. Low blood pressure is treated with vasopressors such as adrenaline.There is tentative clinical evidence and good theoretical evidence of the benefit of lipid emulsion in severe overdoses of CCBs. Methylene blue may also be used for those with low blood pressure that does not respond to other treatments. | Similarly, selective serotonin reuptake inhibitors can cause mydriasis. Dissociatives such as dextromethorphan (an SSRI and sigma-1 agonist). Certain GABAergic drugs, such as phenibut and GHB. Adrenergic agonists, such as phenylephrine and cyclomydril. Adrenergic agonists may be used if strong mydriasis is needed in surgery. Norepinephrine is a hormone and neurotransmitter that regulates the involuntary muscles of the autonomic nervous system, including dilation of the pupil aperture via the muscles of the iris. Hence adrenergic agonists mimic the activity of norepinephrine, which is how they induce mydriasis.Natural release of the hormone oxytocin can cause mild to moderate mydriasis.Long term effects of drugs can also cause mydriasis, for example opioid withdrawal. Autonomic neuropathy
Parasympathetic fibers travel with cranial nerve III, the oculomotor nerve, to innervate the circular layer of muscle of the eye (sphincter pupillae). Damage to this nerve typically manifests itself as mydriasis, because the sympathetic supply to the pupil, which causes mydriasis, remains unaffected, and therefore unopposed. Multiple central nervous system disorders e.g. epilepsy, stroke, and impending brain herniation are known to lead to temporal mydriasis as well. A brain catastrophe, or a rapidly increasing brain mass, can cause compression of the oculomotor nerve. Trauma
In cases of head injury or orbit trauma (eye injury), the iris sphincter (the muscle responsible for closing the pupil) or the nerves controlling it can be damaged, reducing or eliminating the normal pupillary light reflex. References
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This kind of insecurity leads to overcompensation: compulsive formality, strict social observances and exaggerated displays of assurance.In 1950, Kurt Schneider described the "fanatic psychopaths" and divided them into two categories: the combative type that is very insistent about his false notions and actively quarrelsome, and the eccentric type that is passive, secretive, vulnerable to esoteric sects but nonetheless suspicious about others.The descriptions of Leonhard and Sheperd from the sixties describe paranoid people as overvaluing their abilities and attributing their failure to the ill-will of others; they also mention that their interpersonal relations are disturbed and they are in constant conflict with others.In 1975, Polatin described the paranoid personality as rigid, suspicious, watchful, self-centered and selfish, inwardly hypersensitive but emotionally undemonstrative. However, when there is a difference of opinion, the underlying mistrust, authoritarianism and rage burst through.In the 1980s, paranoid personality disorder received little attention, and when it did receive it, the focus was on its
potential relationship to paranoid schizophrenia. The most significant contribution of this decade comes from Theodore Millon who divided the features of paranoid personality disorder to four categories:1) behavioral characteristics of vigilance, abrasive irritability and counterattack,
2) complaints indicating oversensitivity, social isolation and mistrust,
3) the dynamics of denying personal insecurities, attributing these to others and self-inflation through grandiose fantasies
4) coping style of detesting dependence and hostile distancing of oneself from others. Controversy
Due to repeated concerns of the validity of PPD and poor empirical evidence, it has been suggested that PPD be removed from the DSM. | Patients with paraneoplastic SPS generally lack other autoimmune issues but may have other paraneoplastic conditions.Stiff-limb syndrome is a variant of SPS. This syndrome develops into full SPS about 25% of the time. Stiffness and spasms are usually limited to the legs and hyperlordoisis generally does not occur. The stiffness begins in one limb and remains most prominent there. Sphincter and brainstem issues often occur with stiff-limb syndrome. Progressive encephalomyelitis with rigidity, another variant of the condition, includes symptoms of SPS with brainstem issues and autonomic disturbances. It involves polio-encephalomyelitis in the spine and brainstem. There is cerebellar and brainstem involvement. In some cases, the limbic system is affected, as well. Most patients have upper motoneuron issues and autonomic disturbances. Jerking SPS is another subtype of the condition. It begins like classical SPS and progresses for several years; up to 14 in some cases. It is then distinguished by the development of myoclonus as well as seizures and ataxia in some cases. Causes
Patients with SPS generally have high amounts of high glutamic acid decarboxylase antibody titers. About 80 percent of SPS patients have GAD antibodies, compared with about one percent of the general population. The overwhelming majority of people who have GAD antibodies do not contract SPS, indicating that systematic synthesis of the antibody is not the sole cause of SPS. GAD, a presynaptic autoantigen, is generally thought to play a key role in the condition, but exact details of the way that autoantibodies affect SPS patients are not known. | 0-1
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Physical growth slows generally around the age of 18 months, and stops completely by the age of 8. Skeletal abnormalities include a bell-shaped chest, a flattening or curvature of the spine, shortened long bones, and dysplasia of the hips, knees, ankles, and wrists. The bones that stabilize the connection between the head and neck can be malformed (odontoid hypoplasia); in these cases, a surgical procedure called spinal cervical bone fusion can be lifesaving. Restricted breathing, joint stiffness, and heart disease are also common. Children with the more, severe form of Morquio syndrome may not live beyond their twenties or thirties. MPS VI
Children with MPS VI, Maroteaux–Lamy syndrome, usually have normal intellectual development but share many of the physical symptoms found in Hurler syndrome. Caused by the deficient enzyme N-acetylgalactosamine 4-sulfatase, Maroteaux-Lamy syndrome has a variable spectrum of severe symptoms. Neurological complications include clouded corneas, deafness, thickening of the dura (the membrane that surrounds and protects the brain and spinal cord), and pain caused by compressed or traumatized nerves and nerve roots. Growth is normal at first but stops suddenly around age 8. By age 10 children have developed a shortened trunk, crouched stance, and restricted joint movement. In more severe cases, children also develop a protruding abdomen and forward-curving spine. Skeletal changes (particularly in the pelvic region) are progressive and limit movement. Many children also have umbilical or inguinal hernias. Nearly all children have some form of heart disease. | Diagnosis
Diagnosis often can be made through clinical examination and urine tests (excess mucopolysaccharides are excreted in the urine). Enzyme assays (testing a variety of cells or body fluids in culture for enzyme deficiency) are also used to provide definitive diagnosis of one of the mucopolysaccharidoses. Prenatal diagnosis using amniocentesis and chorionic villus sampling can verify if a fetus either carries a copy of the defective gene or is affected with the disorder. Genetic counseling can help parents who have a family history of the mucopolysaccharidoses determine if they are carrying the mutated gene that causes the disorders. Types
Seven distinct clinical types and numerous subtypes of the mucopolysaccharidoses have been identified. Although each mucopolysaccharidosis (MPS) differs clinically, most patients generally experience a period of normal development followed by a decline in physical and/or mental function. (Note: MPS-V and MPS-VIII are no longer in use as designations for any disease.) Overview table
MPS I
MPS I is divided into three subtypes based on severity of symptoms. All three types result from an absence of, or insufficient levels of, the enzyme alpha-L-iduronidase. Children born to an MPS I parent carry the defective gene. MPS I H (also called Hurler syndrome or α-L-iduronidase deficiency), is the most severe of the MPS I subtypes. Developmental delay is evident by the end of the first year, and patients usually stop developing between ages 2 and 4. This is followed by progressive mental decline and loss of physical skills. Language may be limited due to hearing loss and an enlarged tongue. | 11
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His hallucinations consisted of perceptions of men, women, birds, carriages, buildings, tapestries, physically impossible circumstances and scaffolding patterns. Even though his health was in good shape and he had an absence of any psychiatric disorders, the source of the hallucinations remained unknown. At forty years old, Charles Bonnet himself suffered from an unrevealed cause of severe vision loss and experienced the hallucinations.In 1936, Jean Lhermitte and Julian de Ajuriaguerra, concluded that visual hallucinations consist of thalamic lesions as well as ocular pathology.In 1967, French-Swiss neurologist, Georges de Morsier, coined the term Charles Bonnet Syndrome in Bonnets honor. De Morsier’s description of CBS implies a concentrated neurodegeneration, usually occurring in the elderly with typical cognition. In psychiatric literature, the most commonly accepted interpretation of CBS is that of Gold and Rabins’. In 1989, they detailed that the hallucinations associated with CBS are not affecting other sensory modalities. They believed that the visual hallucinations are oftentimes stereotyped, persistent, and/or repetitive in nature. Society and culture
The syndrome is discussed in:
Vilayanur S. Ramachandrans book Phantoms in the Brain. Ramachandran suggests that James Thurber, who was blinded in one eye as a child, may have derived his extraordinary imagination from the syndrome. Vikram Chandras book Sacred Games (2006)
David Eaglemans book Incognito: The Secret Lives of the Brain
Oliver Sacks 2012 book Hallucinations
The Indian movie Jawan of Vellimala, released in 2012, in which Mammootty is a victim of the syndrome
The Black Canvas (2014), a chamber opera by the Greek composer Spyros Syrmos, is about a celebrated painter whose visions are caused by CBS. | Visual release hallucinations, also known as Charles Bonnet syndrome or CBS, are a type of psychophysical visual disturbance in which a person with partial or severe blindness experiences visual hallucinations. First described by Charles Bonnet in 1760, the term Charles Bonnet syndrome was first introduced into English-speaking psychiatry in 1982. A related type of hallucination that also occurs with lack of visual input is the closed-eye hallucination. Signs and symptoms
People with significant vision loss may have vivid recurrent visual hallucinations (fictive visual percepts). One characteristic of these hallucinations is that they usually are "lilliputian" (hallucinations in which the characters or objects are smaller than normal). Depending on the content, visual hallucinations can be classified as either simple or complex. Simple visual hallucinations are commonly characterized by shapes, photopsias, and grid-like patterns. Complex visual hallucinations consist of highly detailed representations of people and objects. The most common hallucination is of faces or cartoons. Sufferers understand that the hallucinations are not real, and the hallucinations are only visual, that is, they do not occur in any other senses (such as hearing, smell or taste). Visual hallucinations generally appear when the eyes are open, fading once the visual gaze shifts. It is widely claimed that sensory deprivation is instrumental in the progression of CBS. During episodes of inactivity, hallucinations are more likely to occur. | 11
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Thus, hypothermia risk factors include: substance use disorders (including alcohol use disorder), homelessness, any condition that affects judgment (such as hypoglycemia), the extremes of age, poor clothing, chronic medical conditions (such as hypothyroidism and sepsis), and living in a cold environment. Hypothermia occurs frequently in major trauma, and is also observed in severe cases of anorexia nervosa. Hypothermia is also associated with worse outcomes in people with sepsis. While most people with sepsis develop fevers (elevated body temperature), some develop hypothermia.In urban areas, hypothermia frequently occurs with chronic cold exposure, such as in cases of homelessness, as well as with immersion accidents involving drugs, alcohol or mental illness. While studies have shown that people experiencing homelessness are at risk of premature death from hypothermia, the true incidence of hypothermia-related deaths in this population is difficult to determine. In more rural environments, the incidence of hypothermia is higher among people with significant comorbidities and less able to move independently. With rising interest in wilderness exploration, and outdoor and water sports, the incidence of hypothermia secondary to accidental exposure may become more frequent in the general population. Alcohol
Alcohol consumption increases the risk of hypothermia in two ways: vasodilation and temperature controlling systems in the brain. Vasodilation increases blood flow to the skin, resulting in heat being lost to the environment. This produces the effect of feeling warm, when one is actually losing heat. | The product was produced as an alternative for people averse to the chalky consistency of regular Rolaids. They were widely advertised in multiple media formats. Rolaids Softchews were originally developed and test marketed in Oklahoma City under the brand name Remegel by Warner Lambert in 1984. Medical information
The active ingredients are calcium carbonate (550 mg) and magnesium hydroxide (110 mg). The inactive ingredients are dextrose, flavoring, magnesium stearate, polyethylene glycol, pregelatinized starch, sucralose and sucrose. The new Chattem varieties have increased the amount of the active ingredients in the product, up to 1000 mg of calcium carbonate and 200 mg of magnesium hydroxide for the "ultra strength" varieties. Minor side effects may include constipation or stomach cramps. Serious side effects include loss of appetite, vomiting, dizziness, and headache. References
External links
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Methotrexate (MTX), formerly known as amethopterin, is a chemotherapy agent and immune-system suppressant. It is used to treat cancer, autoimmune diseases, and ectopic pregnancies. Types of cancers it is used for include breast cancer, leukemia, lung cancer, lymphoma, gestational trophoblastic disease, and osteosarcoma. Types of autoimmune diseases it is used for include psoriasis, rheumatoid arthritis, and Crohns disease. It can be given by mouth or by injection.Common side effects include nausea, feeling tired, fever, increased risk of infection, low white blood cell counts, and breakdown of the skin inside the mouth. Other side effects may include liver disease, lung disease, lymphoma, and severe skin rashes. People on long-term treatment should be regularly checked for side effects. It is not safe during breastfeeding. In those with kidney problems, lower doses may be needed. It acts by blocking the bodys use of folic acid.Methotrexate was first made in 1947 and initially was used to treat cancer, as it was less toxic than the then current treatments. In 1956 it provided the first cures of a metastatic cancer. It is on the World Health Organizations List of Essential Medicines. Methotrexate is available as a generic medication. In 2019, it was the 111th most commonly prescribed medication in the United States, with more than 5 million prescriptions. Medical uses
Chemotherapy
Methotrexate was originally developed and continues to be used for chemotherapy, either alone or in combination with other agents. | Since scapular fractures impair the motion of the shoulder, a person with a scapular fracture has a reduced ability to move the shoulder joint. Signs and symptoms may be masked by other injuries that accompany the scapular fracture. Causes
Usually, it takes a large amount of energy to fracture the scapula; the force may be indirect but is more often direct. The scapula is fractured as the result of significant blunt trauma, as occurs in vehicle collisions. About three quarters of cases are caused by high-speed car and motorcycle collisions. Falls and blows to the shoulder area can also cause the injury. Crushing injuries (as may occur in railroad or forestry accidents) and sports injuries (as may occur in horseback riding, mountain biking, bmxing or skiing) can also fracture the scapula. Scapular fracture can result from electrical shocks and from seizures: muscles pulling in different directions contract powerfully at the same time. In cardiopulmonary resuscitation, the chest is compressed significantly; scapular fracture may occur as a complication of this technique. Anatomy
The scapula has a body, neck, and spine; any of these may be fractured. The most commonly injured areas are the scapular body, spine, neck, and glenoid rim; the scapular body or neck is injured in about 80% of cases. Fractures that occur in the body may be vertical, horizontal, or comminuted (involving multiple fragments). Those that occur in the neck are usually parallel to the glenoid fossa. | 0-1
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It is also characterized by fragile blood vessels and organs that can easily rupture. Affected people are frequently short, and have thin scalp hair. It also has characteristic facial features, including large eyes, an undersized chin, sunken cheeks, a thin nose and lips, and ears without lobes. Joint hypermobility is present, but generally confined to the small joints (fingers, toes). Other common features include club foot, tendon and/or muscle rupture, acrogeria (premature aging of the skin of the hands and feet), early-onset varicose veins, pneumothorax (collapse of a lung), the recession of the gums, and a decreased amount of fat under the skin. It can be caused by the variations in the COL3A1 gene. Rarely, COL1A1 variations can also cause it. Kyphoscoliosis EDS
Kyphoscoliosis EDS (formerly categorized as type 6) is associated with severe hypotonia at birth, delayed motor development, progressive scoliosis (present from birth), and scleral fragility. People may also have easy bruising, fragile arteries that are prone to rupture, unusually small corneas, and osteopenia (low bone density). Other common features include a "marfanoid habitus" characterized by long, slender fingers (arachnodactyly), unusually long limbs, and a sunken chest (pectus excavatum) or protruding chest (pectus carinatum). It can be caused by variations in the gene PLOD1, or rarely, in the FKBP14 gene. Arthrochalasia EDS
Arthrochalasia EDS (formerly categorized as types 7A and B) is characterized by severe joint hypermobility and congenital hip dislocation. Other common features include fragile, elastic skin with easy bruising, hypotonia, kyphoscoliosis (kyphosis and scoliosis), and mild osteopenia. Type-I collagen is usually affected. | Dilation and/or rupture (aneurysm) of ascending aorta
Cardiovascular autonomic dysfunction such as postural orthostatic tachycardia syndrome
Raynauds phenomenon
Varicose veins
Heart murmur
Heart conduction abnormalities
Other manifestations
Hiatal hernia
Gastroesophageal reflux
Poor gastrointestinal motility
Dysautonomia
Gorlins sign (touch tongue to nose)
Anal prolapse
Flat feet
Tracheobronchomalacia / tracheal collapse
Collapsed lung (spontaneous pneumothorax)
Nerve disorders (carpal tunnel syndrome, acroparesthesia, neuropathy, including small fiber neuropathy)
Insensitivity to local anesthetics
Arnold–Chiari malformation
Platelet aggregation failure (platelets do not clump together properly)
Mast cell disorders (including mast cell activation syndrome and mastocytosis)
Pregnancy complications: increased pain, mild to moderate peripartum bleeding, cervical insufficiency, uterine tearing, or premature rupture of membranes
Hearing loss may occur in some types
Eye: Nearsightedness, retinal tearing and retinal detachment, keratoconus, blue sclera, dry eye, Sjogrens syndrome, lens subluxation, angioid streaks, epicanthal folds, strabismus, corneal scarring, brittle cornea syndrome, cataracts, carotid-cavernous sinus fistulas, macular degeneration
Craniocervical instability: caused by trauma(s) to the head and neck areas such as concussion and whiplash. Ligaments in neck are unable to heal properly, so the neck structure does not have the ability to support the skull, which can then sink into the brain stem, blocking the flow of cerebrospinal fluid, leading to issues related to the autonomic nervous system failing to work properly. Osteoporosis and osteopenia are associated with EDS and symptomatic joint hypermobility
There is some evidence that EDS may be associated with greater than expected frequencies of neurodevelopmental disorders such as attention deficit hyperactivity disorder (ADHD) and other learning, communication and motor issues, including autism spectrum conditions and Tourette syndrome. Because it is often undiagnosed or misdiagnosed in childhood, some instances of EDS have been mischaracterized as child abuse. | 11
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Hypertensive heart disease includes a number of complications of high blood pressure that affect the heart. While there are several definitions of hypertensive heart disease in the medical literature, the term is most widely used in the context of the International Classification of Diseases (ICD) coding categories. The definition includes heart failure and other cardiac complications of hypertension when a causal relationship between the heart disease and hypertension is stated or implied on the death certificate. In 2013 hypertensive heart disease resulted in 1.07 million deaths as compared with 630,000 deaths in 1990.According to ICD-10, hypertensive heart disease (I11), and its subcategories: hypertensive heart disease with heart failure (I11.0) and hypertensive heart disease without heart failure (I11.9) are distinguished from chronic rheumatic heart diseases (I05-I09), other forms of heart disease (I30-I52) and ischemic heart diseases (I20-I25). However, since high blood pressure is a risk factor for atherosclerosis and ischemic heart disease, death rates from hypertensive heart disease provide an incomplete measure of the burden of disease due to high blood pressure. Signs and symptoms
The symptoms and signs of hypertensive heart disease will depend on whether or not it is accompanied by heart failure. | The presentation may include headache, fever, shivering, joint pain, vomiting, hemolytic anemia, jaundice, hemoglobin in the urine, retinal damage, and convulsions.The classic symptom of malaria is paroxysm—a cyclical occurrence of sudden coldness followed by shivering and then fever and sweating, occurring every two days (tertian fever) in P. vivax and P. ovale infections, and every three days (quartan fever) for P. malariae. P. falciparum infection can cause recurrent fever every 36–48 hours, or a less pronounced and almost continuous fever.Symptoms typically begin 10–15 days after the initial mosquito bite, but can occur as late as several months after infection with some P. vivax strains. Travellers taking preventative malaria medications may develop symptoms once they stop taking the drugs.Severe malaria is usually caused by P. falciparum (often referred to as falciparum malaria). Symptoms of falciparum malaria arise 9–30 days after infection. Individuals with cerebral malaria frequently exhibit neurological symptoms, including abnormal posturing, nystagmus, conjugate gaze palsy (failure of the eyes to turn together in the same direction), opisthotonus, seizures, or coma. Complications
Malaria has several serious complications. Among these is the development of respiratory distress, which occurs in up to 25% of adults and 40% of children with severe P. falciparum malaria. Possible causes include respiratory compensation of metabolic acidosis, noncardiogenic pulmonary oedema, concomitant pneumonia, and severe anaemia. Although rare in young children with severe malaria, acute respiratory distress syndrome occurs in 5–25% of adults and up to 29% of pregnant women. Coinfection of HIV with malaria increases mortality. | 0-1
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Differential diagnosis
Other conditions with similar symptoms as Crohns disease includes intestinal tuberculosis, Behçets disease, ulcerative colitis, nonsteroidal anti-inflammatory drug enteropathy, irritable bowel syndrome and celiac disease. Irritable bowel syndrome is excluded when there are inflammatory changes. Celiac disease cannot be excluded if specific antibodies (anti-transglutaminase antibodies) are negative, nor in absence of intestinal villi atrophy. Management
There is no cure for Crohns disease and remission may not be possible or prolonged if achieved. In cases where remission is possible, relapse can be prevented and symptoms controlled with medication, lifestyle and dietary changes, changes to eating habits (eating smaller amounts more often), reduction of stress, moderate activity, and exercise. Surgery is generally contraindicated and has not been shown to prevent relapse. Adequately controlled, Crohns disease may not significantly restrict daily living. Treatment for Crohns disease involves first treating the acute problem and its symptoms, then maintaining remission of the disease. Lifestyle changes
Certain lifestyle changes can reduce symptoms, including dietary adjustments, elemental diet, proper hydration, and smoking cessation. Patients with Crohns disease are very interested in diet. Recent reviews underlined the importance to adopt diets that are best supported by evidence, even if little is known about the impact of diets on these patients. Diets that include higher levels of fiber and fruit are associated with reduced risk, while diets rich in total fats, polyunsaturated fatty acids, meat, and omega-6 fatty acids may increase the risk of Crohns. Maintaining a balanced diet with proper portion control can help manage symptoms of the disease. | Although both epididymis cyst and spermatocele may be referred as the same, the epididymis cyst does not contain sperm and it can occur anywhere within the epididymis. It can be differentiated through an ultrasound imagining. Epididymis cysts larger than 10mm in diameter are recommended for surgery but if there is no problem then surgery is discouraged as it can affect fertility in the future. Chronic infectious epididymitis
Chronic infectious epididymitis is rare. Some signs and symptoms include localized tenderness and swelling in the epididymis, which are different from any tenderness/abnormality present in the testis, these are usually not found in lower urinary tract. Chronic infectious epididymitis may be diagnosed in healthy adolescents as well as men. Some factors that predispose individuals to chronic infectious epididymitis include sexual activity, heavy physical exertion, and bicycle or motorcycle riding. Those diagnosed with chronic or recurrent epididymitis should receive a CT scan with contrast and a prostate ultrasonography to rule out structural abnormality of the urinary tract. If suspected to have chronic infectious epididymitis, consider getting a urinalysis, urine culture, and urine nucleic acid amplification tests for presence of Neisseria gonorrhoeae and Chlamydia trachomatis. Management of chronic infectious epididymitis is similar to management of acute infectious epididymitis, rarely does treatment extend to surgical management. Chronic noninfectious epididymitis
Chronic noninfectious epididymitis – Trauma, autoimmune disease, or vasculitis can cause chronic noninfectious epididymitis, but no clear cause or origin of the disease is found in most cases. | 0-1
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Folliculitis is the infection and inflammation of one or more hair follicles. The condition may occur anywhere on hair-covered skin. The rash may appear as pimples that come to white tips on the face, chest, back, arms, legs, buttocks, or head.Although acne can often involve superficial infection and inflammation of some hair follicles, the condition of those follicles is usually not called folliculitis, as that term is usually reserved for the separate set of disease entities comprising infected and inflamed hair follicles with causes other than acne. Signs and symptoms
Rash (reddened skin area)
Itching skin
Pimples or pustules located around a hair or follicle; may be confused with chicken pox
May crust over
Typically occur on neck, armpit, or groin
May present as genital lesions
Spreading from leg to arm to body through improper treatment with antibiotics
Complications
This condition can develop into a more severe skin condition, such as cellulitis or abscess. Causes
Most carbuncles, boils, and other cases of folliculitis are infected with Staphylococcus aureus.Folliculitis starts with the introduction of a skin pathogen to a hair follicle. Hair follicles can also be damaged by friction from clothing, an insect bite, blockage of the follicle, shaving, or braids that are very tight and close to the scalp. The damaged follicles are then infected by Staphylococcus spp. Folliculitis can affect people of all ages.Iron-deficiency anemia is sometimes associated with chronic cases. Bacterial
Staphylococcus aureus folliculitis
Hot-tub folliculitis is caused by the bacterium Pseudomonas aeruginosa. The folliculitis usually occurs after sitting in a hot tub that was not properly cleaned before use. | Phlegmasia alba dolens (also colloquially known as milk leg or white leg; not to be confused with phlegmasia cerulea dolens) is part of a spectrum of diseases related to deep vein thrombosis. Historically, it was commonly seen during pregnancy and in mothers who have just given birth. In cases of pregnancy, it is most often seen during the third trimester, resulting from a compression of the left common iliac vein against the pelvic rim by the enlarged uterus. Today, this disease is most commonly (40% of the time) related to some form of underlying malignancy. Hypercoagulability (a propensity to clot formation) is a well-known state that occurs in many cancer states. The incidence of this disease is not well reported. Cause
The disease presumably begins with a deep vein thrombosis that progresses to total occlusion of the deep venous system. It is at this stage that it is called phlegmasia alba dolens. It is a sudden (acute) process. The leg, then, must rely on the superficial venous system for drainage. The superficial system is not adequate to handle the large volume of blood being delivered to the leg via the arterial system. The result is edema, pain and a white appearance (alba) of the leg. The next step in the disease progression is occlusion of the superficial venous system, thereby preventing all venous outflow from the extremity. At this stage it is called phlegmasia cerulea dolens. The leg becomes more swollen and increasingly more painful. | 0-1
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Railway signal lights also rely heavily on red-green-yellow colors. In both cases, these color combinations can be difficult for the red-green colorblind. Lantern Tests are a common means of simulating these light sources to determine not necessarily whether someone is colorblind, but whether they can functionally distinguish these specific signal colors. Those who cannot pass this test are generally completely restricted from working on aircraft, ships or rail. Fashion
Color analysis is the analysis of color in its use in fashion, to determine personal color combinations that are most aesthetic. Colors to combine can include clothing, accessories, makeup, hair color, skin color, eye color, etc. Color analysis involves many aesthetic and comparative color task that can be difficult for the color blind. Most colorblind individuals conservatively avoid brightly colored clothes to avoid combining colors that may be viewed as unaesthetic by people with normal color vision. Advantages
People with deuteranomaly are better at distinguishing shades of khaki, which may be advantageous when looking for predators, food, or camouflaged objects hidden among foliage. Dichromats tend to learn to use texture and shape clues and so may be able to penetrate camouflage that has been designed to deceive individuals with normal color vision.In the presence of chromatic noise, the colorblind are more capable of seeing a luminous signal, as long as the chromatic noise appears metameric to them. This is the effect behind most "reverse" Pseudoisochromatic plates (e.g. "hidden digit" Ishihara plates) that are discernible to the colorblind, but unreadable to color normals. | The degree to which women who are carriers of either protanomaly or deuteranomaly are demonstrably tetrachromatic and require a mixture of four spectral lights to match an arbitrary light is very variable. Jameson et al. have shown that with appropriate and sufficiently sensitive equipment it can be demonstrated that any female carrier of red–green color blindness (i.e. heterozygous protanomaly, or heterozygous deuteranomaly) is a tetrachromat to a greater or lesser extent. Since the incidence of anomalous trichromacy in males is ~6%, which should equal the incidence of anomalous M opsin or L opsin alleles, it follows that the prevalence of unaffected female carriers of colorblindness (and therefore of potential tetrachromats) is 11.3% (i.e. 94% × 6% × 2), based on the Hardy–Weinberg principle. One such woman has been widely reported to be a true or functional tetrachromat, as she can discriminate colors most other people cant. Diagnosis
There are several color perception tests, or color vision standards that are capable of diagnosing or screening for color blindness. The Ishihara color test, which consists of a series of pictures of colored spots, is the test most often used to detect red–green color deficiencies and most often recognized by the public. However, this can be attributed more to its ease of application, and less to do with its precision. In fact, there are several types of common color perception tests. Most clinical tests are designed to be fast, simple, and effective at identifying broad categories of color blindness. | 11
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In addition, disease that initially involves the testes, breast, or uterus has a relatively high rate of spreading to the central nervous system while disease initially involving the kidneys, adrenal glands, ovaries, or bone marrow has a high rate of spreading to other organs, including the central nervous system. All of these cases as well as cases initially involving the central nervous system have relatively poor to very poor prognoses. Cases initially involving the stomach, thyroid, or a single bone site have relatively good prognoses. Pathophysiology
Most cases of DLBCL, NOS appear to result at least in part from the step-wise development of gene changes such as mutations, altered expressions, amplifications (i.e. increases in the number of copies of specific genes), and tranlocations from normal sites to other chromosomal sites. These changes often result in gains or loses in the production or function of the product of these genes and thereby the activity of cell signaling pathways that regulate the maturation, proliferation, survival, spread, evasion of the immune system, and other malignant behaviors of the cells in which they occur. While scores of genes have been reported to be altered in DLBCL, NOS many of these may not contribute to DLBCL, NOS. Changes in the following genes occur frequently in, and are suspected of contributing to, this diseases development and/or progression. BCL2: This gene is a protooncogene, i.e. a normal gene that can become cancer-causing when mutated or overexpressed. Its product, Bcl-2 protein, regulates cellular apoptosis (i.e. | These lymphomas, termed high-grade B-cell lymphoma with MYC, BL2, and/or BL6 rearrangements or, more simply, DH/THL, are regarded as borderline DLBCL,NOS. They represent 6–14% of all DLBCL, NOS and have had long-term survival rates of only 20–25%. Another variant B-cell lymphoma that is also considered to be a borderline DLBCL, NOS is termed high-grade B-cell lymphoma, not otherwise specified (HGBCL, NOS). These two aggressive borderline B-cell lymphomas were previously grouped together as "B-cell lymphoma, unclassifiable with features intermediate between DLBCL and Burkitt lymphoma" (i.e. BCLU) but were separated into DH/THL and HGBC, NOS by the World Health Organization, 2016. The neoplastic cells in a related variant, double expresser lymphoma (i.e. DEL), express the products of MYC and BCL2 genes, i.e. c-Myc and bcl-2 proteins, respectively, but do not have translocations in either of their genes. DEL, which represents about one-third of all DLBCL, NOS cases, has a poorer prognosis than standard DLBCL, NOS but not as poor as DH/THL cases. Cases in which the neoplastic cells have alterations in the MYC gene or its expression without changes in BLC2 or BLC6 also have a poor prognosis, particularly in cases where the MYC gene translocates (i.e. rearranges) with one of the immunoglobulin gene loci. DLBCL that begin in the testicles are a variant of DLBCL, NOS that some authors suggest should be classified as a distinct DLBCL subtype. This variant, termed Primary testicular diffuse large B-cell lymphoma (PT-DLBCL), is a DLBCL, NOS that in >75% of cases involves activated B-cells, i.e. ABC. | 11
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Cervical and postocciptal lymphadenopathy can also be seen, but this generally presents 2–4 days after the onset of the febrile phase.In rare cases, HHV-6 can become active in an adult previously infected during childhood and can show signs of mononucleosis. Cause
There are nine known human herpesviruses. Of these, roseola has been linked to two: human herpesvirus 6 (HHV-6) and human herpesvirus 7 (HHV-7), which are sometimes referred to collectively as Roseolovirus. These viruses are of the Herpesviridae family and the Betaherpesvirinae subfamily, underwhich Cytomegalovirus is also classified. HHV-6 has been further classified into HHV-6A and HHV-6B, two distinct viruses which share 88% of the same DNA makeup, with HHV-6B the most common cause of roseola.After infection, these viruses enter a latent phase. Roseola caused by HHV-7 has been linked to the ability of HHV-7 infection to reactivate latent HHV-6. Spread
After exposure to roseola, the causative virus becomes latent in its host but is still present in saliva, skin, and lungs. HHV-6 is thought to be transmitted from previously exposed or infected adults to young children by the shedding of virus through saliva. Even so, most cases of roseola are transmitted without known exposure. Diagnosis
The diagnosis of roseola is made clinically based on the presence of the two phases: fever and rash. Laboratory testing is seldom used as the results do not alter management of the disease. | Signs and symptoms
Fever
Symptoms begin with a three to six day febrile illness. During this time, temperatures can peak above 40 °C and children can experience increased irritability with general malaise. However, many children in the febrile phase feel well, engaged, and alert. For these patients, fever is usually diagnosed incidentally.The most common complication (10-15% of children between 6 and 18 months) and most common cause of hospitalization in children with primary infection of HHV-6B is febrile seizures which can precipitate status epilepticus due to the sudden rise in body temperature. Rash
Once the febrile phase subsides, a rash develops. In some cases, the rash can present after one or two days after the fever resolves. The rash is classically described as an erythematous morbilliform exanthem and presents as a distribution of soft pink, discrete, and slightly raised lesions each with a 2-5mm diameter. It classically begins on the trunk (torso) and spreads outward to the neck, extremities, and face. This pattern is referred to as a centrifugal spread. Usually, peeling and itching are not characteristic of this rash. This phase can last anywhere from several hours to 2 days. Other symptoms
A small percentage of children acquire HHV-6 with few signs or symptoms of the disease. Children with HHV-6 infection can also present with myringitis (inflammation of the tympanic membranes), upper respiratory symptoms, diarrhea, and a bulging fontanelle. In addition, children can experience pharyngitis with lymphoid hyperplasia seen on the soft palate and swelling of the eyelids. These symptoms usually present during the febrile phase of roseola. | 11
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Motion sickness occurs due to a difference between actual and expected motion. Symptoms commonly include nausea, vomiting, cold sweat, headache, dizziness, tiredness, loss of appetite, and increased salivation. Complications may rarely include dehydration, electrolyte problems, or a lower esophageal tear.The cause of motion sickness is either real or perceived motion. This may include from car travel, air travel, sea travel, space travel, or reality simulation. Risk factors include pregnancy, migraines, and Ménières disease. The diagnosis is based on symptoms.Treatment may include behavioral measures or medications. Behavioral measures include keeping the head still and focusing on the horizon. Three types of medications are useful: antimuscarinics such as scopolamine, H1 antihistamines such as dimenhydrinate, and amphetamines such as dexamphetamine. Side effects, however, may limit the use of medications. A number of medications used for nausea such as ondansetron are not effective for motion sickness.Nearly all people are affected with sufficient motion and most people will experience motion sickness at least once in their lifetime. Susceptibility, however, is variable, with about one-third of the population being highly susceptible while most other people are affected under extreme conditions. Women are more easily affected than men. Motion sickness has been described since at least the time of Homer (c. eighth century BC). Signs and symptoms
Symptoms commonly include nausea, vomiting, cold sweat, headache, dizziness, tiredness, loss of appetite, and increased salivation. Occasionally, tiredness can last for hours to days after an episode of motion sickness, known as "sopite syndrome". | Studies have shown that exposure to rotational motions in a virtual environment can cause significant increases in nausea and other symptoms of motion sickness.In a study conducted by the U.S. Army Research Institute for the Behavioral and Social Sciences in a report published May 1995 titled "Technical Report 1027 – Simulator Sickness in Virtual Environments", out of 742 pilot exposures from 11 military flight simulators, "approximately half of the pilots (334) reported post-effects of some kind: 250 (34%) reported that symptoms dissipated in less than one hour, 44 (6%) reported that symptoms lasted longer than four hours, and 28 (4%) reported that symptoms lasted longer than six hours. There were also four (1%) reported cases of spontaneously occurring flashbacks." Motion that is seen and felt
When moving within a rotating reference frame such as in a centrifuge or environment where gravity is simulated with centrifugal force, the coriolis effect causes a sense of motion in the vestibular system that does not match the motion that is seen. Pathophysiology
There are various hypotheses that attempt to explain the cause of the condition. Sensory conflict theory
Contemporary sensory conflict theory, referring to "a discontinuity between either visual, proprioceptive, and somatosensory input, or semicircular canal and otolith input", is probably the most thoroughly studied. According to this theory, when the brain presents the mind with two incongruous states of motion; the result is often nausea and other symptoms of disorientation known as motion sickness. | 11
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Orthokeratology
Orthokeratology or simply Ortho-K is a temporary corneal reshaping process using rigid gas permeable (RGP) contact lenses. Overnight wearing of specially designed contact lenses will temporarily reshape cornea, so patients may see clearly without any lenses in daytime. Orthokeratology can correct myopia up to -6D. Several studies shown that Ortho-K can reduce myopia progression also. Risk factors of using Ortho-K lenses include microbial keratitis, corneal edema, etc. Other contact lens related complications like corneal aberration, photophobia, pain, irritation, redness etc. are usually temporary conditions, which may be eliminated by proper usage of lenses. Intrastromal corneal ring segment
The Intrastromal corneal ring segment (ICRS), commonly used in keratoconus treatment now, was originally designed to correct mild to moderate myopia. The thickness is directly related to flattening and the diameter of the ring is proportionally inverse to the flattening of cornea. So, if diameter is smaller or thickness is greater, resulting myopia correction will be greater. Alternative medicine
A number of alternative therapies have been claimed to improve myopia, including vision therapy, "behavioural optometry", various eye exercises and relaxation techniques, and the Bates method. Scientific reviews have concluded that there was "no clear scientific evidence" that eye exercises are effective in treating near-sightedness and as such they "cannot be advocated". Epidemiology
Global refractive errors have been estimated to affect 800 million to 2.3 billion. The incidence of myopia within sampled population often varies with age, country, sex, race, ethnicity, occupation, environment, and other factors. | This means the defective gene responsible for the disorder is located on an autosome (chromosome 8 is an autosome), and two copies of the defective gene (one inherited from each parent) are required in order to be born with the disorder. The parents of an individual with an autosomal recessive disorder both carry one copy of the defective gene, but usually do not experience any signs or symptoms of the disorder. DNA repair
RECQL4 has a crucial role in DNA end resection that is the initial step required for homologous recombination (HR)-dependent double-strand break repair. When RECQL4 is depleted, HR-mediated repair and 5’ end resection are severely reduced in vivo. RECQL4 also appears to be necessary for other forms of DNA repair including non-homologous end joining, nucleotide excision repair and base excision repair. The association of deficient RECQL4-mediated DNA repair with accelerated aging is consistent with the DNA damage theory of aging. Diagnosis
Management
History
The condition was originally described by August von Rothmund (1830–1906) in 1868. Matthew Sydney Thomson (1894–1969) published further descriptions in 1936. See also
Poikiloderma vasculare atrophicans
List of cutaneous conditions
List of radiographic findings associated with cutaneous conditions
References
External links
GeneReviews/NCBI/NIH/UW entry on Rothmund-Thomson Syndrome
Poikiloderma of Rothmund-Thomson at NIHs Office of Rare Diseases | 0-1
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Sure, youve got a headache, youre tense, irritable, but dont take it out on her!" Another commercial had a wife greeting her husband as he pulled into their driveway in his car; the husband responded by yelling "Helen, cant you keep Billys bike out of the driveway?!?" These advertisement scenarios became popular and were parodied a number of times, including in the Allan Sherman song "Headaches", the 1966 film The Silencers and the 1980 film Airplane. The medication was mentioned in the book "The Shining" by Stephen King. Anacin had a large advertisement behind the center field fence of Yankee Stadium from the 1950s through 1973, until the stadiums 1974-75 renovation. Products
Anacin covers a family of pain relievers. There are currently two different formulations:
Anacin Regular Strength – contains 400 mg ASA (aspirin) and 32 mg caffeine per tablet. Anacin Max Strength – contains 500 mg ASA and 32 mg caffeine per tablet. Side effects
Anacins side effects may include dizziness, heartburn, irritability, nausea, nervousness, rashes, hives, bloody stools, drowsiness, hearing loss, ringing in the ears, and trouble sleeping. See also
Anadin, an Anacin brand sold in the United Kingdom, launched in 1932. References
External links
Official website
Prestige Brands Anacin
Insight Pharmaceuticals - Anacin | In the segment, a young Australian woman was stung and developed a severe case.A 2007 fictional Sea Patrol episode (S1, E4) involves two crew members of HMAS Hammersley being stung by an Irukandji jellyfish.On the television program Super Animal, a woman compared her experience with Irukandji syndrome to the pain of childbirth.Steve Backshall reports with accounts from victims of Irukandji stings on his ITV wildlife series Fierce in 2016. == References == | 0-1
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Once nucleus accumbens ΔFosB is sufficiently overexpressed, it begins to increase the severity of addictive behavior (i.e., compulsive drug-seeking) with further increases in its expression. While there are currently no effective drugs for treating amphetamine addiction, regularly engaging in sustained aerobic exercise appears to reduce the risk of developing such an addiction. Sustained aerobic exercise on a regular basis also appears to be an effective treatment for amphetamine addiction; exercise therapy improves clinical treatment outcomes and may be used as an adjunct therapy with behavioral therapies for addiction. Biomolecular mechanisms
Chronic use of amphetamine at excessive doses causes alterations in gene expression in the mesocorticolimbic projection, which arise through transcriptional and epigenetic mechanisms. The most important transcription factors that produce these alterations are Delta FBJ murine osteosarcoma viral oncogene homolog B (ΔFosB), cAMP response element binding protein (CREB), and nuclear factor-kappa B (NF-κB). ΔFosB is the most significant biomolecular mechanism in addiction because ΔFosB overexpression (i.e., an abnormally high level of gene expression which produces a pronounced gene-related phenotype) in the D1-type medium spiny neurons in the nucleus accumbens is necessary and sufficient for many of the neural adaptations and regulates multiple behavioral effects (e.g., reward sensitization and escalating drug self-administration) involved in addiction. Once ΔFosB is sufficiently overexpressed, it induces an addictive state that becomes increasingly more severe with further increases in ΔFosB expression. | The epoophoron or epoöphoron (also called organ of Rosenmüller or the parovarium) is a remnant of the mesonephric tubules that can be found next to the ovary and fallopian tube. Anatomy
It may contain 10–15 transverse small ducts or tubules that lead to the Gartner’s duct (also longitudinal duct of epoophoron) that represents the caudal remnant of the mesonephric duct and passes through the broad ligament and the lateral wall of the cervix and vagina. The epoophoron is a homologue to the epididymis in the male. While the epoophoron is located in the lateral portion of the mesosalpinx and mesovarium, the paroophoron (residual remnant of that part of the mesonephric duct that forms the paradidymis in the male) lies more medially in the mesosalpinx. Histology
It has a unique histological profile. Clinical significance
Clinically the organ may give rise to a local paraovarian cyst or adenoma. See also
List of homologues of the human reproductive system
Vesicular appendages of epoophoron
References
External links
figures/chapter_35/35-8.HTM: Basic Human Anatomy at Dartmouth Medical School
genital-016a—Embryo Images at University of North Carolina
Swiss embryology (from UL, UB, and UF) ugenital/genitinterne05 | 0-1
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Surgical
Surgery is indicated if the case is post-traumatic, iatrogenic, or refractory to other treatments, in which cases surgery reduces mortality by 40%. One invasive surgical intervention called a thoracic duct ligation involves closing off the thoracic ducts. Surgical pleurodesis is another option and can be undertaken if the affected person fails to respond to conservative treatment and is not a candidate for surgical intervention.Another treatment option is pleuroperitoneal shunting (creating a communication channel between the pleural space and peritoneal cavity). Since surgery to close the leak is not reliable, talc pleurodesis is recommended; in a case study of 19 people with refractory malignant chylothorax due to lymphoma, it resulted in success for all affected individuals. Chemical pleurodesis is an option, since the leaking of lymphatic fluids is stopped by irritating the lungs and chest wall, resulting in a sterile inflammation. This causes the lung and the chest wall to fuse together, thus preventing lymphatic fluids from leaking into the pleural space. Prognosis
The morbidity and mortality rates associated with chylothorax have declined as treatments have improved. Malignant, bilateral, and chronic chylothoraces have an inferior prognosis to other types. Currently, the mortality and morbidity rates are about 10% if treated surgically. If cases are post-operative and treated conservatively, mortality rates approach 50%. Complications
Complications of chylothorax include malnutrition, immunosuppression, dehydration, and respiratory distress. The severity of the complications depends on how quickly the chylothorax accumulated, its size, and its chronicity. Epidemiology
Chylothoraces are rare and usually occur as a complication of surgeries in the neck and mediastinum. | Chylotharax after trauma but not after surgery has also been described after central line placement, pacemaker implantation, and embolization of a pulmonary arteriovenous malformation. Blunt trauma to the chest region is another cause of chylothorax, which has occurred after blast injuries and even simple injuries from coughing or sneezing. Mechanism
The main mechanism of chylothorax is the leaking of chyle from the thoracic duct, usually caused by a disturbance affecting the structural integrity of the thoracic duct. For example, placement of a central venous catheter can potentially disrupt drainage of lymph into the subclavian veins, followed by the thoracic duct, resulting in chylothorax. The disturbances cause the pressure in the thoracic duct to increase. Soon, collateral channels form, which eventually drain into the thorax. Trauma affecting the thoracic duct is the most common disturbing mechanism. Whether a chylothorax occurs in the left or right pleural space is a consequence of the thoracic ducts anatomic location in the body and depends on the level where the duct was injured. If the thoracic duct is injured above the fifth thoracic vertebra, then a left-sided chylothorax results. Conversely, a thoracic duct injury below that level will lead to the formation of a right-sided chylothorax. Chylothoraces most commonly occur in the right pleural space (50% of cases). Left-sided and bilateral chylothoraces are less common and occur in 33% and 17% of cases, respectively.In the case of cancer, invasion into the thoracic duct or collateral lymph channels can obstruct lymph. | 11
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If used at all it should only be considered if the blood pH is less than 7.0.People with anaphylactic shock are commonly treated with epinephrine. Antihistamines, such as Benadryl (diphenhydramine) or ranitidine are also commonly administered. Albuterol, normal saline, and steroids are also commonly given. Mechanical support
Intra-aortic balloon pump (IABP) – a device inserted into the aorta that mechanically raises the blood pressure. Use of Intra-aortic balloon pumps is not recommended in cardiogenic shock. Ventricular assist device (VAD) – A mechanical pump that helps pump blood throughout the body. Commonly used in short term cases of refractory primary cardiogenic shock. Artificial heart (TAH)
Extracorporeal membrane oxygenation (ECMO) – an external device that completely replaces the work of the heart. Treatment goals
The goal of treatment is to achieve a urine output of greater than 0.5 mL/kg/h, a central venous pressure of 8–12 mmHg and a mean arterial pressure of 65–95 mmHg. In trauma the goal is to stop the bleeding which in many cases requires surgical interventions. A good urine output indicates that the kidneys are getting enough blood flow. Epidemiology
Septic shock (a form of distributive shock), is the most common form of shock. Shock from blood loss occurs in about 1–2% of trauma cases. Overall, up to one-third of people admitted to the intensive care unit (ICU) are in circulatory shock. Of these, cardiogenic shock accounts for approximately 20%, hypovolemic about 20%, and septic shock about 60% of cases. Prognosis
The prognosis of shock depends on the underlying cause and the nature and extent of concurrent problems. | Herpes simplex is a viral infection caused by the herpes simplex virus. Infections are categorized based on the part of the body infected. Oral herpes involves the face or mouth. It may result in small blisters in groups often called cold sores or fever blisters or may just cause a sore throat. Genital herpes, often simply known as herpes, may have minimal symptoms or form blisters that break open and result in small ulcers. These typically heal over two to four weeks. Tingling or shooting pains may occur before the blisters appear. Herpes cycles between periods of active disease followed by periods without symptoms. The first episode is often more severe and may be associated with fever, muscle pains, swollen lymph nodes and headaches. Over time, episodes of active disease decrease in frequency and severity. Other disorders caused by herpes simplex include: herpetic whitlow when it involves the fingers, herpes of the eye, herpes infection of the brain, and neonatal herpes when it affects a newborn, among others.There are two types of herpes simplex virus, type 1 (HSV-1) and type 2 (HSV-2). HSV-1 more commonly causes infections around the mouth while HSV-2 more commonly causes genital infections. They are transmitted by direct contact with body fluids or lesions of an infected individual. Transmission may still occur when symptoms are not present. Genital herpes is classified as a sexually transmitted infection. It may be spread to an infant during childbirth. | 0-1
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The dosage is 1 to 2 mg/kg, usually given at 100 mg. Ketamine is metabolised by liver and excreted through kidneys. The drug lessen the reuptake of the catecholamine, increases heart rate, blood pressure, and cardiac output, thus suitable for those with hypotension. However, it can worsen the cardiac depression and hypotension for those with depletion of catecholamines. Thus, maximum dose of 1.5 mg/kg is need for this situation. For those with head injuries, ketamine does not appear to increase intracranial pressure, while able to maintain the mean arterial pressure. Ketamine also relieves bronchospasm by relaxing bronchiolar smooth muscles. However, it increases oral secretions during intubation. Ketamine is associated with nightmares, delirium, and hallucinations. Propofol – It is a highly lipid-soluble, GABA agonist. The dosage is 1.5 mg/kg (usually 100 to 200 mg). It has quick onset of action, can cross the blood-brain barrier, wide tissue distribution, and can be cleared by the body quickly. In the elderly, the rate of propofol clearance is low. Therefore, lower doses of propofol (50 to 100 mg) should be given. It is suitable in those with kidney or liver impairment and decreases intra-cranial pressure. For those with bronchospasm, propofol also has mild bronchodilating effect. However, propofol can induce hypotension and bradycardia due to its calcium channel blocker and beta blocker properties. At prolonged high propofol dosages, it can induce propofol infusion syndrome. Pain during peripheral administration of propofol can be reduced by using a large bore cannula. | Melanin is found in the small melanosomes, particles formed in melanocytes from where they are transferred to the surrounding keratinocytes. The size, number, and arrangement of the melanosomes vary between racial groups, but while the number of melanocytes can vary between different body regions, their numbers remain the same in individual body regions in all human beings. In white and Asian skin the melanosomes are packed in "aggregates", but in black skin they are larger and distributed more evenly. The number of melanosomes in the keratinocytes increases with UV radiation exposure, while their distribution remain largely unaffected. Clinical significance
Laboratory culture of keratinocytes to form a 3D structure (artificial skin) recapitulating most of the properties of the epidermis is routinely used as a tool for drug development and testing. Hyperplasia
Epidermal hyperplasia (thickening resulting from cell proliferation) has various forms:
Acanthosis is diffuse epidermal hyperplasia (thickening of the skin, and not to be confused with acanthocytes). It implies increased thickness of the Malpighian layer (stratum basale and stratum spinosum). Acanthosis nigricans is a black, poorly defined, velvety hyperpigmented acanthosis, usually observed in the back of neck, axilla, and other folded regions of the skin. Focal epithelial hyperplasia (Hecks disease) is an asymptomatic, benign neoplastic condition characterized by multiple white to pinkish papules that occur diffusely in the oral cavity. : 411
Pseudoepitheliomatous hyperplasia (PEH) is a benign condition characterized by hyperplasia of the epidermis and epithelium of skin appendages, with irregular squamous strands extending down into the dermis, and closely simulating squamous cell carcinoma (SCC). | 0-1
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Thus, polygyny may also be due to a lower male: female ratio in these areas but this may ultimately be due to male infants having increased mortality from infectious diseases. Another example is that poor socioeconomic factors may ultimately in part be due to high pathogen load preventing economic development. Fossil record
Evidence of infection in fossil remains is a subject of interest for paleopathologists, scientists who study occurrences of injuries and illness in extinct life forms. Signs of infection have been discovered in the bones of carnivorous dinosaurs. When present, however, these infections seem to tend to be confined to only small regions of the body. A skull attributed to the early carnivorous dinosaur Herrerasaurus ischigualastensis exhibits pit-like wounds surrounded by swollen and porous bone. The unusual texture of the bone around the wounds suggests they were affected by a short-lived, non-lethal infection. Scientists who studied the skull speculated that the bite marks were received in a fight with another Herrerasaurus. Other carnivorous dinosaurs with documented evidence of infection include Acrocanthosaurus, Allosaurus, Tyrannosaurus and a tyrannosaur from the Kirtland Formation. The infections from both tyrannosaurs were received by being bitten during a fight, like the Herrerasaurus specimen. Outer space
A 2006 Space Shuttle experiment found that Salmonella typhimurium, a bacterium that can cause food poisoning, became more virulent when cultivated in space. | A perforation is a small hole in a thin material or web. There is usually more than one perforation in an organized fashion, where all of the holes collectively are called a perforation. The process of creating perforations is called perforating, which involves puncturing the workpiece with a tool. Perforations are usually used to allow easy separation of two sections of the material, such as allowing paper to be torn easily along the line. Packaging with perforations in paperboard or plastic film is easy for consumers to open. Other purposes include filtrating fluids, sound deadening, allowing light or fluids to pass through, and to create an aesthetic design.Various applications include plastic films to allow the packages to breathe, medical films, micro perforated plate and sound and vapor barriers. Processes
Pins and needles
Rotary pinned perforation rollers are precision tools that can be used to perforate a wide variety of materials. The pins or needles can be used cold or heated. Cold perforation tools include needle punches. There are a handful of manufacturers that specialize in hot and cold needle perforation tooling and equipment. In materials that have elasticity this can result in a "volcano" hole that is preferred in many applications. Pinned rollers can be made from a variety of materials, including plastic, steel, and aluminum. In more brittle films, cold perforation can cause slitting rather than creating a round hole, which can jeopardize the materials integrity under pressure. The solution to this is often heating the pin; i.e. hot pin perforation. | 0-1
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In some lung diseases, especially emphysema, it is possible for abnormal lung areas such as bullae (large air-filled sacs) to have the same appearance as a pneumothorax on chest X-ray, and it may not be safe to apply any treatment before the distinction is made and before the exact location and size of the pneumothorax is determined. In trauma, where it may not be possible to perform an upright film, chest radiography may miss up to a third of pneumothoraces, while CT remains very sensitive.A further use of CT is in the identification of underlying lung lesions. In presumed primary pneumothorax, it may help to identify blebs or cystic lesions (in anticipation of treatment, see below), and in secondary pneumothorax, it can help to identify most of the causes listed above. Ultrasound
Ultrasound is commonly used in the evaluation of people who have sustained physical trauma, for example with the FAST protocol. Ultrasound may be more sensitive than chest X-rays in the identification of pneumothorax after blunt trauma to the chest. Ultrasound may also provide a rapid diagnosis in other emergency situations, and allow the quantification of the size of the pneumothorax. Several particular features on ultrasonography of the chest can be used to confirm or exclude the diagnosis. Treatment
The treatment of pneumothorax depends on a number of factors and may vary from discharge with early follow-up to immediate needle decompression or insertion of a chest tube. | Known lung diseases that may significantly increase the risk for pneumothorax are
In children, additional causes include measles, echinococcosis, inhalation of a foreign body, and certain congenital malformations (congenital pulmonary airway malformation and congenital lobar emphysema).11.5% of people with a spontaneous pneumothorax have a family member who has previously experienced a pneumothorax. The hereditary conditions – Marfan syndrome, homocystinuria, Ehlers–Danlos syndromes, alpha 1-antitrypsin deficiency (which leads to emphysema), and Birt–Hogg–Dubé syndrome – have all been linked to familial pneumothorax. Generally, these conditions cause other signs and symptoms as well, and pneumothorax is not usually the primary finding. Birt–Hogg–Dubé syndrome is caused by mutations in the FLCN gene (located at chromosome 17p11.2), which encodes a protein named folliculin. FLCN mutations and lung lesions have also been identified in familial cases of pneumothorax where other features of Birt–Hogg–Dubé syndrome are absent. In addition to the genetic associations, the HLA haplotype A2B40 is also a genetic predisposition to PSP. Traumatic
A traumatic pneumothorax may result from either blunt trauma or penetrating injury to the chest wall. The most common mechanism is due to the penetration of sharp bony points at a new rib fracture, which damages lung tissue. Traumatic pneumothorax may also be observed in those exposed to blasts, even though there is no apparent injury to the chest.They may be classified as "open" or "closed". In an open pneumothorax, there is a passage from the external environment into the pleural space through the chest wall. | 11
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Surrounding gingival tissue may experience lymphadenopathy. The alveolar plate may exhibit crepitus when palpated. Complications
Expansion of the cyst causes erosion of the floor of the maxillary sinus. As soon as it enters the maxillary antrum, the expansion rate increases due to available space for expansion. Performing a percussion test by tapping the affected teeth will cause shooting pain. This is often clinically diagnostic of pulpal infection. Causes
Dental cysts are usually caused due to root infection involving tooth decay. Untreated dental caries then allow bacteria to reach the level of the pulp, causing infection. The bacteria gains access to the periapical region of the tooth through deeper infection of the pulp, traveling through the roots. The resulting pulpal necrosis causes proliferation of epithelial rests of Malassez which release toxins at the apex of the tooth. The bodys inflammatory response will attack the source of the toxins, leading to periapical inflammation. The many cells and proteins that rush to an area of infection create osmotic tension in the periapex which is the source of internal pressure increase at the cyst site. These lesions can grow large because they apply pressure over the bone, causing resorption. The toxins released by the breakdown of granulation tissue are one of the common causes of bone resorption. There are two schools of thought regarding cyst expansion. | Commonly known as a dental cyst, the periapical cyst is the most common odontogenic cyst. It may develop rapidly from a periapical granuloma, as a consequence of untreated chronic periapical periodontitis.Periapical is defined as "the tissues surrounding the apex of the root of a tooth" and a cyst is "a pathological cavity lined by epithelium, having fluid or gaseous content that is not created by the accumulation of pus. "Most frequently located in the maxillary anterior region, the cyst is caused by pulpal necrosis secondary to dental caries or trauma. Its lining is derived from the epithelial cell rests of Malassez which proliferate to form the cyst. Such cysts are very common. Although initially asymptomatic, they are clinically significant because secondary infection can cause pain and damage. In radiographs, the cyst appears as a radiolucency (dark area) around the apex of a tooths root. Signs and symptoms
Periapical cysts begin as asymptomatic and progress slowly. Subsequent infection of the cyst causes swelling and pain. Initially, the cyst swells to a round hard protrusion, but later on the body resorbs some of the cyst wall, leaving a softer accumulation of fluid underneath the mucous membrane.Secondary symptoms of periapical cysts include inflammation and infection of the pulp causing dental caries. This infection is what causes necrosis of the pulp.Larger cysts may cause bone expansion or displace roots. Discoloration of the affected tooth may also occur. Patient will present negative results to electric and ice test of the affected tooth but will be sensitive to percussion. | 11
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However, whether the extent of CYP3A4/5 induction at therapeutic doses reaches clinical significance is unclear. Furthermore, for example phenytoin and phenobarbital are known to reduce plasma levels of licarbazepine through induction of Cytochrome P450 enzymes. Pharmacology
Oxcarbazepine is a prodrug, which is largely metabolized to its pharmacologically active 10-monohydroxy derivative licarbazepine (sometimes abbreviated MHD). Oxcarbazepine and MHD exert their action by blocking voltage-sensitive sodium channels, thus leading to the stabilization of hyper-excited neural membranes, suppression of repetitive neuronal firing and diminishment propagation of synaptic impulses. Furthermore, anticonvulsant effects of these compounds could be attributed to enhanced potassium conductance and modulation of high-voltage activated calcium channels. Pharmacokinetics
Oxcarbazepine has high bioavailability upon oral administration. In a study in humans, only 2% of oxcarbazepine remained unchanged, 70% were reduced to licarbazepine; the rest were minor metabolites. The half-life of oxcarbazepine is considered to be about 2 hours, whereas licarbazepine has a half-life of nine hours. Through its chemical difference to carbamazepine metabolic epoxidation is avoided, reducing hepatic risks. Licarbazepine is metabolised by conjugation with Glucuronic acid. Approximately 4% are oxidised to the inactive 10,11-dihydroxy derivative. Elimination is almost completely renal, with faeces accounting to less than 4%. 80% of the excreted substances are to be attributed to licarbazepine or its glucuronides. Pharmacodynamics
Both oxcarbazepine and licarbazepine were found to show anticonvulsant properties in seizure models done on animals. These compounds had protective functions whenever tonic extension seizures were induced electrically, but such protection was less apparent whenever seizures were induced chemically. | History
First made in 1966, it was patent-protected by Geigy in 1969 through DE 2011087 . It was approved for use as an anticonvulsant in Denmark in 1990, Spain in 1993, Portugal in 1997, and eventually for all other EU countries in 1999. It was approved in the US in 2000. In September 2010, Novartis, of which Geigy are part of its corporate roots, pleaded guilty to marketing Trileptal for the unapproved uses of neuropathic pain and bipolar disorder. Research
Research has investigated the use of oxcarbazepine as a mood stabilizer in bipolar disorder, with further evidence needed to fully assess its suitability. It may be beneficial in trigeminal neuralgia. References
External links
"Oxcarbazepine". Drug Information Portal. U.S. National Library of Medicine. | 11
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With more severe iron-deficiency anemia, the peripheral blood smear may show hypochromic, pencil-shaped cells and, occasionally, small numbers of nucleated red blood cells. The platelet count may be slightly above the high limit of normal in iron-deficiency anemia (termed a mild thrombocytosis), but severe cases can present with thrombocytopenia (low platelet count).Iron-deficiency anemia is confirmed by tests that include serum ferritin, serum iron level, serum transferrin, and total iron binding capacity. A low serum ferritin is most commonly found. However, serum ferritin can be elevated by any type of chronic inflammation and thus is not consistently decreased in iron-deficiency anemia. Serum iron levels may be measured, but serum iron concentration is not as reliable as the measurement of both serum iron and serum iron-binding protein levels. The percentage of iron saturation (or transferrin saturation index or percent) can be measured by dividing the level of serum iron by total iron binding capacity and is a value that can help to confirm the diagnosis of iron-deficiency anemia; however, other conditions must also be considered, including other types of anemia.Another finding that can be used is the level of red blood cell distribution width. During haemoglobin synthesis, trace amounts of zinc will be incorporated into protoporphyrin in the place of iron which is lacking. Protoporphyrin can be separated from its zinc moiety and measured as free erythrocyte protoporphyrin, providing an indirect measurement of the zinc-protoporphyrin complex. The level of free erythrocyte protoporphyrin is expressed in either μg/dl of whole blood or μg/dl of red blood cells. | Pathophysiology
An abscess is a defensive reaction of the tissue to prevent the spread of infectious materials to other parts of the body. The organisms or foreign materials kill the local cells, resulting in the release of cytokines. The cytokines trigger an inflammatory response, which draws large numbers of white blood cells to the area and increases the regional blood flow. The final structure of the abscess is an abscess wall, or capsule, that is formed by the adjacent healthy cells in an attempt to keep the pus from infecting neighboring structures. However, such encapsulation tends to prevent immune cells from attacking bacteria in the pus, or from reaching the causative organism or foreign object. Diagnosis
An abscess is a localized collection of pus (purulent inflammatory tissue) caused by suppuration buried in a tissue, an organ, or a confined space, lined by the pyogenic membrane. Ultrasound imaging can help in a diagnosis. Classification
Abscesses may be classified as either skin abscesses or internal abscesses. Skin abscesses are common; internal abscesses tend to be harder to diagnose, and more serious. Skin abscesses are also called cutaneous or subcutaneous abscesses. IV drug use
For those with a history of intravenous drug use, an X-ray is recommended before treatment to verify that no needle fragments are present. If there is also a fever present in this population, infectious endocarditis should be considered. Differential
Abscesses should be differentiated from empyemas, which are accumulations of pus in a preexisting, rather than a newly formed, anatomical cavity. | 0-1
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These cells produce a large amount of proteins from a small amount of precursors and so are constantly working and under a reasonable amount of stress. So with a mutation in eIF2B, slight increases in the amount of stress these cells encounter occur, making them more susceptible to failure due to stress. The large amount of oligodendrocytes which display apoptotic characteristics and express apoptotic proteins suggests cell number reduction in the early stages of the disease. Premature ovarian failure has also been associated with diminishing white matter. However through an intensive survey, it was determined that even if an individual has premature ovarian failure, she does not necessarily have VWM. eIF2Bs role
eIF2B is the guanine nucleotide-exchange factor for eIF2, and is composed of 5 subunits. The largest subunit, eIF2B5 contains the most mutations for VWM. eIF2B is a complex which is very involved with the regulation of in the translation of mRNA into proteins. eIF2B is essential for the exchange of guanosine diphosphate(GDP) for guanosine-5-triphosphate(GTP) in the initiation of translation via eIF2, because eIF2 is regenerated through this exchange. A decrease in eIF2B activity has been correlated with the onset of VWM. A common factor among VWM patients is mutations in the five subunits of eIF2B (21 discovered thus far), expressed in over 60% of the patients. These mutations lead to the decreased activity of eIF2B. The most common mutation is R113H, which is the mutation of histidine to arginine. The homozygous form of the mutation is the least severe form. | Diagnosis
Classification
The World Health Organization (WHO) has identified three subtypes of nasopharyngeal carcinoma:
type 1: squamous cell carcinoma, typically found in older adults
type 2: non-keratinizing carcinoma
type 3: undifferentiated carcinomaType 3 is most commonly found among younger children and adolescents, with a few type 2 cases. Both type 2 and 3 have been found to be associated with elevated levels of Epstein-Barr virus titers, but not type 1. Additionally, type 2 and type 3 may be followed with an influx of inflammatory cells, including lymphocytes, plasma cells, and eosinophils, generating the term lymphoepithelioma.Nasopharyngeal carcinoma, also known as nasopharyngeal cancer, is classified as a malignant neoplasm, or cancer, arising from the mucosal epithelium of the nasopharynx, most often within the lateral nasopharyngeal recess or fossa of Rosenmüller (a recess behind the entrance of the eustachian tube opening). The World Health Organization classifies nasopharyngeal carcinoma in three types, in order of frequency: Non-keratinizing squamous cell carcinoma; keratinizing squamous cell carcinoma; and basaloid squamous cell carcinoma. The tumor must show evidence of squamous differentiation, with the non-keratinizing type (also known as lymphoepithelioma) the tumor most strongly associated with Epstein–Barr virus infection of the cancerous cells. Staging
Staging of nasopharyngeal carcinoma is based on clinical and radiologic examination. Most patients present with Stage III or IV disease. Stage I is a small tumor confined to nasopharynx. Stage II is a tumor extending in the local area, or that with any evidence of limited neck (nodal) disease. Stage III is a large tumor with or without neck disease, or a tumor with bilateral neck disease. | 0-1
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Patients with fluid protein <15 g/L and either Child-Pugh score of at least 9 or impaired renal function may also benefit. Previous SBPPeople with cirrhosis admitted to the hospital should receive prophylactic antibiotics if:
They have bleeding esophageal varicesStudies on the use of rifaximin in cirrhotic patients, have suggested that its use may be effective in preventing spontaneous bacterial peritonitis. Treatment
Antibiotics
Although there is no high-quality evidence, the third-generation cephalosporins are considered the standard empirical treatment for spontaneous bacterial peritonitis in people with cirrhosis.In practice, cefotaxime is the agent of choice for treatment of SBP. After confirmation of SBP, hospital admission is usually advised for observation and intravenous antibiotic therapy.Where there is a risk of kidney malfunction developing in a syndrome called hepatorenal syndrome, intravenous albumin is usually administered too. Paracentesis may be repeated after 48 hours to ensure control of infection. After recovery from a single episode of SBP, indefinite prophylactic antibiotics are recommended. Prokinetics
The addition of a prokinetic drug to an antibiotic regimen reduces the incidence of spontaneous bacterial peritonitis possibly via decreasing small intestinal bacterial overgrowth. Intravenous albumin
A randomized controlled trial found that intravenous albumin on the day of admission and on hospital day 3 can reduce kidney impairment. Epidemiology
Patients with ascites underwent routine paracentesis, the incidence of active SBP ranged from 10% to 27% at the time of hospital admission. History
SBP was first described in 1964 by Harold O. Conn.
References
== External links == | Throughout life, the synthesis of the alpha-like and the beta-like (also called non-alpha-like) chains is balanced so that their ratio is relatively constant and there is no excess of either type.The specific alpha and beta-like chains that are incorporated into Hb are highly regulated during development:
Embryonic Hbs are expressed as early as four to six weeks of embryogenesis and disappear around the eighth week of gestation as they are replaced by fetal Hb. Embryonic Hbs include:
Hb Gower-1, composed of two ζ globins (zeta globins) and two ε globins (epsilon globins) (ζ2ε2)
Hb Gower-2, composed of two alpha globins and two epsilon globins (α2ε2)
Hb Portland, composed of two zeta globins and two gamma globins (ζ2γ2)
Fetal Hb (Hb F) is produced from approximately eight weeks of gestation through birth and constitutes approximately 80 percent of Hb in the full-term neonate. It declines during the first few months of life and, in the normal state, constitutes <1 percent of total Hb by early childhood. Hb F is composed of two alpha globins and two gamma globins (α2γ2). Adult Hb (Hb A) is the predominant Hb in children by six months of age and onward; it constitutes 96-97% of total Hb in individuals without a hemoglobinopathy. It is composed of two alpha globins and two beta globins (α2β2). Hb A2 is a minor adult Hb that normally accounts for approximately 2.5-3.5% of total Hb from six months of age onward. It is composed of two alpha globins and two delta globins (α2δ2). | 0-1
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Small breast buds are present on both sexes. Head hair becomes coarse and thicker. Birth is imminent and occurs around the 38th week after fertilization. The fetus is considered full-term between weeks 37 and 40, when it is sufficiently developed for life outside the uterus. It may be 48 to 53 cm (19 to 21 in) in length, when born. Control of movement is limited at birth, and purposeful voluntary movements continue to develop until puberty. Variation in growth
There is much variation in the growth of the human fetus. When fetal size is less than expected, the condition is known as intrauterine growth restriction also called fetal growth restriction; factors affecting fetal growth can be maternal, placental, or fetal.Maternal factors include maternal weight, body mass index, nutritional state, emotional stress, toxin exposure (including tobacco, alcohol, heroin, and other drugs which can also harm the fetus in other ways), and uterine blood flow. Placental factors include size, microstructure (densities and architecture), umbilical blood flow, transporters and binding proteins, nutrient utilization and nutrient production. Fetal factors include the fetal genome, nutrient production, and hormone output. Also, female fetuses tend to weigh less than males, at full term.Fetal growth is often classified as follows: small for gestational age (SGA), appropriate for gestational age (AGA), and large for gestational age (LGA). SGA can result in low birth weight, although premature birth can also result in low birth weight. | Some of the blood moves from the aorta through the internal iliac arteries to the umbilical arteries, and re-enters the placenta, where carbon dioxide and other waste products from the fetus are taken up and enter the mothers circulation.Some of the blood from the right atrium does not enter the left atrium, but enters the right ventricle and is pumped into the pulmonary artery. In the fetus, there is a special connection between the pulmonary artery and the aorta, called the ductus arteriosus, which directs most of this blood away from the lungs (which are not being used for respiration at this point as the fetus is suspended in amniotic fluid). Postnatal development
With the first breath after birth, the system changes suddenly. Pulmonary resistance is reduced dramatically, prompting more blood to move into the pulmonary arteries from the right atrium and ventricle of the heart and less to flow through the foramen ovale into the left atrium. The blood from the lungs travels through the pulmonary veins to the left atrium, producing an increase in pressure that pushes the septum primum against the septum secundum, closing the foramen ovale and completing the separation of the newborns circulatory system into the standard left and right sides. Thereafter, the foramen ovale is known as the fossa ovalis. The ductus arteriosus normally closes within one or two days of birth, leaving the ligamentum arteriosum, while the umbilical vein and ductus venosus usually closes within two to five days after birth, leaving, respectively, the livers ligamentum teres and ligamentum venosus. | 11
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Under Justinian in the 6th century, the eunuch Narses functioned as a successful general in a number of campaigns. Advantages of eunuchs were that they prevented offices from becoming hereditary, allowing appointments to be made on merit; they were more dedicated to their jobs, not being distracted by family obligations; and they were ineligible for the throne, and for that reason thought by emperors to be safe. Those who had been deprived not only of their testicles but also their penises were known in Greek as carzimasia, and were highly prized.By the last centuries of the Empire, the number of roles reserved for eunuchs had reduced, and their use may have been all but over.Following the Byzantine tradition, eunuchs had important tasks at the court of the Norman Kingdom of Sicily during the middle 12th century. One of them, Philip of Mahdia, has been admiratus admiratorum, and another one, Ahmed es-Sikeli, was prime minister. China
In China, castration included removal of the penis as well as the testicles (see emasculation). Both organs were cut off with a knife at the same time.Eunuchs existed in China from about 4,000 years ago, were imperial servants by 3,000 years ago, and were common as civil servants by the time of the Qin dynasty. From those ancient times until the Sui dynasty, castration was both a traditional punishment (one of the Five Punishments) and a means of gaining employment in the Imperial service. | The young mans thighs and abdomen would be tied and others would pin him down on a table. The genitals would be washed with pepper water and then cut off. A tube would be then inserted into the urethra to allow urination during healing. Many Vietnamese eunuchs were products of self castration to gain access to the palaces and power. In other cases they might be paid to become eunuchs. They served in many capacities, from supervising public works, to investigating crimes, to reading public proclamations. Thailand
In Siam (modern Thailand) Indian Muslims from the Coromandel Coast served as eunuchs in the Thai palace and court. The Thai at times asked eunuchs from China to visit the court in Thailand and advise them on court ritual since they held them in high regard. Burma
Sir Henry Yule saw many Muslims serving as eunuchs in the Konbaung Dynasty of Burma (modern Myanmar) while on a diplomatic mission. Arabian Peninsula
For several centuries, Muslim Eunuchs were tasked with honored roles in Medina and Mecca. They are thought to have been instituted in their role there by Salah ad-Deen Ayubi, but perhaps earlier. Their tasks included caring for the Prophet Muhammad’s tomb, maintaining borders between males and females where needed, and keeping order in the sacred spaces. They were highly respected in their time and remained there throughout the Ottoman Empire’s control of the area and afterward. In the present day, it is reported that only a few remain. | 11
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In October 2011, in an effort to reduce the burden of pertussis in infants, the ACIP recommended that unvaccinated pregnant women receive a dose of Tdap. On 24 October 2012, the ACIP voted to recommend the use of Tdap during every pregnancy.The ACIP and Canadas National Advisory Committee on Immunization (NACI) recommended that both adolescents and adults receive Tdap in place of their next Td booster (recommended to be given every ten years). Tdap and Td can be used as prophylaxis for tetanus in wound management. People who will be in contact with young infants are encouraged to get Tdap even if it has been less than five years since Td or TT to reduce the risk of infants being exposed to pertussis. NACI suggests intervals shorter than five years can be used for catch-up programs and other instances where programmatic concerns make five-year intervals difficult.The World Health Organization (WHO) recommends a pentavalent vaccine, combining the DTP vaccine with vaccines against Haemophilus influenzae type B and hepatitis B. Evidence on how effective this pentavalent vaccine is compared to the individual vaccines has not yet been determined.A 2019 study in the American Economic Journal found that state requirements mandating the use of the Tdap vaccine "increased Tdap vaccine take-up and reduced pertussis (whooping cough) incidence by about 32 percent." Related combination vaccines
Excluding Pertussis
DT and Td vaccines, lack the pertussis component. The Td vaccine is administered to children over the age of seven as well as to adults. | In such cases, administration of Tdap is recommended after 20 weeks gestation, and in earlier pregnancy a single dose of Tdap can be substituted for one dose of Td, and then the series completed with Td. For pregnant women not previously vaccinated with Tdap, if Tdap is not administered during pregnancy, it should be administered immediately postpartum. Postpartum administration of TDaP is not equivalent to administration of the vaccination during pregnancy. Because the vaccine is administered postpartum, the mother is unable to develop antibodies that can be transferred to the infant in utero, consequently, leaving the infant vulnerable to the diseases preventable by the Tdap Vaccine. Postpartum administration of the TdaP vaccine to the mother seeks to reduce the likelihood that the mother will contract disease that can be subsequently passed on the infant, albeit there will still be a two week period prior to the protective effects of the vaccine setting in. Postpartum administration is an extension of the concept of "cocooning," a term that refers to the full vaccination of all individuals that may come into direct contact with the infant. Cocooning, like postpartum Tdap aministration, is not recommended by the CDC. Cocooning depends on ensuring full vaccination of all individuals that the infant may come into contact with, and there may be financial, administrative or personal barriers that preclude full and timely vaccination of all individuals within the "cocoon." Brand names
Australia
United Kingdom
Brand names in the United Kingdom include Revaxis (Sanofi Pasteur). | 11
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The authors of a study on the bubonic plague in Oran stress that the disease "is primarily a bacterial zoonosis affecting rodents. It is caused by Yersinia pestis and is transmitted from animal to animal by fleas. Humans usually become infected through the bite of an infected rodent flea." The sanitary control measure instituted by the public health authority was chemical in nature: "Intra- and peridomestic spraying with permethrin was conducted. Deltamethrin was dusted on the tracks and around the burrows of rodents located in a radius of 10 km around the dwelling of the patients. Uncontrolled killing of rats was prohibited. "A large proportion of viral pathogens that have emerged recently in humans are considered to have originated from various animal species. This is shown by several recent epidemics such as, avian flu, Ebola, monkeypox, and Hanta viruses. There is evidence to suggest that some diseases can potentially be re-introduced to human populations through animal hosts after they have been eradicated in humans. There is a risk of this phenomenon occurring with morbilliviruses as they can readily cross species barriers. CST can also have a significant effect on produce industries. Genotype VI-Avian paramyxovirus serotype 1 (GVI-PMV1) is a virus that arose through cross-species transmission events from Galliformes (i.e. chicken) to Columbiformes, and has become prevalent in the poultry industry.CST of rabies virus variants between many different species populations is a major concern of wildlife management. | Models for CST prediction will vary depending on what parameters need to be accounted for when constructing the model. Most parsimonious reconstruction (MPR)
Parsimony is the principle in which one chooses the simplest scientific explanation that fits the evidence. In terms of building phylogenetic trees, the best hypothesis is the one that requires the fewest evolutionary changes. Using parsimony to reconstruct ancestral character states on a phylogenetic tree is a method for testing ecological and evolutionary hypotheses. This method can be used in CST studies to estimate the number of character changes that exist between pathogens in relation to their host. This makes MPR useful for tracking a CST pathogen to its origins. MPR can also be used to the compare traits of host species populations. Traits and behaviours within a population could make them more susceptible to CST. For example, species which migrate regionally are important for spreading viruses through population networks.Despite the success of parsimony reconstructions, research suggests they are often sensitive and can sometimes be prone to bias in complex models. This can cause problems for CST models that have to consider many variables. Alternatives methods, such as maximum likelihood, have been developed as an alternative to parsimony reconstruction. Using genetic markers
Two methods of measuring genetic variation, variable number tandem repeats (VNTRs) and single nucleotide polymorphisms (SNPs), have been very beneficial to the study of bacterial transmission. | 11
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As expected, with cardiac damage and dysfunction, there can be an elevation of these markers in patients with cardiac amyloidosis. These markers have been incorporated into the various staging/scoring systems used by physicians to determine severity of the disease and prognosis. Biopsies
Extracardiac biopsies of tissues of the kidney, liver, peripheral nerve, or abdominal fat can be used to confirm the presence of amyloid deposits. Amyloid deposits in biopsy samples are confirmed through the use of Congo red dye, which produces a green birefringence when viewed under polarized light. Sirius red staining or electron microscopy examination can also be done. The determination of the type of amyloid can be done by immunohisto-labeling techniques as well as immunofluorescence staining.For light-chain amyloidosis patients, bone marrow biopsies could be conducted to determine the baseline percentage of plasma cells and to rule out multiple myeloma. Catheterization
Right heart catheterization is the test used to test for elevated diastolic ventricular pressures. This test is more invasive and would be performed after inconclusive endomyocardial biopsy samples. Cardiac magnetic resonance imaging
Cardiac magnetic resonance (CMR) is capable of measuring the thickness of different areas of the heart. This can be used for quantification of the deposits in the heart. CMR also shows the characterization of myocardial tissue through patterns of gadolinium enhancements. However, none of the CMR technique is able to differentiate ATTR-CM and AL-CM definitely.For AL-CM, 68% of them have symmetrical and concentric left ventricular hypertrophy. | Staging systems have been developed to stratify severity of the disease, including the Mayo Biomarker Stage, which utilizes various biomarkers such as troponin I, troponin T, BNP, and NT-proBNP, and Free light chain concentrations.Familial (ATTRm-CM) Prognosis: Due to the extensive number of variables involved in this subtype, prognosis varies depending on the specific type of familial cardiac amyloidosis. Variables involve mutant vs wild type transthyretin mutation and age of onset of symptoms. In comparison to light chain amyloidosis, the familial subtype is slower to progress and has a more favorable prognosis. However, the Val 122lle mutation (most common cause of familial cardiac amyloidosis) has a 4-year survival rate of 16% with an average length of 26 months. A delay in recognition plays a major factor in this reduced survival rate. == References == | 11
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an acuity of 6/60 (20/200) or worse, and often leave a person with complete blindness. Non-genetic causes
Physical trauma can cause color blindness, either neurologically – brain trauma which produces swelling of the brain in the occipital lobe – or retinally, either acute (e.g. from laser exposure) or chronic (e.g. from ultraviolet light exposure). Color blindness may also present itself as a symptom of degenerative diseases of the eye, such as cataract and age-related macular degeneration, and as part of the retinal damage caused by diabetes. Vitamin A deficiency may also cause color blindness.Color blindness may be a side effect of prescription drug use. For example, red–green color blindness can be caused by ethambutol, a drug used in the treatment of tuberculosis. Blue-yellow color blindness can be caused by sildenafil, an active component of Viagra. Hydroxychloroquine can also lead to hydroxychloroquine retinopathy, which includes various color defects. Exposure to chemicals such as styrene or organic solvents can also lead to color vision defects. Mechanism
Color blindness is any deviation of color vision from normal trichromatic color vision (often as defined by the standard observer) that produces a reduced gamut. Mechanisms for color blindness are related to the functionality of cone cells, and often to the expression of photopsins, the photopigments that catch photons and thereby convert light into chemical signals. When an individual does not satisfy the requirements for trichromatic vision, they will express dichromacy or monochromacy and be colorblind. | It is now qualified as a condition that could potentially compromise driver safety, therefore a driver may have to be evaluated by an authorized ophthalmologist to determine if they can drive safely. As of May 2008, there is an ongoing campaign to remove the legal restrictions that prohibit colorblind citizens from getting drivers licenses. In June 2020, India relaxed its ban on drivers licenses for the colorblind to now only apply to those with strong CVD. While previously restricted, those who test as mild or moderate can now pass the medical requirements. Australia instituted a tiered ban on the colorblind from obtaining commercial drivers licenses in 1994. This included a ban for all protans, and a stipulation that deutans must pass the Farnsworth Lantern. The stipulation on deutans was revoked in 1997 citing a lack of available test facilities, and the ban on protans was revoked in 2003. All colorblind individuals are banned from obtaining a drivers license in China and since 2016 in Russia (2012 for dichromats). There are several features available that help the colorblind compensate for their color vision deficiency:
British Rail signals use more easily identifiable colors: the red is blood red, the amber is yellow and the green is a bluish color. The relative position of traffic lights is fixed internationally as red, amber, green from top to bottom. Horizontal lights will differ depending on the country, but right hand traffic typically follows a "red light always on the left" pattern. | 11
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Mourshed showed that 33% of ameloblastomas arose from the epithelial lining of a dentigerous cyst. Although undeniably this can occur, the frequency of such neoplastic transformation is low. In addition, a squamous cell carcinoma may rarely arise in the lining of a dentigerous cyst. Transformation from normal epithelial cyst lining to SCC is due to chronic inflammation. It is likely that some intraosseous mucoepidermoid carcinomas develop from mucous cells in the lining of a dentigerous cyst. Malignancy in the cyst wall is usually unexpected at the time of presentation and the diagnosis is usually made following enucleation. Jagged or irregular margins with indentations and indistinct borders are considered to be suggestive of possible malignant change. Due to the potential for occurrence of an odontogenic keratocyst or the development of an ameloblastoma or, more rarely, mucoepidermoid carcinoma, all such lesions, when excised, should be submitted for histopathologic evaluation. Differential Diagnosis
The differential diagnoses of dentigerous cysts are as follows:
Radicular cystIt is an odontogenic cyst that is a sequela of periapical granuloma in a carious tooth. Odontogenic keratocyst(OKC)It is often multilocular and most commonly located in the body or the ramus of the mandible.Histologically, the epithelium is uniform in nature, usually four to eight cells in thickness. The basilar layer consists of a palisaded row of cuboidal to columnar cells that may demonstrate hyperchromatism. Characteristically, a corrugated or wavy layer of parakeratin is produced on the epithelial surface and desquamated keratin may be present in the cyst lumen. | Some dentigerous cysts may result in considerable displacement of the involved tooth. Infrequently, a third molar may be displaced to the lower border of the mandible or into the ascending ramus. On the other hand, maxillary anterior teeth may be displaced into the floor of the nasal cavity, while other maxillary teeth may be displaced through the maxillary sinus to the floor of the orbit. Furthermore, larger cysts can lead to resorption of adjacent unerupted teeth. Some dentigerous cysts may also grow to considerable size and produce bony expansion that is usually painless, unless secondarily infected. However, any particularly large dentigerous radiolucency should clinically be suspected of a more aggressive odontogenic lesion such as an odontogenic keratocyst or ameloblastoma. For this reason, biopsy is mandated for all significant pericoronal radiolucencies to confirm the diagnosis. The role of CT (computerized tomography) imaging in the evaluation of cystic lesions has been well-documented. CT imaging aids to rule out solid and fibro-osseous lesions, displays bony detail, and provides precise information about the size, origin, content, and relationships of the lesions.On CT imaging, a mandibular dentigerous cyst appears as a well-circumscribed unilocular area of osteolysis that incorporates the crown of a tooth. Displacement of adjacent teeth may be seen and they may be partly eroded. Dentigerous cysts in the maxilla often extend into the antrum, displacing and remodeling the bony sinus wall. Large cysts which may project into the nasal cavity or infratemporal fossa and may elevate the floor of the orbit can be noted on CT imaging. | 11
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There is probably no single reference that lists all the pharmaceutical variations of laudanum that were created and used in different countries during centuries since it was initially formulated. The reasons are that in addition to official variations described in pharmacopeias, pharmacists and drug manufacturers were free to alter such formulas. The alcohol content of Laudanum probably varied substantially; on the labels of turn-of-the-century bottles of Laudanum, alcoholic content is stated as 48%. In contrast, the current version of Laudanum contains about 18% alcohol. The four variations of laudanum listed here were used in the United States during the late 19th century. The first, from an 1870 publication, is "Best Turkey opium 1 oz., slice, and pour upon it boiling water 1 gill, and work it in a bowl or mortar until it is dissolved; then pour it into the bottle, and with alcohol of 70 percent proof 1⁄2 pt., rinse the dish, adding the alcohol to the preparation, shaking well, and in 24 hours it will be ready for use. Dose—From 10 to 30 drops for adults, according to the strength of the patient, or severity of the pain. Thirty drops of this laudanum will be equal to one grain of opium. And this is a much better way to prepare it than putting the opium into alcohol, or any other spirits alone, for in that case much of the opium does not dissolve." | Dryness in aqueous tear deficiency dry eye patients tends to be worse as the day progresses due to progressive exposure. Frequent blinking exacerbates mechanical dry eye symptoms, while increasing blinking improves symptoms of aqueous tear deficiency dry eye. Diagnosis can be further made under a slit lamp upon the observation of redundant conjunctival folds. These folds can be made more apparent by staining with fluorescein dye and by applying gentle upward pressure with a finger to the eyeball through the lower lid. In pure aqueous tear deficiency dry eye patients, fluorescein stains in the interpalpebral exposure zone. In mechanical dry eye patients, fluorescein staining can be seen by pulling down the lower lid spread to the non-exposure zone. A tear-clearance test can also detect irregularities in the tear film.Diagnosis can also be made by measuring the replenishment of the tear meniscus. The test can be done as follows: Apply 5 μl of fluorescein to the base of the inferior fornix, maximally deplete the lower tear meniscus by a capillary tube or Weckcel sponge, and then monitor the recovery of the tear meniscus height with or without blinking. Patients with mechanical dry eye detectable within 3–8 seconds without blinking or within 0.5-1.5 seconds with blinking. Treatment
Mild Conjunctivochalasis can be asymptomatic and in such cases does not require treatment. Lubricating eye drops may be tried but are often ineffective.If discomfort persists after standard dry eye treatment and anti-inflammatory therapy, surgery may be undertaken to remove the conjunctival folds and restore a smooth tear film. | 0-1
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The endothelial cells produce cytokines and antimicrobial peptides against the bacteria. These products regulate the coagulation cascade and movements of white blood cells. Macrophages presented in humans are able to engulf Leptospira. However, Leptospira are able to reside and proliferate in the cytoplasmic matrix after being ingested by macrophages. Those with severe leptospirosis can experience a high level of cytokines such as interleukin 6, tumor necrosis factor alpha (TNF-α), and interleukin 10. The high level of cytokines causes sepsis-like symptoms which is life-threatening instead of helping to fight against the infection. Those who have a high risk of sepsis during a leptospirosis infection are found to have the HLA-DQ6 genotype, possibly due to superantigen activation, which damages bodily organs.Leptospira LPS only activates toll-like receptor 2 (TLR2) in monocytes in humans. The lipid A molecule of the bacteria is not recognised by human TLR4 receptors. Therefore, the lack of Leptospira recognition by TLR4 receptors probably contributes to the leptospirosis disease process in humans.Although there are various mechanisms in the human body to fight against the bacteria, Leptospira is well adapted to such an inflammatory condition created by it. In the bloodstream, it can activate host plasminogen to become plasmin that breaks down extracellular matrix, degrades fibrin clots and complemental proteins (C3b and C5) to avoid opsonisation. It can also recruit complement regulators such as Factor H, C4b-binding protein, factor H-like binding protein, and vitronectin to prevent the activation of membrane attack complex on its surface. It also secretes proteases to degrade complement proteins such as C3. | For those with signs of inflammation of membranes covering the brain and the brain itself, altered level of consciousness can happen. A variety of neurological problems such as paralysis of half of the body, complete inflammation of a whole horizontal section of spinal cord, and muscle weakness due to immune damage of the nerves supplying the muscles are the complications. Signs of bleeding such as non-traumatic bruises at 1 mm (0.039 in), non-traumatic bruises more than 1 cm (0.39 in), nose bleeding, blackish stools due to bleeding in the stomach, vomiting blood and bleeding from the lungs can also be found. Prolongation of prothrombin time in coagulation testing is associated with severe bleeding manifestation. However, low platelet count is not associated with severe bleeding. Pulmonary haemorrhage is alveolar haemorrhage (bleeding into the alveoli of the lungs) leading to massive coughing up of blood, and causing acute respiratory distress syndrome, where the risk of death is more than 50%. Rarely, inflammation of the heart muscles, inflammation of membranes covering the heart, abnormalities in the hearts natural pacemaker and abnormal heart rhythms may occur. Cause
Bacteria
Leptospirosis is caused by spirochaete bacteria that belong to the genus Leptospira, which are aerobic, right-handed helical, and 6–20 micrometers long. Like Gram-negative bacteria, Leptospira have an outer membrane studded with lipopolysaccharide (LPS) on the surface, an inner membrane and a layer of peptidoglycan cell wall. However, unlike Gram-negative bacteria, the peptidoglycan layer in Leptospira lies closer to the inner than the outer membrane. | 11
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Urban epidemics continued in the United States until 1905, with the last outbreak affecting New Orleans.At least 25 major outbreaks took place in the Americas during the 18th and 19th centuries, including particularly serious ones in Cartagena, Chile, in 1741; Cuba in 1762 and 1900; Santo Domingo in 1803; and Memphis, Tennessee, in 1878.In the early nineteenth century, the prevalence of yellow fever in the Caribbean "led to serious health problems" and alarmed the United States Navy as numerous deaths and sickness curtailed naval operations and destroyed morale. One episode began in April 1822 when the frigate USS Macedonian left Boston and became part of Commodore James Biddles West India Squadron. Unbeknownst to all, they were about to embark on a cruise to disaster and their assignment "would prove a cruise through hell". Secretary of the Navy Smith Thompson had assigned the squadron to guard United States merchant shipping and suppress piracy. During their time on deployment from 26 May to 3 August 1822, 76 of the Macedonians officers and men died, including John Cadle, surgeon USN. Seventy-four of these deaths were attributed to yellow fever. Biddle reported that another 52 of his crew were on sick-list. In their report to the secretary of the Navy, Biddle and Surgeons Mate Charles Chase stated the cause as "fever". As a consequence of this loss, Biddle noted that his squadron was forced to return to Norfolk Navy Yard early. Upon arrival, the Macedonians crew were provided medical care and quarantined at Craney Island, Virginia. | The virus, as well as the vector A. aegypti, were probably transferred to North and South America with the trafficking of slaves from Africa, part of the Columbian exchange following European exploration and colonization.The first definitive outbreak of yellow fever in the New World was in 1647 on the island of Barbados. An outbreak was recorded by Spanish colonists in 1648 in the Yucatán Peninsula, where the indigenous Mayan people called the illness xekik ("blood vomit"). In 1685, Brazil suffered its first epidemic in Recife. The first mention of the disease by the name "yellow fever" occurred in 1744. (John Mitchell) (1805) (Mitchells account of the Yellow Fever in Virginia in 1741–2) Archived 2017-02-23 at the Wayback Machine, The Philadelphia Medical Museum, 1 (1) : 1–20. (John Mitchell) (1814) "Account of the Yellow fever which prevailed in Virginia in the years 1737, 1741, and 1742, in a letter to the late Cadwallader Colden, Esq. of New York, from the late John Mitchell, M.D.F.R.S. of Virginia," Archived 2017-02-23 at the Wayback Machine American Medical and Philosophical Register, 4 : 181–215. The term "yellow fever" appears on p. 186. On p. 188, Mitchell mentions "… the distemper was what is generally called yellow fever in America". However, on pages 191–192, he states "… I shall consider the cause of the yellowness which is so remarkable in this distemper, as to have given it the name of the Yellow Fever. | 11
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His description is as follows: "The ventricular muscle is thrown into a state of irregular arrhythmic contraction, whilst there is a great fall in the arterial blood pressure, the ventricles become dilated with blood as the rapid quivering movement of their walls is insufficient to expel their contents; the muscular action partakes of the nature of a rapid incoordinate twitching of the muscular tissue … The cardiac pump is thrown out of gear, and the last of its vital energy is dissipated in the violent and the prolonged turmoil of fruitless activity in the ventricular walls." MacWilliam spent many years working on ventricular fibrillation and was one of the first to show that ventricular fibrillation could be terminated by a series of induction shocks through the heart.The first electrocardiogram recording of ventricular fibrillation was by August Hoffman in a paper published in 1912. At this time, two other researchers, George Ralph Mines and Garrey, working separately, produced work demonstrating the phenomenon of circus movement and re-entry as possible substrates for the generation of arrhythmias. This work was also accompanied by Lewis, who performed further outstanding work into the concept of "circus movement. "Later milestones include the work by W. J. Kerr and W. L. Bender in 1922, who produced an electrocardiogram showing ventricular tachycardia evolving into ventricular fibrillation. The re-entry mechanism was also advocated by DeBoer, who showed that ventricular fibrillation could be induced in late systole with a single shock to a frog heart. | Ventricular fibrillation (V-fib or VF) is an abnormal heart rhythm in which the ventricles of the heart quiver. It is due to disorganized electrical activity. Ventricular fibrillation results in cardiac arrest with loss of consciousness and no pulse. This is followed by sudden cardiac death in the absence of treatment. Ventricular fibrillation is initially found in about 10% of people with cardiac arrest.Ventricular fibrillation can occur due to coronary heart disease, valvular heart disease, cardiomyopathy, Brugada syndrome, long QT syndrome, electric shock, or intracranial hemorrhage. Diagnosis is by an electrocardiogram (ECG) showing irregular unformed QRS complexes without any clear P waves. An important differential diagnosis is torsades de pointes.Treatment is with cardiopulmonary resuscitation (CPR) and defibrillation. Biphasic defibrillation may be better than monophasic. The medication epinephrine or amiodarone may be given if initial treatments are not effective. Rates of survival among those who are out of hospital when the arrhythmia is detected is about 17% while in hospital it is about 46%. Signs and symptoms
Ventricular fibrillation is a cause of cardiac arrest. The ventricular muscle twitches randomly rather than contracting in a co-ordinated fashion (from the apex of the heart to the outflow of the ventricles), and so the ventricles fail to pump blood around the body – because of this, it is classified as a cardiac arrest rhythm, and patients in V-fib should be treated with cardiopulmonary resuscitation (CPR) and prompt defibrillation. | 11
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Finally, a biopsy of a skin lesion can be performed to rule out any other causes.The immunohistochemical features are very similar to those presented in mycosis fungoides except for the following differences:
More monotonous cellular infiltrates (large, clustered atypical pagetoid cells) in Sézary syndrome
Sometimes absent epidermotropism
Increased lymph node involvement with infiltrates of Sézary syndrome. Treatment
Treatment typically includes some combination of photodynamic therapy, radiation therapy, chemotherapy, and biologic therapy.Treatments are often used in combination with phototherapy and chemotherapy, though pure chemotherapy is rarely used today. No single treatment type has revealed clear-cut benefits in comparison to others, treatment for all cases remains problematic. Radiation therapy
A number of types of radiation therapy may be used including total skin electron therapy. While this therapy does not generally result in systemic toxic effects it can produce side effects involving the skin. It is only available at a few institutions. Chemotherapy
Romidepsin, vorinostat and a few others are a second-line drug for cutaneous T-cell lymphoma. Mogamulizumab has been approved in Japan and the United States. Epidemiology
In the Western population, there are around 3 cases of Sézary syndrome per 1,000,000 people. Sézary disease is more common in males with a ratio of 2:1, and the mean age of diagnosis is between 55 and 60 years of age. See also
List of cutaneous conditions
References
External links
Sezary Syndrome lymphoma information | excision of the tumor’s protruding portion followed by treatment of the proximal perionych [i.e., skin around a nail] with phenol to eradicate the root of the tumor); and in individuals with tuberous sclerosis, topical application of sirolimus, i.e. rapamycin, (1% solution). Some of the latter methods have been used in order to preserve the nail matrix and nailplate and may be ideal for younger patients with few tumors since they may leave a normal appearing nail. See also
List of cutaneous conditions
Nail anatomy
== References == | 0-1
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In developed countries, obesity, alcoholism, and drug use are common causes of poor immune function, while malnutrition is the most common cause of immunodeficiency in developing countries. Diets lacking sufficient protein are associated with impaired cell-mediated immunity, complement activity, phagocyte function, IgA antibody concentrations, and cytokine production. Additionally, the loss of the thymus at an early age through genetic mutation or surgical removal results in severe immunodeficiency and a high susceptibility to infection. Immunodeficiencies can also be inherited or acquired. Severe combined immunodeficiency is a rare genetic disorder characterized by the disturbed development of functional T cells and B cells caused by numerous genetic mutations. Chronic granulomatous disease, where phagocytes have a reduced ability to destroy pathogens, is an example of an inherited, or congenital, immunodeficiency. AIDS and some types of cancer cause acquired immunodeficiency. Autoimmunity
Overactive immune responses form the other end of immune dysfunction, particularly the autoimmune disorders. Here, the immune system fails to properly distinguish between self and non-self, and attacks part of the body. Under normal circumstances, many T cells and antibodies react with "self" peptides. One of the functions of specialized cells (located in the thymus and bone marrow) is to present young lymphocytes with self antigens produced throughout the body and to eliminate those cells that recognize self-antigens, preventing autoimmunity. Common autoimmune diseases include Hashimotos thyroiditis, rheumatoid arthritis, diabetes mellitus type 1, and systemic lupus erythematosus. Hypersensitivity
Hypersensitivity is an immune response that damages the bodys own tissues. | One class of non-self molecules are called antigens (originally named for being antibody generators) and are defined as substances that bind to specific immune receptors and elicit an immune response. Surface barriers
Several barriers protect organisms from infection, including mechanical, chemical, and biological barriers. The waxy cuticle of most leaves, the exoskeleton of insects, the shells and membranes of externally deposited eggs, and skin are examples of mechanical barriers that are the first line of defense against infection. Organisms cannot be completely sealed from their environments, so systems act to protect body openings such as the lungs, intestines, and the genitourinary tract. In the lungs, coughing and sneezing mechanically eject pathogens and other irritants from the respiratory tract. The flushing action of tears and urine also mechanically expels pathogens, while mucus secreted by the respiratory and gastrointestinal tract serves to trap and entangle microorganisms.Chemical barriers also protect against infection. The skin and respiratory tract secrete antimicrobial peptides such as the β-defensins. Enzymes such as lysozyme and phospholipase A2 in saliva, tears, and breast milk are also antibacterials. Vaginal secretions serve as a chemical barrier following menarche, when they become slightly acidic, while semen contains defensins and zinc to kill pathogens. In the stomach, gastric acid serves as a chemical defense against ingested pathogens.Within the genitourinary and gastrointestinal tracts, commensal flora serve as biological barriers by competing with pathogenic bacteria for food and space and, in some cases, changing the conditions in their environment, such as pH or available iron. | 11
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Wilsons disease is a genetic disorder in which excess copper builds up in the body. Symptoms are typically related to the brain and liver. Liver-related symptoms include vomiting, weakness, fluid build up in the abdomen, swelling of the legs, yellowish skin and itchiness. Brain-related symptoms include tremors, muscle stiffness, trouble in speaking, personality changes, anxiety, and psychosis.Wilsons disease is caused by a mutation in the Wilson disease protein (ATP7B) gene. This protein transports excess copper into bile, where it is excreted in waste products. The condition is autosomal recessive; for a person to be affected, they must inherit a mutated copy of the gene from both parents. Diagnosis may be difficult and often involves a combination of blood tests, urine tests and a liver biopsy. Genetic testing may be used to screen family members of those affected.Wilsons disease is typically treated with dietary changes and medication. Dietary changes involve eating a low-copper diet and not using copper cookware. Medications used include chelating agents such as trientine and d-penicillamine and zinc supplements. Complications of Wilsons disease can include liver failure, liver cancer and kidney problems. A liver transplant may be helpful to those for whom other treatments are not effective or if liver failure occurs.Wilsons disease occurs in about 1 in 30,000 people. Symptoms usually begin between the ages of 5 and 35 years. It was first described in 1854 by a German pathologist Friedrich Theodor von Frerichs and is named after British neurologist Samuel Wilson. | Kidneys: renal tubular acidosis (Type 2), a disorder of bicarbonate handling by the proximal tubules leads to nephrocalcinosis (calcium accumulation in the kidneys), a weakening of bones (due to calcium and phosphate loss), and occasionally aminoaciduria (loss of essential amino acids needed for protein synthesis). Heart: cardiomyopathy (weakness of the heart muscle) is a rare but recognized problem in Wilsons disease; it may lead to heart failure (fluid accumulation due to decreased pump function) and cardiac arrhythmias (episodes of irregular and/or abnormally fast or slow heart beat). Hormones: hypoparathyroidism (failure of the parathyroid glands leading to low calcium levels), infertility, and recurrent miscarriage. Genetics
The Wilsons disease gene (ATP7B) is on chromosome 13 (13q14.3) and is expressed primarily in the liver, kidney, and placenta. The gene codes for a P-type (cation transport enzyme) ATPase that transports copper into bile and incorporates it into ceruloplasmin. Mutations can be detected in 90% of cases. Most (60%) are homozygous for ATP7B mutations (two abnormal copies), and 30% have only one abnormal copy. Ten percent have no detectable mutation.Although 300 mutations of ATP7B have been described, in most populations the cases of Wilsons disease are due to a small number of mutations specific for that population. For instance, in Western populations the H1069Q mutation (replacement of a histidine by a glutamine at position 1069 in the protein) is present in 37–63% of cases, while in China this mutation is very uncommon and R778L (arginine to leucine at 778) is found more often. | 11
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Sex assignment
The decision of whether to raise an individual with PAIS as a boy or a girl may not be obvious; grades 3 and 4 in particular present with a phenotype that may be difficult to classify as primarily male or female, and some will be incapable of virilization at puberty. Parents of an affected newborn should seek immediate help at a center with an experienced multidisciplinary team, and should avoid gender assignment beforehand. Older guidelines from 2006 advised against waiting for the child to decide for themselves. According to them, key considerations involved in assigning gender include the appearance of the genitalia, the extent to which the child can virilize at puberty, surgical options and the postoperative sexual function of the genitalia, genitoplasty complexity, potential for fertility, and the projected gender identity of the child. The majority of individuals with PAIS are raised male. More recently, the interests of intersex people themselves are being taken into consideration by the medical community. Some parents have pushed their children with intersex variations to display gender normative roles and behaviours, or to engage in hormonal and surgical interventions to make their bodies appear more aesthetically normative. Research based on interviews of people with intersex variations indicate a need for more family protection from intervention and more family support. | These effects may not manifest at all in men with AIS, or might only manifest at a much higher concentration of testosterone, depending on the degree of androgen insensitivity. Those undergoing high dose androgen therapy should be monitored for safety and efficacy of treatment, possibly including regular breast and prostate examinations. Some individuals with PAIS have a sufficiently high sperm count to father children; at least one case report has been published that describes fertile men who fit the criteria for grade 2 PAIS (micropenis, penile hypospadias, and gynecomastia). Several publications have indicated that testosterone treatment can correct low sperm counts in men with MAIS. At least one case report has been published that documents the efficacy of treating a low sperm-count with tamoxifen in an individual with PAIS. Counseling
Depending on phenotypic features, impotence and other sexual problems such as anejaculation or sexual aversion may be fairly common among individuals with PAIS, but do not necessarily indicate low libido. Support groups for individuals with PAIS may help affected individuals discuss their concerns more comfortably. Some individuals with PAIS may try to avoid intimate relationships out of fear of rejection; individual therapy may help some to overcome social anxiety, and restore focus to interpersonal relationships instead of solely on sexual function and activity. Society and culture
Adults with partial androgen insensitivity syndrome include Australian-Maltese advocate Tony Briffa, considered to be the worlds first openly intersex mayor and public office-bearer. | 11
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Reinstatement of the AVIP (2006-2016)
On October 16, 2006, the DoD announced the reinstatement of mandatory anthrax vaccinations for more than 200,000 troops and defense contractors. (Another lawsuit was filed by the same attorneys as before, challenging the basis of the vaccines license on scientific grounds.) The reinstated policy required vaccinations for most military units and civilian contractors assigned to homeland bioterrorism defense or deployed in Iraq, Afghanistan or South Korea. A modification of previous policy allowed military personnel no longer deployed to higher threat areas to receive follow up doses and booster shots on a voluntary basis. As of June 2008, over 8 million doses of AVA had been administered to over 2 million US military personnel as part of the AVIP. In December 2008, the FDA approved the new BioThrax IM formulation for intramuscular injections thus reducing the immunization schedule from a total of 6 shots to 5 shots (see below). On February 12, 2009, Emergent BioSolutions announced that the Drugs Controller General of India (DGCI) had approved licensing of BioThrax for distribution by Biological E. of Hyderabad.In 2011, BioThrax was approved for sale in Singapore by the Singapore Health Sciences Authority. Building 55 approval (2016)
The FDA approved the companys license (officially called a supplemental Biologics License Application, or sBLA) to manufacture BioThrax in a large building in Lansing, Michigan known as "Building 55." According to Homeland Preparedness News, "The use of Building 55 to manufacture BioThrax could expand manufacturing capacity to an estimated 20 to 25 million doses annually. | Hundreds of service members were compelled to leave the military (some of them court-martialed) for resisting the inoculations during the first six years of the program. Adverse events following AVA administration were assessed in several studies conducted by the DoD in the context of the routine anthrax vaccination program. Between 1998 and 2000, at U.S. Forces, Korea, data were collected at the time of anthrax vaccination from 4,348 service personnel regarding adverse events experienced from a previous dose of anthrax vaccine. Most reported events were localized, minor, and self-limited. After the first or second dose, 1.9% reported limitations in work performance or had been placed on limited duty. Only 0.3% reported >1 day lost from work; 0.5% consulted a clinic for evaluation; and one person (0.02%) required hospitalization for an injection-site reaction. Adverse events were reported more commonly among women than among men. A second study, also between 1998 and 2000, at Tripler Army Medical Center, Hawaii, assessed adverse events among 603 military health-care workers. Rates of events that resulted in seeking medical advice or taking time off work were 7.9% after the first dose; 5.1% after the second dose; 3.0% after the third dose; and 3.1% after the fourth dose. Events most commonly reported included muscle or joint aches, headache, and fatigue. | 11
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Enema as a scientific medical treatment and colon cleansing as alternative medical treatment became more common in medical practice.Since the 1700s in the West there has been some popular thought that people with constipation have some moral failing with gluttony or laziness. Special populations
Children
Approximately 3% of children have constipation, with girls and boys being equally affected. With constipation accounting for approximately 5% of general pediatrician visits and 25% of pediatric gastroenterologist visits, the symptom carries a significant financial impact upon the healthcare system. While it is difficult to assess an exact age at which constipation most commonly arises, children frequently experience constipation in conjunction with life-changes. Examples include: toilet training, starting or transferring to a new school, and changes in diet. Especially in infants, changes in formula or transitioning from breast milk to formula can cause constipation. The majority of constipation cases are not tied to a medical disease, and treatment can be focused on simply relieving the symptoms. Postpartum women
The six-week period after pregnancy is called the postpartum stage. During this time, women are at increased risk of being constipated. Multiple studies estimate the prevalence of constipation to be around 25% during the first 3 months. Constipation can cause discomfort for women, as they are still recovering from the delivery process especially if they have had a perineal tear or underwent an episiotomy. | Risk factors that increase the risk of constipation in this population include:
Damage to the levator ani muscles (pelvic floor muscles) during childbirth
Forceps-assisted delivery
Lengthy second stage of labor
Delivering a large child
HemorrhoidsHemorrhoids are common in pregnancy and also may get exacerbated when constipated. Anything that can cause pain with stooling (hemorrhoids, perineal tear, episiotomy) can lead to constipation because patients may withhold from having a bowel movement so as to avoid pain.The pelvic floor muscles play an important role in helping pass a bowel movement. Injury to those muscles by some of the above risk factors (examples- delivering a large child, lengthy second stage of labor, forceps delivery) can result in constipation. Enemas may be administered during labor and these can also alter bowel movements in the days after giving birth. However, there is insufficient evidence to make conclusions about the effectiveness and safety of laxatives in this group of people. See also
Obstructed defecation
Rectal tenesmus
References
External links
09-129b. at Merck Manual of Diagnosis and Therapy Home Edition
Constipation - Introduction (UK NHS site)
MedlinePlus Overview constipation
Constipation Guideline - the World Gastroenterology Organisation (WGO) | 11
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In the same study, adding the further dietary elements of curcumin or caffeic acid to the rats high fat (30% beef tallow) diet reduced the deoxycholate in their feces to levels comparable to levels seen in the rats on a low fat diet. Curcumin is a component of the spice turmeric, and caffeic acid is a component high in some fruits and spices. Caffeic acid is also a digestive break-down product of chlorogenic acid, high in coffee and some fruits and vegetables.In addition to fats, the type or amount of protein in the diet may also affect bile acid levels. Switching from a diet with protein provided by casein to a diet with protein provided by salmon protein hydrolysate led to as much as a 6-fold increase in levels of bile acids in the blood plasma of rats. In humans, adding high protein to a high fat diet raised the level of deoxycholate in the plasma by almost 50%.Obesity has been linked to cancer, and this link is in part through deoxycholate. In obese people, the relative proportion of Firmicutes (Gram-positive bacteria) in gut microbiota is increased resulting in greater conversion of the non-genotoxic primary bile acid, cholic acid, to carcinogenic deoxycholate.Exercise decreases deoxycholate in the colon. Humans whose level of physical activity placed them in the top third had a 17% decrease in fecal bile acid concentration compared to those whose level of physical activity placed them in the lowest third. | Fumarylacetoacetate accumulates in hepatocytes and proximal renal tubal cells and causes oxidative damage and DNA damage leading to cell death and dysfunctional gene expression which alters metabolic processes like protein synthesis and gluconeogenesis. The increase in fumarylacetoacetate inhibits previous steps in tyrosine degradation leading to an accumulation of tyrosine in the body. Tyrosine is not directly toxic to the liver or kidneys but causes dermatologic and neurodevelopmental problems. Tyrosine metabolic pathway
Fumarylacetoacetate hydrolase (FAH) is the final enzyme in the tyrosine metabolic pathway. The mutation of FAH enzyme results in nonfunctional FAH in all cells expressing this gene and thus metabolizing tyrosine is impaired. FAH catalyzes the conversion of fumarylacetoacetate to fumarate and acetoacetate. Loss of FAH results in the accumulation of upstream compounds in the catabolic pathway. These include maleylacetoacetate (MAA) and fumarylacetoacetate (FAA). MAA and FAA are converted to succinylacetoacetate (SAA) which is then catabolized to succinylacetone (SA).The accumulation of MAA, FAA, and SA in cells inhibits the breakdown of thiol derivatives, leading to post-translational modifications to the antioxidant glutathione. This inhibits the antioxidant activity of glutathione, leading to reactive oxygen species (ROS) damaging cell components. Over time, the combined effect of accumulation of toxic metabolic intermediates and elevated ROS levels in liver and kidney cells leads to apoptosis in these tissues which ultimately results in organ failure. Accumulated SA in liver and kidney cells results in its release into the bloodstream, which leads to secondary effects. SA inhibits the enzyme 5-ALA dehydratase which converts aminolevulinic acid (5-ALA) into porphobilinogen, a precursor to porphyrin. | 0-1
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Horseshoe kidney, also known as ren arcuatus (in Latin), renal fusion or super kidney, is a congenital disorder affecting about 1 in 500 people that is more common in men, often asymptomatic, and usually diagnosed incidentally. In this disorder, the patients kidneys fuse to form a horseshoe-shape during development in the womb. The fused part is the isthmus of the horseshoe kidney. The abnormal anatomy can affect kidney drainage resulting in increased frequency of kidney stones and urinary tract infections as well as increase risk of certain renal cancers.Fusion abnormalities of the kidney can be categorized into two groups: horseshoe kidney and crossed fused ectopia. The horseshoe kidney is the most common renal fusion anomaly. Signs and symptoms
Although often asymptomatic, the most common presenting symptom of patients with a horseshoe kidney is abdominal or flank pain. However, presentation is often non-specific. Approximately a third of patients with horseshoe kidneys remain asymptomatic throughout their entire life with over 50% of patients having no medical issues related to their renal fusion when followed for a 25-year period. As a result, it is estimated that approximately 25% of patients with horseshoe kidneys are diagnosed incidentally with ultrasound or CT imaging. | Ecthyma( ec·thy·ma | \ ek-ˈthī-mə )is a variation of impetigo, presenting at a deeper level of tissue.It is usually associated with Group A (beta-hemolytic) Streptococcus (abbreviated GAS). See also
Ecthyma gangrenosum
References
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Enamel is very thin but hard & translucent, and may have random pits & grooves. Condition is of autosomal dominant, autosomal recessive, or x-linked pattern. Enamel differs in appearance from dentine radiographically as normal functional enamel. Type 2 - Hypomaturation
Enamel has sound thickness, with a pitted appearance. It is less hard compared to normal enamel, and are prone to rapid wear, although not as intense as Type 3 AI. Condition is of autosomal dominant, autosomal recessive, or x-linked pattern. Enamel appears to be comparable to dentine in its radiodensity on radiographs. Type 3 - Hypocalcified
Enamel defect due to malfunction of enamel calcification, therefore enamel is of normal thickness but is extremely brittle, with an opaque/chalky presentation. Teeth are prone to staining and rapid wear, exposing dentine. Condition is of autosomal dominant and autosomal recessive pattern. Enamel appears less radioopaque compared to dentine on radiographs. Type 4 - Hypomature hypoplastic enamel with taurodontism
Enamel has a variation in appearance, with mixed features from Type 1 and Type 2 AI. All Type 4 AI has taurodontism in common. Condition is of autosomal dominant pattern. Other common features may include an anterior open bite, taurodontism, sensitivity of teeth.Differential diagnosis would include dental fluorosis, molar-incisor hypomineralization, chronological disorders of tooth development. Treatment
Preventive and restorative dental care is very important as well as considerations for esthetic issues since the crown are yellow from exposure of dentin due to enamel loss. | SLC24A4 encodes a calcium transporter that mediates calcium transport to developing enamel during tooth development. Less is known about the function of other genes implicated in amelogenesis imperfecta.Researchers expect that mutations in further genes are likely to be identified as causes of amelogenesis imperfecta. Types include:
Amelogenesis imperfecta can have different inheritance patterns depending on the gene that is altered. Mutations in the ENAM gene are the most frequent known cause and are most commonly inherited in an autosomal dominant pattern. This type of inheritance means one copy of the altered gene in each cell is sufficient to cause the disorder.Amelogenesis imperfecta is also inherited in an autosomal recessive pattern; this form of the disorder can result from mutations in the ENAM, MMP20, KLK4, FAM20A, C4orf26 or SLC24A4 genes. Autosomal recessive inheritance means two copies of the gene in each cell are altered.About 5% of amelogenesis imperfecta cases are caused by mutations in the AMELX gene and are inherited in an X-linked pattern. A condition is considered X-linked if the mutated gene that causes the disorder is located on the X chromosome, one of the two sex chromosomes. In most cases, males with an X-linked form of this condition experience more severe dental abnormalities than affected females. Recent genetic studies suggest that the cause of a significant proportion of amelogenesis imperfecta cases remains to be discovered. Diagnosis
AI can be classified according to their clinical appearances:
Type 1 - Hypoplastic
Enamel of abnormal thickness due to malfunction in enamel matrix formation. | 11
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The 71 syntheses of physostigmine yield 33 racemic mixtures and 38 products of a single enantiomer. The first total synthesis of physostigmine was achieved by Julian and Pikl in 1935. The main goal of Julians formal physostigmine synthesis was to prepare the key compound (L)-eseroline (compound 10 in the adjacent diagram), the conversion of which to physostigmine would be straightforward. In one of his earlier works Julian synthesized the ring of physostigmine from 1-methyl-3-formyl-oxindole as starting material, which was discovered by Paul Friedländer. However, the starting material was expensive, and the reduction of a nitrile to an amine (similar to the reaction of compound 6 to give compound 7 in the diagram) with sodium and alcohol did not proceed in good yield. In his second work “Studies in the Indole Series III,” he had improved the yield of amine from nitrile significantly by using palladium and hydrogen. Although he succeeded in the synthesis of the target compound, the route had several drawbacks. First, the chemical resolution of compound 8 is unreliable, and the chemical resolution of rac-eserethole gives optically pure product only after eight recrystallizations of its tartrate salt. Second, the reductive amination going from compound 8 to compound 9 requires a large amount of Na. In the years since this initial work, many other groups have used a variety of approaches to construct the ring system and showcase new synthetic methods. | The bean was studied throughout the 1860s by a few different Edinburgh scientists, including Douglas Argyll Robertson who wrote a paper on the use of Calabar bean extract on the eye and was the first to use it medicinally, and Thomas Richard Fraser, who researched how to best extract the active principle, which was later determined to be physostigmine. Fraser also studied the antagonism between physostigmine and atropine extremely rigorously, at a time when the concept of antagonism had little if any experimental support. Frasers research is still the basis of todays knowledge about the interactions between atropine and physostigmine at many different and specific doses. Physostigmines first use as a treatment for glaucoma was by Ludwig Laqueur in 1876. Laqueur himself had glaucoma so, like Christison, he experimented on himself, although Laqueur was much more scientific and methodical in his self-treatment. In the 1920s, Otto Loewi determined the biomechanical mechanism for the effects of physostigmine on the body. Loewi was studying how actions that we now consider to be controlled by the parasympathetic nervous system, were directed by chemicals. During his studies, Loewi discovered acetylcholine and that physostigmine acted by preventing acetylcholine inhibition. In 1936, Loewi was awarded the Nobel Prize for his work on discovering acetylcholine and biological chemical transmitters. More important discoveries surrounding physostigmine were made at the University of Edinburgh in 1925. Edgar Stedman and George Barger determined the structure of physostigmine using a method called chemical degradation. In 1935 Percy Lavon Julian was later the first to synthesize physostigmine. | 11
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History
1980–1987
In April 1980, as part of a formal research project at the French pharmaceutical company Roussel-Uclaf for the development of glucocorticoid receptor antagonists, endocrinologist Étienne-Émile Baulieu and chemist Georges Teutsch synthesized mifepristone (RU-38486, the 38,486th compound synthesized by Roussel-Uclaf from 1949 to 1980; shortened to RU-486), which was discovered to also be a progesterone receptor antagonist. In October 1981, Étienne-Émile Baulieu, a consultant to Roussel-Uclaf, arranged tests of its use for medical abortion in 11 women in Switzerland by gynecologist Walter Herrmann at the University of Genevas Cantonal Hospital, with successful results announced on 19 April 1982. On 9 October 1987, following worldwide clinical trials in 20,000 women of mifepristone with a prostaglandin analogue (initially sulprostone or gemeprost, later misoprostol) for medical abortion, Roussel-Uclaf sought approval in France for their use for medical abortion, with approval announced on 23 September 1988. 1988–1990
On 21 October 1988, in response to antiabortion protests and concerns of majority (54.5%) owner Hoechst AG of Germany, Roussel-Uclafs executives and board of directors voted 16 to 4 to stop distribution of mifepristone, which they announced on 26 October 1988. Two days later, the French government ordered Roussel-Uclaf to distribute mifepristone in the interests of public health. French Health Minister Claude Évin explained: "I could not permit the abortion debate to deprive women of a product that represents medical progress. From the moment Government approval for the drug was granted, RU-486 became the moral property of women, not just the property of a drug company." | While the Fourth Circuit had granted a preliminary injunction to allow this distribution, the Supreme Court of the United States issued a stay order in January 2021 to retain the FDAs rule pending the results of the ongoing litigation.On 16 December 2021, the FDA voluntarily adopted a new rule permanently relaxing the requirement that the pill be obtained in person, allowing it to be sent through the mail. A prescription is still required, so that a doctor can screen for risk factors that would make taking the pill unsafe for the mother. Subsection H
Some drugs are approved by the FDA under subsection H, which has two subparts. The first sets forth ways to rush experimental drugs, such as aggressive HIV and cancer treatments, to market when speedy approval is deemed vital to the health of potential patients. The second part of subsection H applies to drugs that not only must meet restrictions for use due to safety requirements, but also are required to meet postmarketing surveillance to establish that the safety results shown in clinical trials are seconded by use in a much wider population. Until December 2021, Mifepristone was approved under the second part of subsection H. The result is that women could not pick the drug up at a pharmacy, but were required to receive it directly from a doctor. | 11
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Today
Minamata disease remains an important issue in contemporary Japanese society. Lawsuits against Chisso and the prefectural and national governments are still continuing and many regard the government responses to date as inadequate. The companys "historical overview" in its current website makes no mention of their role in the mass contamination of Minamata and the dreadful aftermath. Their 2004 Annual Report, however, reports an equivalent of about US$50 million (5,820 million yen) in "Minamata Disease Compensation Liabilities". From 2000 to 2003, the company also reported total compensation liabilities of over US$170 million. Their 2000 accounts also show that the Japanese and Kumamoto prefectural governments waived an enormous US$560 million in related liabilities. Their FY2004 and FY2005 reports refer to Minamata disease as "mad hatters disease", a term coined from the mercury poisoning experienced by hat-makers of the last few centuries (cf. Erethism).A memorial service was held at the Minamata Disease Municipal Museum on 1 May 2006 to mark 50 years since the official discovery of the disease. Despite bad weather, the service was attended by over 600 people, including Chisso chairman Shunkichi Goto and Environment Minister Yuriko Koike.On Monday, March 29, 2010, a group of 2,123 uncertified victims reached a settlement with the government of Japan, the Kumamoto Prefectural government, and Chisso Corporation to receive individual lump-sum payments of 2.1 million yen and monthly medical allowances.Most congenital patients are now in their forties and fifties and their health is deteriorating. | Two radars monitor the slope of a mountain above the road. The system automatically closes the road by activating several barriers and traffic lights within seconds such that no people are harmed. Survival, rescue, and recovery
Avalanche accidents are broadly differentiated into 2 categories: accidents in recreational settings, and accidents in residential, industrial, and transportation settings. This distinction is motivated by the observed difference in the causes of avalanche accidents in the two settings. In the recreational setting most accidents are caused by the people involved in the avalanche. In a 1996 study, Jamieson et al. (pages 7–20) found that 83% of all avalanches in the recreational setting were caused by those who were involved in the accident. In contrast, all of the accidents in the residential, industrial, and transportation settings were due to spontaneous natural avalanches. Because of the difference in the causes of avalanche accidents, and the activities pursued in the two settings, avalanche and disaster management professionals have developed two related preparedness, rescue, and recovery strategies for each of the settings. Notable avalanches
Two avalanches occurred in March 1910 in the Cascade and Selkirk Mountain ranges; On 1 March the Wellington avalanche killed 96 in Washington state, United States. Three days later 62 railroad workers were killed in the Rogers Pass avalanche in British Columbia, Canada. During World War I, an estimated 40,000 to 80,000 soldiers died as a result of avalanches during the mountain campaign in the Alps at the Austrian-Italian front, many of which were caused by artillery fire. | 0-1
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When dealing with chronic pain, these practices generally fall into the following four categories: biological, mind-body, manipulative body, and energy medicine.Implementing dietary changes, which is considered a biological-based alternative medicine practice, has been shown to help improve symptoms of chronic pain over time. Adding supplements to ones diet is a common dietary change when trying to relieve chronic pain, with some of the most studied supplements being: Acetyl-L-carnitine, alpha lipoic acid, and vitamin E. Vitamin E is perhaps the most studied out of the three, with strong evidence that it helps lower neurotoxicity in those with cancer, multiple sclerosis, and cardiovascular diseases.Hypnosis, including self-hypnosis, has tentative evidence. Hypnosis, specifically, can offer pain relief for most people and may be a safe alternative to pharmaceutical medication. Evidence does not support hypnosis for chronic pain due to a spinal cord injury.Preliminary studies have found medical marijuana to be beneficial in treating neuropathic pain, but not other kinds of long term pain. As of 2018, the evidence for its efficacy in treating neuropathic pain or pain associated with rheumatic diseases is not strong for any benefit and further research is needed. For chronic non-cancer pain, a recent study concluded that it is unlikely that cannabinoids are highly effective. However, more rigorous research into cannabis or cannabis-based medicines is needed.Tai chi has been shown to improve pain, stiffness, and quality of life in chronic conditions such as osteoarthritis, low back pain, and osteoporosis. | Fabry disease, also known as Anderson–Fabry disease, is a rare genetic disease that can affect many parts of the body, including the kidneys, heart, and skin. Fabry disease is one of a group of conditions known as lysosomal storage diseases. The genetic mutation that causes Fabry disease interferes with the function of an enzyme that processes biomolecules known as sphingolipids, leading to these substances building up in the walls of blood vessels and other organs. It is inherited in an X-linked manner. Fabry disease is sometimes diagnosed using a blood test that measures the activity of the affected enzyme called alpha-galactosidase, but genetic testing is also sometimes used, particularly in females. The treatment for Fabry disease varies depending on the organs affected by the condition, and the underlying cause can be addressed by replacing the enzyme that is lacking. The first descriptions of the condition were made simultaneously by dermatologist Johannes Fabry and the surgeon William Anderson in 1898. Signs and symptoms
Symptoms are typically first experienced in early childhood and can be very difficult to diagnose; the rarity of Fabry disease to many clinicians sometimes leads to misdiagnoses. Manifestations of the disease usually increase in number and severity as an individual ages. Pain
Full-body or localized pain to the extremities (known as acroparesthesia) or gastrointestinal (GI) tract is common in patients with Fabry disease. This pain can increase over time. This acroparesthesia is believed to be related to the damage of peripheral nerve fibers that transmit pain. | 0-1
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The protozoan is in the smaller of its two forms, called an amastigote, which is round, non-motile, and only 3–7 micrometers in diameter. Inside the stomach of the sandfly, the amastigotes quickly transform into elongated and motile forms called the promastigotes. Promastigote is spindle-shaped, triple the size of the amastigote, and has a single flagellum that allows mobility. The promastigotes live extracellularly in the alimentary canal, reproducing asexually, then migrate to the proximal end of the gut where they become poised for a regurgitational transmission. As the fly bites, the promastigotes are released from the proboscis and introduced locally at the bite site.Once inside the human host, promastigotes invade macrophages. Inside the cells they transform back into the smaller amastigote form. The amastigotes replicate in the most hostile part of the macrophage cell, inside the phagolysosome, whose normal defensive response they are able to prevent. After repeated multiplication, they break down their host cell by sheer pressure of mass, but there is some recent speculation that they are able to leave the cell by triggering the exocytosis response of the macrophage. The daughter cells protozoans then migrate to fresh cells or through the bloodstream to find new hosts. In this way the infection is progressive, spreading to the hosts mononuclear phagocyte system, particularly the spleen and liver. The free amastigotes in peripheral tissues are then ingested by sandfly to enter another cycle. Regulatory T and B cells
The cell-mediated immunity (CMI) that kills Leishmania also produces inflammation. If the inflammation is excessive, it can cause tissue damage. | Granuloma faciale is an uncommon benign chronic skin disease of unknown origin characterized by single or multiple cutaneous nodules, usually occurring over the face. : 836 Occasionally, extrafacial involvement is noted, most often on sun-exposed areas. Diagnosis
Skin biopsy for histopathology:
Focal LCV, diffuse dermal neutrophilia with leukocytoclasia, tissue eosinophilia & perivascular fibrosis. Differential diagnosis
The disease mimics many other dermatoses and can be confused with conditions, such as sarcoidosis, discoid lupus erythematosus, mycosis fungoides, and fixed drug eruption. Treatment
Topical corticosteroid,
Intralesional corticosteroid,
Dapsone,
Colchicine,
Antimalarial,
Pulse dye laser,
Carbon dioxide laser. History
GF was first described in 1945 by John Edwin Mackonochie Wigley (1892–1962). See also
Cutaneous small-vessel vasculitis
List of cutaneous conditions
References
== External links == | 0-1
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Sexual abuse can be a particular trigger for suicidal behavior in adolescents with BPD tendencies. Sense of self
People with BPD tend to have trouble seeing their identity clearly. In particular, they tend to have difficulty knowing what they value, believe, prefer, and enjoy. They are often unsure about their long-term goals for relationships and jobs. This can cause people with BPD to feel "empty" and "lost". Self-image can also change rapidly from healthy to unhealthy. People with BPD may base their identity on others, leading to chameleon-like changes in identity. Cognitions
The often intense emotions people with BPD experience may make it difficult for them to concentrate. They may also tend to dissociate, which can be thought of as an intense form of "zoning out". Others can sometimes tell when someone with BPD is dissociating because their facial or vocal expressions may become flat or expressionless, or they may appear distracted and "numb" to emotional stimuli.Dissociation most often occurs in response to a painful event (or something that triggers the memory of a painful event). It involves the mind automatically redirecting attention away from the current event or situation or blocking it out entirely. This is done presumably to protect against - based on similar or related past experiences - what the mind perceives and forecasts as arousing intense negative emotions and unwanted behavioral impulses that the present emotive event might trigger. | Treatment
The malabsorption resulting from lack of bile acid has resulted in elemental formula being suggested, which are low in fat with < 3% of calories derived from long chain triglycerides (LCT). However, reduced very long chain fatty acids (VLCFA) has not been shown to reduce blood VLCFA levels, likely because humans can endogenously produce most VLCFA. Plasma VLCFA levels are decreased when dietary VLCFA is reduced in conjunction with supplementation of Lorenzos oil (a 4:1 mixture of glyceryl trioleate and glyceryl trierucate) in X-ALD patients. Since docosahexaenoic acid (DHA) synthesis is impaired [59], DHA supplementation was recommended, but a placebo-controlled study has since showed no clinical efficacy. Due to the defective bile acid synthesis, fat soluble supplements of vitamins A, D, E, and K are recommended. Prognosis
Currently, no cure for Zellweger syndrome is known, nor is there a standard course of treatment. Infections should be guarded against to prevent such complications as pneumonia and respiratory distress. Other treatment is symptomatic and supportive. Patients usually do not survive beyond one year of age. References
External links
Zellweger-Syndrome at NINDS
Zellweger syndrome at NIHs Office of Rare Diseases | 0-1
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Scattering leaves of plants with microscopic hooked hairs around a bed at night, then sweeping them up in the morning and burning them, was a technique reportedly used in Southern Rhodesia and in the Balkans.Bean leaves have been used historically to trap bedbugs in houses in Eastern Europe. The trichomes on the bean leaves capture the insects by impaling the feet (tarsi) of the insects. The leaves are then destroyed. 20th century
Before the mid-20th century, bed bugs were very common. According to a report by the UK Ministry of Health, in 1933, all the houses in many areas had some degree of bed bug infestation. The increase in bed bug populations in the early 20th century has been attributed to the advent of electric heating, which allowed bed bugs to thrive year-round instead of only in warm weather.Bed bugs were a serious problem at US military bases during World War II. Initially, the problem was solved by fumigation, using Zyklon Discoids that released hydrogen cyanide gas, a rather dangerous procedure. Later, DDT was used to good effect, though bedbugs have since become largely resistant to it.The decline of bed bug populations in the 20th century is often credited to potent pesticides that had not previously been widely available. | Butabarbital sodium can be used as a pre-surgical anesthetic aid or in the short-term treatment of sleep and anxiety disorders. For short term sleep maintenance and induction butabarbital sodium treatment is recommended to be limited to two weeks, after which it begins to lose efficacy. The half life is approximately 100hrs, eliminated primarily by hepatic microsomal enzymes and excreted in the urine. Overdose & abuse
Butabarbital sodium is a Schedule III controlled substance in the US and Canada due to risk of abuse and dependence. A psychoactive substance, butabarbital drugs are often abused with risk of acute intoxication and addiction. Barbiturates such as butabarbital are thought to be habit-forming and addictive, and have severe risks from withdrawal including death. Due to the high risk of dependence and overdose, use of butabarbital is highly regulated and has become less common in clinical application. Side effects
Those taking Butabarbital are asked to watch out for signs of severe allergic reaction, such as swelling or difficulty breathing. Less serious side effects include dizziness or drowsiness, excitation, headache, nausea, vomiting, or constipation. Psychiatric disturbances (hallucinations, agitation, confusion, depression, or memory problems), ataxia, difficulty breathing, or slow heartbeat could be signs of serious adverse effects and should be brought to the attention of a doctor immediately. Adverse effects
1-10%
Somnolence<1%
Contradictions
Those with a hypersensitivity to barbiturates or a history of porphyria should discuss with a doctor before using butisol sodium treatment. | 0-1
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Two forms of theological determinism exist, referred to as strong and weak theological determinism.Strong theological determinism is based on the concept of a creator deity dictating all events in history: "everything that happens has been predestined to happen by an omniscient, omnipotent divinity. "Weak theological determinism is based on the concept of divine foreknowledge—"because Gods omniscience is perfect, what God knows about the future will inevitably happen, which means, consequently, that the future is already fixed." There exist slight variations on this categorisation, however. Some claim either that theological determinism requires predestination of all events and outcomes by the divinity—i.e., they do not classify the weaker version as theological determinism unless libertarian free will is assumed to be denied as a consequence—or that the weaker version does not constitute theological determinism at all.With respect to free will, "theological determinism is the thesis that God exists and has infallible knowledge of all true propositions including propositions about our future actions," more minimal criteria designed to encapsulate all forms of theological determinism.Theological determinism can also be seen as a form of causal determinism, in which the antecedent conditions are the nature and will of God. Some have asserted that Augustine of Hippo introduced theological determinism into Christianity in 412 CE, whereas all prior Christian authors supported free will against Stoic and Gnostic determinism. However, there are many Biblical passages that seem to support the idea of some kind of theological determinism. | Determinism is a philosophical view, where all events are determined completely by previously existing causes. Deterministic theories throughout the history of philosophy have developed from diverse and sometimes overlapping motives and considerations. The opposite of determinism is some kind of indeterminism (otherwise called nondeterminism) or randomness. Determinism is often contrasted with free will, although some philosophers claim that the two are compatible.Determinism is often used to mean causal determinism, which in physics is known as cause-and-effect. This is the concept that events within a given paradigm are bound by causality in such a way that any state of an object or event is completely determined by its prior states. This meaning can be distinguished from other varieties of determinism mentioned below. Debates about determinism often concern the scope of determined systems; some maintain that the entire universe is a single determinate system, and others identifying more limited determinate systems (or multiverse). Historical debates involve many philosophical positions and varieties of determinism. They include debates concerning determinism and free will, technically denoted as compatibilistic (allowing the two to coexist) and incompatibilistic (denying their coexistence is a possibility). Determinism should not be confused with the self-determination of human actions by reasons, motives, and desires. Determinism is about interactions which affect our cognitive processes in our life. It is about the cause and the result of what we have done. Cause and result are always bounded together in cognitive processes. | 11
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Following these results, Wyeth experienced a significant decline in its sales of Premarin, Prempro (CEEs and medroxyprogesterone acetate), and related products, from over $2 billion in 2002 to just over $1 billion in 2006. Litigation
This drug has been the subject of litigation; more than 13,000 people have sued Wyeth between 2002 and 2009. Wyeth and Pharmacia & Upjohn prevailed in the vast majority of hormone therapy cases previously set for trial through a combination of rulings by judges, verdicts by juries, and dismissals by plaintiffs themselves. Of the companys losses, two of the jury verdicts were reversed post-trial and others are being challenged on appeal. Wyeth also won five summary judgments on Prempro cases and had 15 cases voluntarily dismissed by plaintiffs. The company won dismissals in another 3,000 cases. In 2006, Mary Daniel, in a trial in Philadelphia, was awarded $1.5 million in compensatory damages as well as undisclosed punitive damages. As of 2010, Wyeth had won the last four of five cases, most recently in Virginia, finding that they were not responsible for the breast cancer of plaintiff Georgia Torkie-Tork. Wyeth has been quoted as saying "many risk factors associated with breast cancer have been identified, but science cannot establish what role any particular risk factor or combination play in any individual womans breast cancer." Wyeths counsel in the case also noted that in the WHI trial, 99.62% of women took the drug and "did not get breast cancer." | Antihistamines are a commonly prescribed first-line treatment for conventional urticaria, but its effectiveness in the treatment of CU is rather limited in most cases. Some research suggests that first-generation antihistamines with anticholinergic properties such as diphenhydramine are most successful at treating CU. Treatment(s) with mixed success: omalizumab (anti-IgE therapy), danazol (synthetic androgen), propranolol (beta blocker), zileuton (antileukotriene). Acquired anhidrosis and/or hypohidrosis
This subtype of CU refers to those who have abnormally reduced sweating. Diagnosis
Sweat is readily visualized by a topical indicator such as iodinated starch or sodium alizarin sulphonate. Both undergo a dramatic colour change when moistened by sweat. A thermoregulatory sweat test evaluates the bodys response to a thermal stimulus by inducing sweating through the use of a hot box ⁄ room, thermal blanket or exercise. Failure of the topical indicator to undergo a colour change during thermoregulatory sweat testing can indicate anhidrosis and/or hypohidrosis (see Minor test).A skin biopsy may reveal cellular infiltrates in sweat glands or ducts. Features
Severe heat intolerance (e.g., nausea, dizziness, and headache), and tingling, pricking, pinchy or burning pain over the entire body on exposure to hot environments or prolonged exercise which improve after cooling the body. Occurs in the absence of any causative skin, metabolic, or neurological disorders. Pathophysiology
The wheals, hypohidrosis, and pain seems to result from the low expression levels of acetylcholinesterase (AchE) and cholinergic receptor, muscarinic 3 (CHRM3) in the eccrine gland epithelial cells. | 0-1
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While awaiting rescue, swimming or treading water should be limited to conserve energy, and the person should attempt to remove as much of the body from the water as possible; attaching oneself to a buoyant object can improve the chance of survival should unconsciousness occur.Hypothermia (and cardiac arrest) presents a risk for survivors of immersion. This risk increases if the survivor—feeling well again—tries to get up and move, not realizing their core body temperature is still very low and will take a long time to recover.Most people who experience cold-water drowning do not develop hypothermia quickly enough to decrease cerebral metabolism before ischemia and irreversible hypoxia occur. The neuroprotective effects appear to require water temperatures below about 5 °C (41 °F). Diagnosis
The World Health Organization in 2005 defined drowning as "the process of experiencing respiratory impairment from submersion/immersion in liquid." This definition does not imply death or even the necessity for medical treatment after removing the cause, nor that any fluid enters the lungs. The WHO further recommended that outcomes should be classified as death, morbidity, and no morbidity. There was also consensus that the terms wet, dry, active, passive, silent, and secondary drowning should no longer be used.Experts differentiate between distress and drowning. Distress – people in trouble, but who can still float, signal for help, and take action. Drowning – people suffocating and in imminent danger of death within seconds. Forensics
Forensic diagnosis of drowning is considered one of the most difficult in forensic medicine. | The first virus isolates were obtained during this outbreak from mosquitoes and human blood samples collected from Gulu in northern Uganda in 1959.The second epidemic in 1996–1997 was confined to Uganda. The 35-year hiatus between the two outbreaks and evidence of an outbreak in 1904–1906 in Uganda indicate a 30–50 year cycle for epidemics.In 2013, ONNV was confirmed as the cause of disease in a 60-year-old German woman who became infected while traveling in East Africa. In 2015–2016 there was a minor outbreak in Uganda with 51 suspected cases.There has been a minor outbreak in Mombasa (Kenya) and the County Government of Mombasa issued a warning. A 2015 study indicated that ONNV is endemic in coastal East Africa, along with chikungunya virus. References
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As a response, a novel oral polio vaccine type 2 (nOPV2) was developed with the aim to provide a safer form of vaccination against type 2 strains with less risk of reverting to infectious polio. Schedule
In countries with endemic polio or where the risk of imported cases is high, the WHO recommends OPV vaccine at birth followed by a primary series of three OPV doses and at least one IPV dose starting at 6 weeks of age, with a minimum of 4 weeks between OPV doses. In countries with >90% immunization coverage and low risk of importation, the WHO recommends one or two IPV doses starting at 2 months of age followed by at least two OPV doses, with the doses separated by 4–8 weeks depending on the risk of exposure. In countries with the highest levels of coverage and the lowest risks of importation and transmission, the WHO recommends a primary series of three IPV injections, with a booster dose after an interval of six months or more if the first dose was administered before 2 months of age. Side effects
The inactivated polio vaccines are very safe. Mild redness or pain may occur at the site of injection. Oral polio vaccine results in vaccine-associated paralytic poliomyelitis in about three per million doses. They are generally safe to give to those who are pregnant, and those who have HIV/AIDS, but who are otherwise well. | Types
Examples of mitochondrial diseases include:
Mitochondrial myopathy
Diabetes mellitus and deafness (DAD)
this combination at an early age can be due to mitochondrial disease
Diabetes mellitus and deafness can be found together for other reasons
Lebers hereditary optic neuropathy (LHON)
visual loss beginning in young adulthood
eye disorder characterized by progressive loss of central vision due to degeneration of the optic nerves and retina
affects 1 in 50,000 people in Finland
Leigh syndrome, subacute necrotizing encephalomyelopathyafter normal development the disease usually begins late in the first year of life, although onset may occur in adulthood
a rapid decline in function occurs and is marked by seizures, altered states of consciousness, dementia, ventilatory failure
Neuropathy, ataxia, retinitis pigmentosa, and ptosis (NARP)
progressive symptoms as described in the acronym
dementia
Myoneurogenic gastrointestinal encephalopathy (MNGIE)
gastrointestinal pseudo-obstruction
neuropathy
MERRF syndrome
progressive myoclonic epilepsy
"Ragged Red Fibers" are clumps of diseased mitochondria that accumulate in the subsarcolemmal region of the muscle fiber and appear when muscle is stained with modified Gömöri trichrome stain
short stature
hearing loss
lactic acidosis
exercise intolerance
MELAS syndrome
Mitochondrial DNA depletion syndromeConditions such as Friedreichs ataxia can affect the mitochondria but are not associated with mitochondrial proteins. Presentation
Associated conditions
Acquired conditions in which mitochondrial dysfunction has been involved are:
diabetes
Huntingtons disease
cancer
Alzheimers disease,
Parkinsons disease
bipolar disorder, schizophrenia, aging and senescence, anxiety disorders
cardiovascular disease
sarcopenia
chronic fatigue syndromeThe body, and each mutation, is modulated by other genome variants; the mutation that in one individual may cause liver disease might in another person cause a brain disorder. The severity of the specific defect may also be great or small. Some defects include exercise intolerance. | 0-1
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Nutritional support with tube feeds is essential until eclabium resolves and infants can begin nursing. Ophthalmology consultation is useful for the early management of ectropion, which is initially pronounced and resolves as scales are shed. Liberal application of petrolatum is needed multiple times a day. In addition, careful debridement of constrictive bands of hyperkeratosis should be performed to avoid digital ischemia.Cases of digital autoamputation or necrosis have been reported due to cutaneous constriction bands. Relaxation incisions have been used to prevent this morbid complication. Prognosis
In the past, the disorder was nearly always fatal, whether due to dehydration, infection (sepsis), restricted breathing due to the plating, or other related causes. The most common cause of death was systemic infection, and sufferers rarely survived for more than a few days. However, improved neonatal intensive care and early treatment with oral retinoids, such as the drug Isotretinoin, may improve survival. Early oral retinoid therapy has been shown to soften scales and encourage desquamation. After as little as two weeks of daily oral isotretinoin, fissures in the skin can heal, and plate-like scales can nearly resolve. Improvement in the eclabium and ectropion can also be seen in a matter of weeks. Children who survive the neonatal period usually evolve to a less severe phenotype, resembling a severe congenital ichthyosiform erythroderma. Patients continue to suffer from temperature dysregulation and may have heat and cold intolerance. Patients can also have generalized poor hair growth, scarring alopecia, contractures of digits, arthralgias, failure to thrive, hypothyroidism, and short stature. | Harlequin-type ichthyosis is a genetic disorder that results in thickened skin over nearly the entire body at birth. The skin forms large, diamond/trapezoid/rectangle-shaped plates that are separated by deep cracks. These affect the shape of the eyelids, nose, mouth, and ears and limit movement of the arms and legs. Restricted movement of the chest can lead to breathing difficulties. These plates fall off over several weeks. Other complications can include premature birth, infection, problems with body temperature, and dehydration. The condition is the most severe form of ichthyosis, a group of genetic disorders characterised by scaly skin.Harlequin-type ichthyosis is caused by mutations in the ABCA12 gene. This gene codes for a protein necessary for transporting lipids out of cells in the outermost layer of skin. The disorder is autosomal recessive and inherited from parents who are carriers. Diagnosis is often based on appearance at birth and confirmed by genetic testing. Before birth, amniocentesis or ultrasound may support the diagnosis.There is no cure for the condition. Early in life, constant supportive care is typically required. Treatments may include moisturizing cream, antibiotics, etretinate or retinoids. Around half of those affected die within the first few months; however, retinoid treatment can increase chances of survival. Children who survive the first year of life often have long-term problems such as red skin, joint contractures and delayed growth. The condition affects around 1 in 300,000 births. It was first documented in a diary entry by Reverend Oliver Hart in America in 1750. | 11
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The number of strings on a violin, a viola, a cello, double bass, a cuatro, a typical bass guitar, and a ukulele, and the number of string pairs on a mandolin. "Four calling birds" is the gift on the fourth day of Christmas in the carol "The Twelve Days of Christmas". Groups of four
Big Four (disambiguation)
Four basic operations of arithmetic: addition, subtraction, multiplication, division. Greek classical elements (fire, air, water, earth). Four seasons: spring, summer, autumn, winter. The Four Seasons (disambiguation)
A leap year generally occurs every four years. Approximately four weeks (4 times 7 days) to a lunar month (synodic month = 29.53 days). Thus the number four is universally an integral part of primitive sacred calendars. Four weeks of Advent (and four Advent candles on the Advent wreath). Four cardinal directions: north, south, east, west. Four Temperaments: sanguine, choleric, melancholic, phlegmatic. Four Humors: blood, yellow bile, black bile, phlegm. Four Great Ancient Capitals of China. Four-corner method. Four Asian Tigers, referring to the economies of Hong Kong, Taiwan, South Korea, and Singapore
Cardinal principles. Four cardinal virtues: justice, prudence, temperance, fortitude. Four suits of playing cards: hearts, diamonds, clubs, spades. Four nations of the United Kingdom: England, Wales, Scotland, Northern Ireland. Four provinces of Ireland: Munster, Ulster, Leinster, Connacht. Four estates: politics, administration, judiciary, journalism. Especially in the expression "Fourth Estate", which means journalism. Four Corners is the only location in the United States where four states come together at a single point: Colorado, Utah, New Mexico, and Arizona. | The dosage of 1–10 mg/L is preferred as scale inhibitor, 10–50 mg/L as corrosion inhibitor, and 1000–2000 mg/L as detergent. Usually, HEDP is also used together with polycarboxylic acid (superplasticizer), in which it acts as reducing agent. Chelating agent and antioxidant
Etidronic acid is a chelating agent and may be added to bind or, to some extent, counter the effects of substances, such as calcium, iron or other metal ions, which may be discharged as a component of grey wastewater and could conceivably contaminate groundwater supplies. As a phosphonate it has corrosion inhibiting properties on unalloyed steel. Etidronic acid also acts to retard rancidification and oxidation of fatty acids. HEDP and its salts are added to detergents and other cleaning agents to prevent the effects of hard water. It is also used in peroxide bleaching to prevent degradation of peroxides by transition metals. Etidronic acid is listed as an ingredient of several cosmetic formulations where it is used for suppressing radical formation, emulsion stabiliser and viscosity control. While etidronic acid has not been limited from inclusion in cosmetics and does have legitimate uses, it is recommended that, as with most cosmetic products (particularly soaps), the product should be thoroughly rinsed from the skin after use. Etidronic acid is also included among swimming pool chemicals. It is used as a stain inhibitor to prevent metal ions coming out of solution and staining the sides of swimming pools. Etidronic acid is used in bars of soap. | 0-1
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Long-term use of amphetamines may increase the risk of Parkinsons disease (PD): in one retrospective study with over 40,000 participants it was concluded that amphetamine abusers generally had a 200% higher chance of developing PD versus those with no history of abuse; the risk was much higher in women, almost 400%. There remains some controversy as of 2017.Levodopa-induced dyskinesia (LID) is evident in patients with Parkinsons disease who have been on levodopa (L‑DOPA) for prolonged periods of time. LID commonly first appears in the foot, on the most affected side of the body. There are three main types that can be classified on the basis of their course and clinical presentation following an oral dose of L‑DOPA:
Off-period dystonia – correlated to the akinesia that occurs before the full effect of L‑DOPA sets in, when the plasma levels of L‑DOPA are low. In general, it occurs as painful spasms in the foot. Patients respond to L‑DOPA therapy. Diphasic dyskinesia – occurs when plasma L-DOPA levels are rising or falling. This form occurs primarily in the lower limbs (though they can happen elsewhere) and is usually dystonic (characterized by apparent rigidity within muscles or groups thereof) or ballistic (characterized by involuntary movement of muscles) and will not respond to L‑DOPA dosage reductions. Peak-dose dyskinesia – the most common form of levodopa-induced dyskinesia; it correlates with the plateau L‑DOPA plasma level. This type usually involves the upper limbs more (but could also affect the head, trunk and respiratory muscles), is choreic (of chorea), and less disabling. | Patients will respond to L‑DOPA reduction but may be accompanied by deterioration of parkinsonism. Peak-dose L-DOPA-induced dyskinesia has recently been suggested to be associated with cortical dysregulation of dopamine signaling. Chronic or tardive
Late-onset dyskinesia, also known as tardive dyskinesia, occurs after long-term treatment with an antipsychotic drug such as haloperidol (Haldol) or amoxapine (Asendin). The symptoms include tremors and writhing movements of the body and limbs, and abnormal movements in the face, mouth, and tongue – including involuntary lip smacking, repetitive pouting of the lips, and tongue protrusions.Rabbit syndrome is another type of chronic dyskinesia, while orofacial dyskinesia may be related to persistent replication of Herpes simplex virus type 1. Non-motor
Two other types, primary ciliary dyskinesia and biliary dyskinesia, are caused by specific kinds of ineffective movement of the body, and are not movement disorders. See also
References
Works cited
David Healy (2008). Psychiatric Drugs Explained. Elsevier Health Sciences. ISBN 978-0-7020-2997-4. == External links == | 11
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"The proponents of such hypotheses often question the adequacy of nutrient tissue levels, especially vitamin C, for those children currently or recently ill, bacterial infections, those with higher individual requirements, those with environmental challenges (e.g. allergies), and perhaps transient vaccination-related stresses. At the time of this writing, infantile scurvy in the United States is practically nonexistent. No cases of scurvy mimicking SBS or sudden infant death syndrome have been reported, and scurvy typically occurs later in infancy, rarely causes death or intracranial bleeding, and is accompanied by other changes of the bones and skin and invariably an unusually deficient dietary history.In one study vaccination was shown not associated with retinal hemorrhages. Gestational problems
Gestational problems affecting both mother and fetus, the birthing process, prematurity and nutritional deficits can accelerate skeletal and hemorrhagic pathologies that can also mimic SBS, even before birth. Prevention
Interventions by neonatal nurses include giving parents information about abusive head trauma, normal infant crying and reasons for crying, teaching how to calm an infant, and how to cope if the infant was inconsolable may reduce rates of SBS. Treatment
Treatment involves monitoring intracranial pressure (the pressure within the skull). Treatment occasionally requires surgery, such as to place a cerebral shunt to drain fluid from the cerebral ventricles, and, if an intracranial hematoma is present, to drain the blood collection. Prognosis
Prognosis depends on severity and can range from total recovery to severe disability to death when the injury is severe. | The court reported that "the unified hypothesis [could] no longer be regarded as a credible or alternative cause of the triad of injuries": subdural haemorrhage, retinal bleeding and encephalopathy due to hypoxemia (low blood oxygen) found in suspected SBS.On 31 January 2008, the Wisconsin Court of Appeals granted Audrey A. Edmunds a new trial based on "competing credible medical opinions in determining whether there is a reasonable doubt as to Edmundss guilt." Specifically, the appeals court found that "Edmunds presented evidence that was not discovered until after her conviction, in the form of expert medical testimony, that a significant and legitimate debate in the medical community has developed in the past ten years over whether infants can be fatally injured through shaking alone, whether an infant may suffer head trauma and yet experience a significant lucid interval prior to death, and whether other causes may mimic the symptoms traditionally viewed as indicating shaken baby or shaken impact syndrome. "In 2012, A. Norman Guthkelch, the neurosurgeon often credited with "discovering" the diagnosis of SBS, published an article "after 40 years of consideration," which is harshly critical of shaken baby prosecutions based solely on the triad of injuries. | 11
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It can also occur in patients with increased hydrostatic venous pressure or decreased oncotic venous pressure, due to obstruction of lymphatic or venous vessels draining the lower extremity. Certain drugs (for example, amlodipine) can cause pedal edema. Cerebral edema is extracellular fluid accumulation in the brain. It can occur in toxic or abnormal metabolic states and conditions such as systemic lupus or reduced oxygen at high altitudes. It causes drowsiness or loss of consciousness, leading to brain herniation and death. Pulmonary edema occurs when the pressure in blood vessels in the lung is raised because of obstruction to the removal of blood via the pulmonary veins. This is usually due to failure of the left ventricle of the heart. It can also occur in altitude sickness or on inhalation of toxic chemicals. Pulmonary edema produces shortness of breath. Pleural effusions may occur when fluid also accumulates in the pleural cavity. Edema may also be found in the cornea of the eye with glaucoma, severe conjunctivitis or keratitis or after surgery. Affected people may perceive coloured haloes around bright lights. Edema surrounding the eyes is called periorbital edema (puffy eyes) . The periorbital tissues are most noticeably swollen immediately after waking, perhaps as a result of the gravitational redistribution of fluid in the horizontal position. Common appearances of cutaneous edema are observed with mosquito bites, spider bites, bee stings (wheal and flare), and skin contact with certain plants such as poison ivy or western poison oak, the latter of which are termed contact dermatitis. | It is effective in the treatment of benign prostatic hyperplasia. Dosages
Nafarelin is used to treat precocious puberty at a dosage of 1,600 to 1,800 μg per day. The 1,600 μg/day dosage is achieved by two sprays (400 μg total) into each nostril in the morning (four sprays, 800 μg total) and two sprays (400 μg total) into each nostril in the evening (four sprays, 800 μg total). If 1,600 μg/day is insufficient for adequate pubertal suppression, the 1,800 μg/day dosage can be used instead. This is achieved by three sprays (600 μg total) into alternating nostrils three times per day (nine sprays per day total). When administering the sprays, the head should be tilted back slightly and 30 seconds should elapse between each spray. A bottle of nafarelin nasal spray (brand name Synarel) lasts for about 7 days at a dosage of 1,600 μg/day.Nafarelin is used to treat endometriosis at lower dosages of 400 to 800 μg per day. This is achieved by one or two sprays (200 or 400 μg total) into alternating nostrils once in the morning and once in the evening (two to four sprays per day total). A bottle of nafarelin nasal spray (brand name Synarel) lasts for about 30 days at a dosage of 400 μg/day. Available forms
Nafarelin is available in the form of a 0.2% nasal spray for use one, two, or three times per day. Each bottle of nafarelin nasal spray (brand name Synarel) contains about 60 sprays delivering approximately 200 μg nafarelin in 100 μL solution per actuation. | 0-1
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Other uses
It 2008, ESPN reported that four NFL players were being suspended under the steroid policy as a result of taking bumetanide. It is sometimes used for weight loss because, as a diuretic, it removes water, but it also masks other drugs, including steroids, by diluting the contents of the users urine, yielding a lower concentration of filtered substances, which makes them less likely to be detected. Bumetanide was an undisclosed active ingredient in the over-the-counter weight loss supplement StarCaps, which was removed from the market after its presence was discovered by the United States Food and Drug Administration. Side effects
Common side effects include dizziness, low blood pressure, low blood potassium, muscle cramps, and kidney problems. Other serious side effects may include hearing loss and low blood platelets. A large observational study concluded that people with a sulfonamide antibiotic allergy may be allergic to sulfonamide non-antibiotics, such as bumetanide, but this is likely due to certain people being at an increased risk in general to developing allergic reactions rather than cross-reactivity between sulfonamide-containing drugs. In smaller studies, the lack of cross-reactivity between sulfonamide antibiotics and sulfonamide non-antibiotics has been demonstrated. Blood tests are recommended regularly for those on treatment. Safety during pregnancy and breastfeeding is unclear. Mechanism of action
Bumetanide is a loop diuretic and works by decreasing the reabsorption of sodium by the kidneys. The main difference between bumetanide and furosemide is in their bioavailability and potency. | About 60% of furosemide is absorbed in the intestine, and there are substantial inter- and intraindividual differences in bioavailability (range 10-90%). About 80% of bumetanide is absorbed, and its absorption does not change when it is taken with food. It is said to be a more predictable diuretic, meaning that the predictable absorption is reflected in a more predictable effect. Bumetanide is 40 times more potent than furosemide for people with normal renal function. Synthesis
Bumetanide is synthesized from 4-chlorobenzoic acid. In the first stage of synthesis, it undergoes sulfonylchlorination by chlorosulfonic acid, forming 4-chloro-3-chlorosulfonylbenzoic acid, which is further nitrated with nitric acid to 4-chloro-3-chlorosulfonyl-5-nitrobenzoic acid. Reacting this with ammonia gives 5-aminosulfonyl-4-chloro-3-nitrobenzoic acid, which when reacted with sodium phenolate is transformed into 5-amino-sulfonyl-3-nitro-5-phenoxybenzoic acid. Reduction of the nitro group in this product by hydrogen using a palladium on carbon catalyst gives 3-amino-5-aminosulfonyl-5-phenoxybenzoic acid. Finally, reacting this with butyl alcohol in the presence of sulfuric acid, followed by treatment with sodium hydroxide to hydrolyze the butyl ester, gives the desired bumetanide. Research
In the brain, bumetanide blocks the NKCC1 cation-chloride co-transporter, and thus decreases internal chloride concentration in neurons. In turn, this concentration change makes the action of GABA more hyperpolarizing, which may be useful for treatment of neonatal seizures, which quite often are not responsive to traditional GABA-targeted treatment, such as barbiturates. Bumetanide is therefore under evaluation as a prospective antiepileptic drug.The drug has also been studied as a treatment for autism. References
External links
"Bumetanide". Drug Information Portal. U.S. National Library of Medicine. | 11
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Although it might be described by some as a "degeneration", the term macular degeneration should be reserved for the specific disease "age-related macular degeneration". Very few affected individuals go completely blind from retinoschisis, but some sufferers have very limited reading vision and are "legally blind". Visual acuity can be reduced to less than 20/200 in both eyes. Individuals affected by XLRS are at an increased risk for retinal detachment and eye hemorrhage, among other potential complications. Retinoschisis causes acuity loss in the center of the visual field through the formation of tiny cysts in the retina, often forming a "spoke-wheel" pattern that can be very subtle. The cysts are usually only detectable by a trained clinician. In some cases vision cannot be improved by glasses, as the nerve tissue itself is damaged by these cysts. The National Eye Institute (NEI) of the National Institutes of Health (NIH) is conducting clinical and genetic studies of X-Linked Juvenile Retinoschisis. This study began in 2003 and as of 2018 is continuing to recruit patients. A better understanding of why and how XLRS develops might lead to improved treatments. Males diagnosed with X-linked juvenile retinoschisis and females who are suspected carriers may be eligible to participate. In addition to giving a medical history and submitting medical records, participants submit a blood sample and the NEI will perform a genetic analysis. There is no cost to participate in this study. Tractional retinoschisis
This may be present in conditions causing traction on the retina especially at the macula. | Penile pain refers to pain in the penis, human or otherwise. Common causes
Penile injury
Circumcision
Penile fracture
Priapism
Phimosis
Peyronies disease
Sexually transmitted infections
Chronic prostatitis/chronic pelvic pain syndrome
Cultural references
Priapus, a minor Greek god of fertility, is marked by his oversized, permanent erection, which gave rise to the medical term priapism. Some researchers believe the depiction of Priapus penis referred to a penile disease, and that paintings of Priapus were used to ward off the disease. See also
Genital pain
Vaginal pain
Testicular pain
List of the causes of genital pain
== References == | 0-1
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Once his condition stabilized, he continued to be put in the spa twice a day over the next few months. Drug therapies
Anti-inflammatories and analgesicsAnti-inflammatories are always used when treating acute case of laminitis, and include Nonsteroidal anti-inflammatory medications (NSAIDS), DMSO, pentoxpfylline, and cryotherapy. For analgesia, NSAIDs are often the first line of defense. Phenylbutazone is commonly used for its strong effect and relatively low cost. Flunixin (Banamine), ketofen, and others are also used. Nonspecific NSAIDs such as suxibuzone, or COX-2-specific drugs, such as firocoxib and diclofenac, may be somewhat safer than phenylbutazone in preventing NSAID toxicity such as right dorsal colitis, gastric ulcers, and kidney damage. However, firocoxib provides less pain relief than phenylbutazone or flunixin. Care must be taken that pain is not totally eliminated, since this will encourage the horse to stand and move around, which increases mechanical separation of the laminae.Pentafusion, or the administration of ketamine, lidocaine, morphine, detomidine, and acepromazine at a constant rate of infusion, may be of particular benefit to horses suffering from laminitis. Epidurals may also be used in hind-limb laminitis. VasodilatorsVasodilators are often used with the goal of improving laminar blood flow. However, during the developmental phases of laminitis, vasodilation is contraindicated, either through hot water or vasodilatory drugs. Systemic acepromazine as a vasodilator with the fringe benefit of mild sedation which reduces the horse/ponys movements and thus reduces concussion on the hooves, may be beneficial after lamellar damage has occurred, although no effects on laminar blood flow with this medication have been shown. | Myxoid variant: Loose, basophilic matrix, with thick fibrous septa, and foamy histiocytes
Lipoma-like variant: Univacuolated lipocytes, with only isolated hibernoma cells
Spindle cell variant: Spindle cell lipoma combined with hibernoma
Histochemistry
Oil Red O-positive droplets of cytoplasmic lipid can be seen in most cases
Immunohistochemistry
The neoplastic cells are S100 protein positive (approximately 80%), and show membrane and vacuole CD31 immunoreactivity. Uncoupling protein 1 (UCP1), a unique brown fat mitochondrial protein, is also positive. Cytogenetics
There are structural rearrangements of 11q13-21, which are considered most characteristic. This alteration can be detected by metaphase fluorescent in situ hybridization (FISH). MEN1 gene (11q13.1) is most frequently deleted, while GARP gene (11q13.5) may also be involved. Cytology
The fine needle aspiration smears show small, round, brown fat-like cells, with uniform, small cytoplasmic vacuoles and regular, small, round nuclei. There is usually a rich vascular background of branching capillaries. It is not uncommon to also have mature fat cells. Differential diagnoses
It is important to separate hiberoma from adult rhabdomyoma, a granular cell tumor and a true liposarcoma. Classification
This lesion has been called a fetal lipoma, lipoma of embryonic fat or a lipoma of immature fat. Management
Complete surgical excision is the treatment of choice, associated with an excellent long term clinical outcome. Epidemiology
The tumor is rare, affecting adults in the 4th decade most commonly. Patients are usually younger than those who present with a lipoma. There is a slight male predominance. Hibernoma are most commonly identified in the subcutaneous and muscle tissue of the head and neck region (shoulders, neck, scapular), followed by thigh, back, chest, abdomen, and arms. | 0-1
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Treatment
Technique
The complete or partial absence of the pectoralis muscle is the malformation that defines Poland syndrome. It can be treated by inserting a custom implant designed by CAD (computer aided design). A 3D reconstruction of the patients chest is done using an implant shaped from a medical scan and designed to be perfectly adapted to the anatomy. The implant is made of medical grade silicone rubber. The treatment is purely cosmetic and does not restore the patients imbalanced upper body strength.The Poland syndrome malformations are morphological, so correction by custom implant is the first-line treatment. This technique allows a wide variety of patients to be treated with good outcomes. Poland Syndrome can be associated with bones, subcutaneous and mammary atrophy: the first, as for pectus excavatum, is successfully corrected by a custom implant, while the others can require surgical intervention such as lipofilling or silicone breast implant, in a second operation. Surgery
The surgery takes place under general anaesthesia and lasts less than 1 hour. The surgeon prepares the locus to the size of the implant after performing an 8-centimetre (3.1 in) axillary incision, then inserts the implant beneath the skin. The closure is made in two planes.The implant replaces the pectoralis major muscle, thus enabling the thorax to be symmetrical and, in women, the breast as well. If necessary, especially in the case of women, a second operation will complement the result by the implantation of a breast implant and / or lipofilling.Lipomodelling is progressively used in the correction of breast and chest wall deformities. | Clarkson published his series of three cases and named the syndrome after Poland in his article. Notable cases
TV presenter Jeremy Beadle (1948–2008) was known for having this condition. His Poland syndrome manifested itself in the form of his disproportionately small right hand. Olympic boxer Jérôme Thomas is also affected by Poland syndrome, as his left arm and hand are significantly shorter and smaller than his right. Thomas also lacks a left pectoral muscle. PGA Tour golfer Bryce Molder has Poland syndrome, with an absent left pectoral muscle and a small left hand. Several surgeries in his childhood repaired syndactyly on the left hand. Actor Ted Danson, famous for starring in the TV show Cheers, admitted he had the condition in 2000 to Orange Coast magazine and said that he was bullied as a child because of it. Formula One World Champion Fernando Alonso is affected by Poland syndrome; he is missing the right pectoral muscle. Cricketer Lewis Hatchett was born with Poland syndrome. Australian Paralympian Mathew Silcocks is affected by Poland syndrome. Hailey Dawson of Nevada (born 2010) has a missing right pectoral muscle and is missing three fingers on her right hand due to the condition. She has thrown out the ceremonial first pitch at all 30 Major League Baseball parks, using a 3D-printed robotic right hand fitted for her by engineers at the University of Nevada, Las Vegas. Actor Gary Burghoff, best known for the television series M*A*S*H, has Poland syndrome manifesting in brachydactyly on his left hand. | 11
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Some physicians are also leading a movement to begin prescribing it out of the emergency department (ED), as some small studies have shown ED-initiated Suboxone to be effective with people more likely to remain in addiction treatment compared to those either referred to addiction treatment programs or those receiving just a brief intervention in the department.Access to Suboxone can be limited due to varying prior authorization requirements across different insurers. Prior authorization is used by insurance companies to limit certain medications use by requiring approval before the insurance company will pay for them. This can influence a persons financial access and adherence. Financial access is determined through prior authorization approval, which the prescriber must request before the person can start the medication. The time it takes to have the request approved can delay the person in starting the medication. The prior authorization process can also affect adherence, because the approval is needed for every prescription. This presents the potential for a gap in treatment and withdrawal symptoms as the person waits for approval. Several insurance companies, as well as Medicaid in various states, have removed the use of prior authorization for Suboxone in an attempt to increase access to this treatment. Controversies
In July 2019, the British company Reckitt Benckiser Group (RB Group) and its current/former affiliated entities (notably Indivior, which split from RB Group in 2014) settled with the US Department of Justice (DOJ) regarding the sale and marketing of brand name Suboxone (buprenorphine/naloxone). | The most common side effects (in order of most to least common) of sublingual tablets include headaches, opioid withdrawal syndrome, pain, nausea, increased sweating, and difficulty sleeping. The most common side effects seen in film formulations are tongue pain, decreased sensation and redness in the mouth, headache, nausea, vomiting, excessive sweating, constipation, signs and symptoms of opioid withdrawal, sleeping difficulties, pain, and swelling of the extremities.Buprenorphine/naloxone has a milder side effect profile than methadone and limited respiratory effects, due to both agonist/antagonist effects. But buprenorphine/naloxone may be less safe than methadone in people with stable liver disease, since it can elevate liver enzymes. Dependence and withdrawal
When taken in excess, buprenorphine/naloxone can produce dysphoric symptoms for non opioid-dependent/tolerant people because buprenorphine is a partial opioid agonist. The sublingual formulation of the buprenorphine/naloxone combination was designed to reduce the potential to inject the medication in comparison to buprenorphine alone. If the combination is taken sublingually, as directed, the addition of naloxone does not diminish buprenorphines effects. When an opioid-dependent person dissolves and injects a combination sublingual tablet, a withdrawal effect may be triggered because of naloxones high parenteral bioavailability. While this mechanism can act to deter intravenous injection, the Suboxone formulation can still produce an opioid agonist "high" if used sublingually by non-dependent persons, leading to opioid dependence. Interactions
Buprenorphines sedating/narcotic effect is increased by other sedating substances, such as other opioids, benzodiazepines, first-generation antihistamines, alcohol, and antipsychotics. | 11
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In Stage 5 chronic kidney disease patients renal replacement therapy may become necessary. History
Analgesics are a class of medications widely used in the treatment of pain. They include aspirin and other non-steroidal anti-inflammatory drugs (NSAIDs), as well as the antipyretics paracetamol (known as acetaminophen in the United States) and phenacetin. Introduced in the late 19th century, phenacetin was once a common component of mixed analgesics in parts of Europe, Australia, and the United States. These analgesics contained aspirin or other NSAIDs combined with phenacetin, paracetamol, or salicylamide, and caffeine or codeine.In the 1950s, Spühler and Zollinger reported an association between kidney injury and the chronic use of phenacetin. They noted that chronic users of phenacetin had an increased risk of developing specific kidney injuries, namely renal papillary necrosis and chronic interstitial nephritis. This condition was dubbed analgesic nephropathy and was attributed to phenacetin, although no absolute causative role was demonstrated. With further reports of the increased risk of kidney injury with prolonged and excessive phenacetin use, however, phenacetin was banned in several countries between the 1960s and 1980s.As the use of phenacetin declined, so too did the prevalence of analgesic nephropathy as a cause of end-stage kidney disease. Data from Switzerland, for example, demonstrated a decline in the prevalence of analgesic nephropathy among people with end-stage kidney disease, from 28% in 1981 to 12% in 1990. | After growth of a few millimeters has occurred, the cysts detach from the parent tubule, this detachment induced by excessive proliferation of tubular epithelium or excessive fluid secretions. Diagnosis
Diagnosis includes imaging with ultrasound, CT and/or MRI. The least expensive, non-invasive, and most reliable method is ultrasonography but smaller cysts may escape detection, while the resolution of CT and MRI will enable smaller cysts to be captured. However, the increased complexity and expense of CT and MRI is usually reserved for higher risk situations. MRI can be used to monitor the development of cysts and growth of kidneys.Genetic test can be applicable to those who have a family history of PKD but is expensive and fails to detect PKD in 15% of cases where it is present. Antenatal scans
Many forms of cystic kidney disease can be detected in children prior to birth. Abnormalities which only affect one kidney are unlikely to cause a problem with the healthy arrival of a baby. Abnormalities which affect both kidneys can have an effect on the babys amniotic fluid volume which can in turn lead to problems with lung development. Some forms of obstruction can be very hard to differentiate from cystic renal disease on early scans. Treatment
The goal of treatment is to manage the disease and its symptoms, and to avoid or delay complications. Options include pain medication (except ibuprofen and other ‘non-steroidal anti-inflammatory agents (NSAID’s)’ which may worsen kidney function), low protein and sodium diet, diuretics, antibiotics to treat urinary tract infection, or interventions to drain cysts. | 0-1
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The examination of the larynx requires some expertise, which may require specialist referral.The physical exam includes a systematic examination of the whole patient to assess general health and to look for signs of associated conditions and metastatic disease. The neck and supraclavicular fossa are palpated to feel for cervical adenopathy, other masses, and laryngeal crepitus. The oral cavity and oropharynx are examined under direct vision. The larynx may be examined by indirect laryngoscopy using a small angled mirror with a long handle (akin to a dentists mirror) and a strong light. Indirect laryngoscopy can be highly effective, but requires skill and practice for consistent results. For this reason, many specialist clinics now use fibre-optic nasal endoscopy where a thin and flexible endoscope, inserted through the nostril, is used to clearly visualise the entire pharynx and larynx. Nasal endoscopy is a quick and easy procedure performed in clinic. Local anaesthetic spray may be used.If there is a suspicion of cancer, biopsy is performed, usually under general anaesthetic. This provides histological proof of cancer type and grade. If the lesion appears to be small and well localised, the surgeon may undertake excision biopsy, where an attempt is made to completely remove the tumour at the time of first biopsy. In this situation, the pathologist will not only be able to confirm the diagnosis, but can also comment on the completeness of excision, i.e., whether the tumour has been completely removed. | Intractable may refer to:
Intractable conflict, a form of complex, severe, and enduring conflict
Intractable pain, pain which cannot be controlled/cured by any known treatment
Intractability (complexity), in computational complexity theory | 0-1
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This results in bradycardia, vasodilation, flushing, pupillary constriction and nasal stuffiness above the spinal lesion, while there is piloerection, pale and cool skin below the lesion due to the prevailing sympathetic outflow.Initial treatment involves sitting the patient upright, removing any constrictive clothing (including abdominal binders and support stockings), rechecking blood pressure frequently, and then checking for and removing the inciting issue, which may require urinary catheterization or bowel disimpaction. If systolic blood pressure remains elevated (over 150 mm Hg) after initial steps, fast-acting short-duration antihypertensives are considered, while other inciting causes must be investigated for the symptoms to resolve.Prevention of AD involves educating the patient, family and caregivers of the precipitating cause, if known, and how to avoid it, as well as other triggers. Since bladder and bowel are common causes, prevention involves routine bladder and bowel programs and urological follow-up for cystoscopy/urodynamic studies. Signs and symptoms
This condition is distinct and usually episodic, with the people potentially experiencing remarkably high blood pressure, intense headaches, profuse sweating, facial erythema, goosebumps, nasal stuffiness, a "feeling of doom" or apprehension, and blurred vision. An elevation of 20 mm Hg over baseline systolic blood pressure, with a potential source below the neurological level of injury, meets the current definition of dysreflexia. Complications
Autonomic dysreflexia can become chronic and recurrent, often in response to longstanding medical problems like soft tissue pressure injuries or hemorrhoids. Long term therapy may include alpha blockers or calcium channel blockers.Complications of severe acute hypertension can include seizures, pulmonary edema, myocardial infarction, or cerebral hemorrhage. | References
Further reading
== External links == | 11
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There are numerous examples of people in creative or artistic careers, such as composers, novelists and filmmakers, developing new ideas through daydreaming. Similarly, research scientists, mathematicians and physicists have developed new ideas by daydreaming about their subject areas. Hallucination
A hallucination, in the broadest sense of the word, is a perception in the absence of a stimulus. In a stricter sense, hallucinations are perceptions in a conscious and awake state, in the absence of external stimuli, and have qualities of real perception, in that they are vivid, substantial, and located in external objective space. The latter definition distinguishes hallucinations from the related phenomena of dreaming, which does not involve wakefulness. Nightmare
A nightmare is an unpleasant dream that can cause a strong negative emotional response from the mind, typically fear or horror, but also despair, anxiety and great sadness. The dream may contain situations of danger, discomfort, psychological or physical terror. Sufferers usually awaken in a state of distress and may be unable to return to sleep for a prolonged period of time. Night terror
A night terror, also known as a sleep terror or pavor nocturnus, is a parasomnia disorder that predominantly affects children, causing feelings of terror or dread. Night terrors should not be confused with nightmares, which are bad dreams that cause the feeling of horror or fear. | Vesical tenesmus refers to the feeling of incomplete emptying of the bladder following urination. When the word tenesmus is used without modification, it usually refers to rectal tenesmus. Vesical tenesmus is caused by urogenital diaphragm muscle spasms. See also
Post-void dribbling
Prostate
Rectal tenesmus
References
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Athletes with tricuspid regurgitation also had enlarged right atrium diameter when compared to control group. See also
Heart valves
References
Sources
Mestres, Carlos A.; Bernal, Jose M.; Pomar, Jose L. (2016). "Surgical Treatment of Tricuspid Valve Diseases". In Frank Sellke; Pedro J. del Nido (eds.). Sabiston and Spencer Surgery of the Chest. ISBN 978-0-323-24126-7. Further reading
Haddad, F; Doyle, R; Murphy, D. J; Hunt, S. A (2008). "Right Ventricular Function in Cardiovascular Disease, Part II: Pathophysiology, Clinical Importance, and Management of Right Ventricular Failure". Circulation. 117 (13): 1717–1731. doi:10.1161/CIRCULATIONAHA.107.653584. PMID 18378625. Desai, Ravi R; Vargas Abello, Lina Maria; Klein, Allan L; Marwick, Thomas H; Krasuski, Richard A; Ye, Ying; Nowicki, Edward R; Rajeswaran, Jeevanantham; Blackstone, Eugene H; Pettersson, Gösta B (2013). "Tricuspid regurgitation and right ventricular function after mitral valve surgery with or without concomitant tricuspid valve procedure". The Journal of Thoracic and Cardiovascular Surgery. 146 (5): 1126–1132.e10. doi:10.1016/j.jtcvs.2012.08.061. PMC 4215162. PMID 23010580. Badano, Luigi P; Muraru, Denisa; Enriquez-Sarano, Maurice (2013). "Assessment of functional tricuspid regurgitation". European Heart Journal. 34 (25): 1875–1885. doi:10.1093/eurheartj/ehs474. PMID 23303656. al. ], Hugh D. Allen ... [et; Shaddy, Robert E.; Feltes, Timothy F. (2013). Moss and Adams heart disease in infants, children, and adolescents : including the fetus and young adult (8th ed.). Philadelphia: Wolters Kluwer Health/Lippincott Williams & Wilkins. ISBN 9781451118933. == External links == | Antifungal drugs are the first line of defense to kill the agents causing phaeohyphomycosis, but despite the significant progress made in the last two decades and a 30% increase in available antifungal drugs since 2000, many drugs are not effective against black fungi. Diseases caused by black fungi are hard to treat because the fungi are very difficult to kill. This high resilience may be contributed to the presence of melanin in their cell walls, as well as the greater similarity to host cells which are both eukaryotes than other pathogens such as bacteria or viruses. Current antifungal agents the fungi are not resistant to are posaconazole, voriconazole, and azole isavuconazole.In 2006, a free-living Eastern box turtle, Terrapene carolina carolina, was found with a form of phaeohyphomycosis and was brought in the Wildlife Center of Virginia. Its symptom was swelling of the right hindfoot; it was diagnosed as having chromomycosis by histopathology. The center provided a series of antimicrobial treatments and a one-month course of 1 mg itraconazole, administered orally once a day. The eastern box turtle was euthanized due to further complications and the caretakers’ belief that the turtle would not be able to survive if placed back in the wild. A recent case of a form of phaeohyphomycosis infection was found in a dog in 2011. The Journal of the American Veterinary Medical Association published a case study in which researchers successfully managed an intracranial phaeohyphomycotic fungal granuloma in a one-year-old male Boxer dog. | 0-1
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These cells play an important role in the development of the craniofacial appearance, and loss of one copy of treacle affects the cells ability to form the bones and tissues of the face. Other mutations
POLR1C and POLR1D mutations are responsible for a minority of cases of Treacher Collins. POLR1C is found on chromosome 6 at position 6q21.2 and POLR1D is found on chromosome 13 at position 13q12.2. Those genes code for a protein subunits shared between RNA polymerase I and III. Both of these polymerases are important for ribosome biogenesis. Diagnosis
Genetic counseling
TCS is inherited in an autosomal dominant manner and the penetrance of the affected gene is almost complete. Some recent investigations, though, described some rare cases in which the penetrance in TCS was not complete. Causes may be a variable expressivity, an incomplete penetrance or germline mosaicism. Only 40% of the mutations are inherited. The remaining 60% are a result of a de novo mutation, where a child has a new mutation in the responsible gene and did not inherit it from either parent. In the outcome of the disease, inter- and intrafamilial variability occurs. This suggests that when an affected child is born, it is important to investigate the parents to determine whether the affected gene is present, because the parent could have a mild form of the disease that has not been diagnosed. In this case, the risk of having another affected child is 50%. If the parents do not have the affected gene, the recurrence risk appears to be low. | Mutations in TCOF1 and POLR1D cause the autosomal dominant form of Treacher Collins, and mutations in POLR1C cause the autosomal recessive form. TCOF1
TCOF1 is the primary gene associated with TCS, a mutation in this gene being found in 90–95% of the individuals with TCS. However, in some individuals with typical symptoms of TCS, mutations in TCOF1 have not been found. Investigation of the DNA has resulted in the identification of the kind of mutations found in TCOF1. The majority of mutations are small deletions or insertions, though splice site and missense mutations also have been identified.Mutation analysis has unveiled more than 100 disease-causing mutations in TCOF1, which are mostly family-specific mutations. The only recurrent mutation accounts for about 17% of the cases.TCOF1 is found on the 5th chromosome in the 5q32 region. It codes for a relatively simple nucleolar protein called treacle, that is thought to be involved in ribosome assembly. Mutations in TCOF1 lead to haploinsufficiency of the treacle protein. Haploinsufficiency occurs when a diploid organism has only one functional copy of a gene, because the other copy is inactivated by a mutation. The one normal copy of the gene does not produce enough protein, causing disease. Haploinsufficiency of the treacle protein leads to a depletion of the neural crest cell precursor, which leads to a reduced number of crest cells migrating to the first and second pharyngeal arches. | 11
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Signs and symptoms
Obsessive–compulsive personality disorder (OCPD) is marked by an excessive obsession with rules, lists, schedules, and order; a need for perfectionism that interferes with efficiency and the ability to complete tasks; a devotion to productivity that hinders interpersonal relationships and leisure time; rigidity and zealousness on matters of morality and ethics; an inability to delegate responsibilities or work to others; restricted functioning in interpersonal relationships; restricted expression of emotion and affect; and a need for control over ones environment and self.Some of OCPDs symptoms are persistent and stable, whilst others are unstable. The obsession with perfectionism, reluctance to delegate tasks to others, and rigidity and stubbornness are stable symptoms. On the other hand, the symptoms that were most likely to change over time were the miserly spending style and the excessive devotion to productivity. This discrepancy in the stability of symptoms may lead to mixed results in terms of the course of the disorder, with some studies showing a remission rate of 58% after a 12-month period, whilst others suggesting that the symptoms are stable and may worsen with age. Attention to order and perfection
People with OCPD tend to be obsessed with controlling their environments; to satisfy this need for control, they become preoccupied with trivial details, lists, procedures, rules, and schedules.This preoccupation with details and rules makes the person unable to delegate tasks and responsibilities to other people unless they submit to their exact way of completing a task because they believe that there is only one correct way of doing something. | In autoimmune polyendocrinopathy-candidiasis-ectodermal dystrophy (APECED) also autoimmunity and infections coexist: organ-specific autoimmune manifestations (e.g., hypoparathyroidism and adrenocortical failure) and chronic mucocutaneous candidiasis. Finally, IgA deficiency is also sometimes associated with the development of autoimmune and atopic phenomena. Causes
The cause of immunodeficiency varies depending on the nature of the disorder. The cause can be either genetic or acquired by malnutrition and poor sanitary conditions. Only for some genetic causes, the exact genes are known. Treatment
Available treatment falls into two modalities: treating infections and boosting the immune system. Prevention of Pneumocystis pneumonia using trimethoprim/sulfamethoxazole is useful in those who are immunocompromised. In the early 1950s Immunoglobulin(Ig) was used by doctors to treat patients with primary immunodeficiency through intramuscular injection. Ig replacement therapy are infusions that can be either subcutaneous or intravenously administrated, resulting in higher Ig levels for about three to four weeks, although this varies with each patient. Prognosis
Prognosis depends greatly on the nature and severity of the condition. Some deficiencies cause early mortality (before age one), others with or even without treatment are lifelong conditions that cause little mortality or morbidity. Newer stem cell transplant technologies may lead to gene based treatments of
debilitating and fatal genetic immune deficiencies. Prognosis of acquired immune deficiencies depends on avoiding or treating the causative agent or
condition (like AIDS). See also
Acquired immune deficiency syndrome (AIDS)
Immune disorder
Autoimmune disease, immune response to self-proteins
Allergy, immune response to harmless non-self proteins
Histamine
Immunosenescence, age-associated immune deficiency
Steroids, commonly administered drugs like prednisone that suppress the immune system
Human genetic enhancement
Immune system
Immunology
References
External links
Immune Deficiency Foundation
The European Society of Immunodeficiencies | 0-1
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18F-fluorodeoxyglucose positron emission tomography/computed tomography (FDG-PET/CT)has become a widely used imaging tool in patients with suspected Large Vessel Vasculitis, due to the enhanced glucose metabolism of inflamed vessel walls. The combined evaluation of the intensity and the extension of FDG vessel uptake at diagnosis can predict the clinical course of the disease, separating patients with favourable or complicated progress. Acute onset of vasculitis-like symptoms in small children or babies may instead be the life-threatening purpura fulminans, usually associated with severe infection.In this table: ANA = antinuclear antibodies, CRP = C-reactive protein, ESR = erythrocyte sedimentation rate, dsDNA = double-stranded DNA, ENA = extractable nuclear antigens, RNP = ribonucleoproteins; VDRL = Venereal Disease Research Laboratory
Treatment
Treatments are generally directed toward stopping the inflammation and suppressing the immune system. Typically, corticosteroids such as prednisone are used. Additionally, other immune suppression medications, such as cyclophosphamide and others, are considered. In case of an infection, antimicrobial agents including cephalexin may be prescribed. Affected organs (such as the heart or lungs) may require specific medical treatment intended to improve their function during the active phase of the disease. References
== External links == | After decades of use, Squibb, the manufacturer, voluntarily withdrew the Delalutin product in the United States in 1999. Renewed interest in OHPC in the United States was sparked with a large NIH-sponsored study in 2003 that found that OHPC reduced the risk of premature birth in selected at-risk pregnant women. With follow-up data showing no evidence of harmful effects on the offspring, the FDA approved the medication, as sponsored by KV Pharmaceutical as Makena, as an orphan drug in February 2011 to reduce the risk of premature birth in women prior to 37 weeks gestation with a single fetus who had at least one previous premature birth. Society and culture
Generic names
Hydroxyprogesterone caproate is the generic name of OHPC and its INN, USAN, BANM, and JAN, while hydroxyprogesterone hexanoate was its former BANM.OHPC is often mislabeled as and confused with progesterone and 17α-hydroxyprogesterone. It should also not be confused with hydroxyprogesterone acetate, hydroxyprogesterone heptanoate, or medroxyprogesterone acetate. Brand names
OHPC is marketed throughout the world under a variety of brand names including Proluton, Proluton Depot, and Makena (US), among many others. It was also formerly marketed under brand names including Delalutin, Prodrox, and Hylutin among others, but these formulations have since been discontinued. It has been marketed under the brand names Gravibinon and Injectable No. 1 (or Chinese Injectable No. 1) in combination with estradiol valerate and under the brand name Primosiston in combination with estradiol benzoate. Availability
OHPC is marketed in the United States and throughout Europe, Asia, and Central and South America. | 0-1
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Brain death can sometimes be difficult to differentiate from other medical states such as barbiturate overdose, acute alcohol poisoning, sedative overdose, hypothermia, hypoglycemia, coma, and chronic vegetative states. Some comatose patients can recover to pre-coma or near pre-coma level of functioning, and some patients with severe irreversible neurological dysfunction will nonetheless retain some lower brain functions, such as spontaneous respiration, despite the losses of both cortex and brain stem functionality. Such is the case with anencephaly. Brain electrical activity can stop completely, or drop to such a low level as to be undetectable with most equipment. An EEG will therefore be flat, though this is sometimes also observed during deep anesthesia or cardiac arrest. Although in the United States a flat EEG test is not required to certify death, it is considered to have confirmatory value. In the UK it is not considered to be of value because any continuing activity it might reveal in parts of the brain above the brain stem is held to be irrelevant to the diagnosis of death on the Code of Practice criteria.The diagnosis of brain death is often required to be highly rigorous, in order to be certain that the condition is irreversible. Legal criteria vary, but in general require neurological examinations by two independent physicians. | Atrichia with papular lesions (a.k.a. "Papular atrichia") is a diffuse hair loss caused by an abnormality of the human homologue of the mouse hairless gene. : 635 : 762 It is associated with HR. See also
Cicatricial alopecia
List of cutaneous conditions
References
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Möbius syndrome is a rare congenital neurological disorder which is characterized by facial paralysis and the inability to move the eyes from side to side. Most people with Möbius syndrome are born with complete facial paralysis and cannot close their eyes or form facial expressions. Limb and chest wall abnormalities sometimes occur with the syndrome. People with Möbius syndrome have normal intelligence, although their lack of facial expression is sometimes incorrectly taken to be due to dullness or unfriendliness. It is named for Paul Julius Möbius, a German neurologist who first described the syndrome in 1888. In 1994, the "Moebius Syndrome Foundation" was founded, and later that year the first "Moebius Syndrome Foundation Conference" was held in Los Angeles. A charity for Möbius syndrome was set up and registered in the UK in 1999 by Linda Anderson from Tyne and Wear, whose son had been born with the condition in 1980. She campaigned for many years, held conferences and gave medical speeches in America before she had to step away from the charity because of ill health. Signs and symptoms
People with Möbius syndrome are born with facial paralysis and the inability to move their eyes laterally. Often, their upper lip is retracted due to muscle shrinkage. Occasionally, the cranial nerves V and VIII are affected. | Sometimes called smile surgery by the media, muscle transfers grafted from the thigh to the corners of the mouth can be performed to provide the ability to smile. Although "smile surgery" may provide the ability to smile, the procedure is complex and can take twelve hours for each side of the face. Also, the surgery cannot be considered a "cure" for Möbius syndrome, because it does not improve the ability to form other facial expressions. Epidemiology
It is estimated that there are, on average, 2 to 20 cases of Möbius syndrome per million births. Although its rarity often leads to late diagnosis, infants with this disorder can be identified at birth by a "mask-like" lack of expression that is detectable during crying or laughing and by an inability to suck while nursing because of paresis (palsy) of the sixth and seventh cranial nerves. Also, because a person with Möbius syndrome cannot follow objects by moving their eyes from side to side, they turn their head instead. Society and culture
Literature and media with mentions of Möbius syndrome include:
The protagonist of the novel Painted by Eliza Wyatt and Christian Leffler has Möbius syndrome. In the second season of Scream Queens, Daira Janessen (Riley McKenna Weinstein), also known as Chanel 8, has Möbius syndrome. In the first season of American medical drama The Good Doctor, a teenage patient is depicted with Möbius syndrome. In the BBC TV series Face, Loraine Deveney, a Möbius syndrome patient, was portrayed as a successful example of "smile surgery" performed by R. M. Zuker, M.D. | 11
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s.2 of the 1953 Act requires that registration of a birth takes place within 42 days of the birth except where an inquest takes place or the child has been "found exposed" in which latter case the time limit runs from the time of finding. Extracts from the register of stillbirths are restricted to those who have obtained consent from the Registrar General for England and Wales. Scotland
Section 56(1) of the Registration of Births, Deaths and Marriages (Scotland) Act 1965 (as amended) contains the definition:"still-born child" means a child which has issued forth from its mother after the twenty-fourth week of pregnancy and which did not at any time after being completely expelled from its mother breathe or show any other signs of life, and the expression "still-birth" shall be construed accordinglys.21(1) of the same Act requires that:Except so far as otherwise provided by this section or as may be prescribed, the provisions of this Part of this Act shall, so far as applicable, apply to still-births in like manner as they apply to births of children born alive.In the general case, s.14 of the Act requires that a birth has to be registered within 21 days of the birth or of the child being found. Unlike the registers for births, marriages, civil partnerships and deaths, the register of still-births is not open to public access and issue of extracts requires the permission of the Registrar General for Scotland. | Lancet series on stillbirth 2016 | 11
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Preliminary research showed that health providers might have less empathy for black patients and underestimated their pain levels, resulting in treatment delays. Minorities may experience a language barrier, limiting the high level of engagement between the person with pain and health providers for treatment. Perceptions of injustice
Similar to the damaging effects seen with catastrophizing, perceived injustice is thought to contribute to the severity and duration of chronic pain. Pain-related injustice perception has been conceptualized as a cognitive appraisal reflecting the severity and irreparability of pain- or injury-related loss (e.g., "I just want my life back"), and externalizing blame and unfairness ("I am suffering because of someone elses negligence."). It has been suggested that understanding problems with top down processing/cognitive appraisals can be used to better understand and treat this problem. Chronic pain and COVID-19
COVID-19 has disrupted the lives of many, leading to major physical, psychological and socioeconomic impacts in the general population. Social distancing practices defining the response to the pandemic alter familiar patterns of social interaction, creating the conditions for what some psychologists are describing as a period of collective grief. Individuals with chronic pain tend to embody an ambiguous status, at times expressing that their type of suffering places them between and outside of conventional medicine. With a large proportion of the global population enduring prolonged periods of social isolation and distress, one study found that people with chronic pain from COVID-19 experienced more empathy towards their suffering during the pandemic. | Research
There are ongoing efforts to develop sublingual epinephrine to treat anaphylaxis. Trials of sublingual epinephrine, currently called AQST-108 (dipivefrin) and sponsored by Aquestive Therapeutics, are in phase 1 trials as of December 2021. Subcutaneous injection of the anti-IgE antibody omalizumab is being studied as a method of preventing recurrence, but it is not yet recommended. References
External links
Anaphylaxis at Curlie
National Institute for Health and Clinical Excellence. Clinical guideline 134: Anaphylaxis: assessment to confirm an anaphylactic episode and the decision to refer after emergency treatment for a suspected anaphylactic episode. London, 2011. and Anaphylaxis pathway
"Anaphylaxis". MedlinePlus. U.S. National Library of Medicine. | 0-1
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Motion studies have revealed that when a woman runs, more than 50% of the breasts total movement is vertical, 22% is side-to-side, and 27% is in-and-out. A 2007 study found that encapsulation-type sports bras, in which each cup is separately molded, are more effective than compression-type bras, which press the breasts close to the body, at reducing total breast motion during exercise. Encapsulation bras reduce motion in two of the three planes, while compression bras reduce motion in only one plane. Previously, it was commonly believed that a woman with small to medium-size breasts benefited most from a compression-type sports bra, and women with larger breasts need an encapsulation-type sports bra. Mechanism
Anatomically, a females breasts do not contain any muscle but are composed of soft, glandular tissue. Breasts are composed of mammary glands, milk ducts, adipose tissue (fat tissue) and Coopers ligaments. Mammary glands remain relatively constant throughout life. Fat tissue surrounds the mammary glands, and its volume will normally vary throughout life. Although the exact mechanisms that determine breast shape and size are largely unknown, the amount and distribution of fat tissue and, to a lesser extent, mammary tissue, cause variations in breast size, shape and volume. Some experts believe Coopers ligaments, which are connective tissue with the breast, provide some support within breasts, but theres no agreement on whether they provide support or simply divide breast tissue into compartments. Treatment
Bras
Since breasts are an external organ and do not contain muscle, exercise cannot improve their shape. | The study results were presented at a conference of the American Society of Plastic Surgeons.According to Rinkers research, there are several key factors. A history of cigarette smoking "breaks down a protein in the skin called elastin, which gives youthful skin its elastic appearance and supports the breast." The number of pregnancies was strongly correlated with ptosis, with the effects increasing with each pregnancy. As most women age, breasts naturally yield to gravity and tend to sag and fold over the inframammary crease, the lower attachment point to the chest wall. This is more true for larger-breasted women. The fourth reason was significant weight gain or loss (greater than 50 pounds (23 kg)). Other significant factors were higher body mass index and larger bra cup size.In Rinkers study, 55% of respondents reported an adverse change in breast shape after pregnancy. Many women mistakenly attribute the changes and their sagging breasts to breastfeeding, and as a result some are reluctant to nurse their infants. Research shows that breastfeeding is not the factor that many thought it was. Rinker concluded that "Expectant mothers should be reassured that breastfeeding does not appear to have an adverse effect upon breast appearance." Also discounted as causes affecting ptosis are weight gain during pregnancy and lack of participation in regular upper body exercise. Effect of vigorous exercise
When running, breasts may move three-dimensionally: vertically, horizontally and laterally, in an overall figure-8 motion. Unrestrained movement of large breasts may contribute to sagging over time. | 11
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Obstetric: Pseudo-sinusoidal fetal heart rhythm.Other possible, but rare side effects include speech difficulty, urinary urgency, blurred vision, flushing and warmth. A 2014 Cochrane Systematic Review by Schnabel et al., concluded that due to limited data, analysis of adverse events for children treated with nalbuphine compared to other opioids or placebo for postoperative pain, could not be definitively reported. Overdose
In case of overdose or adverse reaction, the immediate intravenous administration of naloxone (Narcan) is a specific antidote. Oxygen, intravenous fluids, vasopressors and other supportive measures should be used as indicated. Pharmacology
Pharmacodynamics
Nalbuphine is a semisynthetic mixed agonist/antagonist opioid modulator of the phenanthrene or morphinan series. It is structurally related to the widely used opioid antagonists naloxone and naltrexone, and to the potent opioid analgesic oxymorphone. Nalbuphine binds with high affinity to the MOR and KOR, and has relatively low affinity for the DOR. It behaves as a moderate-efficacy partial agonist (or mixed agonist/antagonist) of the MOR and as a high-efficacy partial agonist of the KOR. Nalbuphine has weak or no affinity for the sigma receptor(s) (e.g., Ki > 100,000 nM).Nalbuphine is said to be more morphine-like at lower doses. However at higher doses, it produces more sedation, drunkenness, dysphoria, and dissociation. As such, its effects are dose-dependent. Such effects include sedation (21–36%), dizziness or vertigo (5%), lightheadedness (1%), anxiety (<1%), dysphoria (<1%), euphoria (<1%), confusion (<1%), hallucinations (<1%), depersonalization (1%), unusual dreams (<1%), and feelings of "unreality" (<1%).Nalbuphine is a potent analgesic. | Penicillin-binding proteins are bacterial proteins that help to catalyze the last stages of peptidoglycan synthesis, which is needed to maintain the cell wall. They remove the D-alanine from the precursor of the peptidoglycan. The lack of synthesis causes the bacteria to lyse because they also continually break down their cell walls. Cefazolin is bactericidal, meaning it kills the bacteria rather than inhibiting their growth. Cost
Cefazolin is relatively inexpensive. Trade names
It was initially marketed by GlaxoSmithKline under the trade name Ancef.Other trade names include: Cefacidal, Cefamezin, Cefrina, Elzogram, Faxilen, Gramaxin, Kefol, Kefzol, Kefzolan, Kezolin, Novaporin, Reflin, Zinol, and Zolicef. References
External links
MedlinePlus Drug Information: Cefazolin Sodium Injection. | 0-1
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Phthalates in certain types of PVC are associated with asthma in both children and adults. While exposure to pesticides is linked to the development of asthma, a cause and effect relationship has yet to be established.The majority of the evidence does not support a causal role between acetaminophen (paracetamol) or antibiotic use and asthma. A 2014 systematic review found that the association between acetaminophen use and asthma disappeared when respiratory infections were taken into account. Acetaminophen use by a mother during pregnancy is also associated with an increased risk of the child developing asthma. Maternal psychological stress during pregnancy is a risk factor for the child to develop asthma.Asthma is associated with exposure to indoor allergens. Common indoor allergens include dust mites, cockroaches, animal dander (fragments of fur or feathers), and mold. Efforts to decrease dust mites have been found to be ineffective on symptoms in sensitized subjects. Weak evidence suggests that efforts to decrease mold by repairing buildings may help improve asthma symptoms in adults. Certain viral respiratory infections, such as respiratory syncytial virus and rhinovirus, may increase the risk of developing asthma when acquired as young children. Certain other infections, however, may decrease the risk. Hygiene hypothesis
The hygiene hypothesis attempts to explain the increased rates of asthma worldwide as a direct and unintended result of reduced exposure, during childhood, to non-pathogenic bacteria and viruses. It has been proposed that the reduced exposure to bacteria and viruses is due, in part, to increased cleanliness and decreased family size in modern societies. | This is associated with poorer asthma control. Cognitive behavioral therapy may improve quality of life, asthma control, and anxiety levels in people with asthma.Improving peoples knowledge about asthma and using a written action plan has been identified as an important component of managing asthma. Providing educational sessions that include information specific to a persons culture is likely effective. More research is necessary to determine if increasing preparedness and knowledge of asthma among school staff and families using home-based and school interventions results in long term improvements in safety for children with asthma. School-based asthma self-management interventions, which attempt to improve knowledge of asthma, its triggers and the importance of regular practitioner review, may reduce hospital admissions and emergency department visits. These interventions may also reduce the number of days children experience asthma symptoms and may lead to small improvements in asthma-related quality of life. More research is necessary to determine if shared-decision-making is helpful for managing adults with asthma or if a personalized asthma action plan is effective and necessary. Some people with asthma use pulse oximeters to monitor their own blood oxygen levels during an asthma attack. However, there is no evidence regarding the use in these instances. Lifestyle modification
Avoidance of triggers is a key component of improving control and preventing attacks. The most common triggers include allergens, smoke (from tobacco or other sources), air pollution, non selective beta-blockers, and sulfite-containing foods. Cigarette smoking and second-hand smoke (passive smoke) may reduce the effectiveness of medications such as corticosteroids. | 11
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Cardiovascular disease (CVD) is a class of diseases that involve the heart or blood vessels. CVD includes coronary artery diseases (CAD) such as angina and myocardial infarction (commonly known as a heart attack). Other CVDs include stroke, heart failure, hypertensive heart disease, rheumatic heart disease, cardiomyopathy, abnormal heart rhythms, congenital heart disease, valvular heart disease, carditis, aortic aneurysms, peripheral artery disease, thromboembolic disease, and venous thrombosis.The underlying mechanisms vary depending on the disease. It is estimated that dietary risk factors are associated with 53% of CVD deaths. Coronary artery disease, stroke, and peripheral artery disease involve atherosclerosis. This may be caused by high blood pressure, smoking, diabetes mellitus, lack of exercise, obesity, high blood cholesterol, poor diet, excessive alcohol consumption, and poor sleep, among other things. High blood pressure is estimated to account for approximately 13% of CVD deaths, while tobacco accounts for 9%, diabetes 6%, lack of exercise 6%, and obesity 5%. Rheumatic heart disease may follow untreated strep throat.It is estimated that up to 90% of CVD may be preventable. Prevention of CVD involves improving risk factors through: healthy eating, exercise, avoidance of tobacco smoke and limiting alcohol intake. Treating risk factors, such as high blood pressure, blood lipids and diabetes is also beneficial. Treating people who have strep throat with antibiotics can decrease the risk of rheumatic heart disease. The use of aspirin in people, who are otherwise healthy, is of unclear benefit.Cardiovascular diseases are the leading cause of death worldwide except Africa. | Normally, the lungs are examined first, then the heart is examined if there are no apparent problems with the lungs. These examinations are typically performed using ultrasound, known as an echocardiogram when performed on the heart. Chest x-rays and electrocardiograms may also be used in reaching or confirming a diagnosis; however, an x-ray may appear normal immediately following birth. If d-TGA is accompanied by both a VSD and pulmonary stenosis, a systolic murmur will be present.On the rare occasion (when there is a large VSD with no significant left ventricular outflow tract obstruction), initial symptoms may go unnoticed, resulting in the infant being discharged without treatment in the event of a hospital or birthing center birth, or a delay in bringing the infant for diagnosis in the event of a home birth. On these occasions, a layperson is likely not to recognize symptoms until the infant is experiencing moderate to serious congestive heart failure (CHF) as a result of the heart working harder in a futile attempt to increase oxygen flow to the body; this overworking of the heart muscle eventually leads to hypertrophy and may result in cardiac arrest if left untreated. Description
In a normal heart, oxygen-depleted ("blue") blood is pumped from the right side of the heart, through the pulmonary artery, to the lungs where it is oxygenated. | 0-1
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Body-focused repetitive behavior (BFRB) is an umbrella name for impulse control behaviors involving compulsively damaging ones physical appearance or causing physical injury.Body-focused repetitive behavior disorders (BFRBDs) in ICD-11 is in development.BFRB disorders are currently estimated to be under the obsessive-compulsive spectrum. They are also associated with ADHD and anxiety. Causes
The cause of BFRBs is unknown.Emotional variables may have a differential impact on the expression of BFRBs.Research has suggested that the urge to repetitive self-injury is similar to a body-focused repetitive behavior but others have argued that for some the condition is more akin to a substance abuse disorder.Researchers are investigating a possible genetic component. Onset
BFRBs most often begin in late childhood or in the early teens. Diagnosis
Types
The main BFRB disorders are:
Skin
Dermatillomania (excoriation disorder), skin picking
Dermatophagia, skin nibbling
Mouth
Morsicatio buccarum, cheek biting
Morsicatio labiorum, inner lip biting
Morsicatio linguarum, tongue biting
Hands
Onychophagia, nail biting
Onychotillomania, nail picking
Nose
Rhinotillexomania, compulsive nose picking
Hair
Trichophagia, hair nibbling
Trichotemnomania, hair cutting
Trichotillomania, hair pulling
Eyes
Mucus fishing syndrome - compulsion to remove or "fish" strands of mucus from the eye
Treatment
Psychotherapy
Treatment can include behavior modification therapy, medication, and family therapy. The evidence base criteria for BFRBs is strict and methodical. Individual behavioral therapy has been shown as a "probably effective" evidence-based therapy to help with thumb sucking, and possibly nail biting. Cognitive behavioral therapy was cited as experimental evidence based therapy to treat trichotillomania and nail biting; a systematic review found best evidence for habit reversal training and decoupling. Another form of treatment that focuses on mindfulness, stimuli and rewards has proven effective in some people. | However, no treatment was deemed well-established to treat any form of BFRBs. Pharmacotherapy
Excoriation disorder, and trichotillomania have been treated with inositol and N-acetylcysteine. Prevalence
BFRBs are among the most poorly understood, misdiagnosed, and undertreated groups of disorders. BFRBs may affect at least 1 out of 20 people. These collections of symptoms have been known for a number of years, but only recently have appeared in widespread medical literature. Trichotillomania alone is believed to affect 10 million people in the United States. See also
Stereotypic movement disorder
Rhythmic Movement Disorder
Body dysmorphic disorder
Habit reversal training
Decoupling treatment
References
== External links == | 11
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Septal panniculitis is a condition of the subcutaneous fat affecting the layer of adipose tissue that lies between the dermis and underlying fascia, of which there are two forms: acute erythema nodosum and chronic erythema nodosum. : 487–9
See also
Erythema nodosum
List of cutaneous conditions
Panniculitis
Skin lesion
== References == | Idiopathic unilateral circumscribed hyperhidrosis
Reported association with:
Blue rubber bleb nevus
Glomus tumor
POEMS syndrome
Burning feet syndrome (Gopalans)
Trench foot
Causalgia
Pachydermoperiostosis
Pretibial myxedema
Gustatory sweating associated with:
Encephalitis
Syringomyelia
Diabetic neuropathies
Herpes zoster (shingles)
Parotitis
Parotid abscesses
Thoracic sympathectomy
Auriculotemporal or Freys syndrome
Miscellaneous
Lacrimal sweating (due to postganglionic sympathetic deficit, often seen in Raeders syndrome)
Harlequin syndrome
Emotional hyperhidrosis
Cancer
A variety of cancers have been associated with the development of secondary hyperhidrosis including lymphoma, pheochromocytoma, carcinoid tumors (resulting in carcinoid syndrome), and tumors within the thoracic cavity. Endocrine
Certain endocrine conditions are also known to cause secondary hyperhidrosis including diabetes mellitus (especially when blood sugars are low), acromegaly, hyperpituitarism, pheochromocytoma (tumor of the adrenal glands, present in 71% of patients) and various forms of thyroid disease. Medications
Use of selective serotonin reuptake inhibitors (e.g., sertraline) is a common cause of medication-induced secondary hyperhidrosis. Other medications associated with secondary hyperhidrosis include tricyclic antidepressants, stimulants, opioids, nonsteroidal anti-inflammatory drugs (NSAIDs), glyburide, insulin, anxiolytic agents, adrenergic agonists, and cholinergic agonists. Miscellaneous
In people with a history of spinal cord injuries
Autonomic dysreflexia
Orthostatic hypotension
Posttraumatic syringomyelia
Associated with peripheral neuropathies
Familial dysautonomia (Riley-Day syndrome)
Congenital autonomic dysfunction with universal pain loss
Exposure to cold, notably associated with cold-induced sweating syndrome
Associated with probable brain lesions
Episodic with hypothermia (Hines and Bannick syndrome)
Episodic without hypothermia
Olfactory
Associated with systemic medical problems
Parkinsons disease
Fibromyalgia
Congestive heart failure
Anxiety
Obesity
Menopausal state
Night sweats
Compensatory
Infantile acrodynia induced by chronic low-dose mercury exposure, leading to elevated catecholamine accumulation and resulting in a clinical picture resembling pheochromocytoma. Febrile diseases
Vigorous exercise
A hot, humid environment
Diagnosis
Symmetry of excessive sweating in hyperhidrosis is most consistent with primary hyperhidrosis. To diagnose this condition, a dermatologist gives the patient a physical exam. | 0-1
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Irritation can also be reduced by avoiding harsh facial cleansers and wearing sunscreen prior to sun exposure.One in 500 people experience hypersensitivity to BPO and are liable to experience burning, itching, crusting, and possibly swelling. About one-third of people experience phototoxicity under exposure to ultraviolet (UVB) light. Dosage
In the U.S., the typical concentration for benzoyl peroxide is 2.5% to 10% for both prescription and over-the-counter drug preparations that are used in treatment for acne. Other medical uses
Benzoyl peroxide is used in dentistry as a tooth whitening product. Non-medical uses
Bleaching
Like most peroxides, it is a powerful bleaching agent. It has been used for the bleaching of flour, fats, oils, waxes, and cheeses, as well as a stain remover. Polymerization
Benzoyl peroxide is also used as a radical initiator to induce chain-growth polymerization reactions, such as for polyester and poly(methyl methacrylate) (PMMA) resins and dental cements and restoratives. It is the most important among the various organic peroxides used for this purpose, a relatively safe alternative to the much more hazardous methyl ethyl ketone peroxide. It is also used in rubber curing and as a finishing agent for some acetate yarns. Safety
Explosion hazard
Concentrated benzoyl peroxide is potentially explosive like other organic peroxides, and can cause fires without external ignition. The hazard is acute for the pure material, so the compound is generally used as a solution or a paste. For example, cosmetics contain only a small percentage of benzoyl peroxide and pose no explosion risk. | Historically cyanide has been used for mass suicide and by the Nazis for genocide. Signs and symptoms
Acute exposure
If hydrogen cyanide is inhaled it can cause a coma with seizures, apnea, and cardiac arrest, with death following in a matter of seconds. At lower doses, loss of consciousness may be preceded by general weakness, dizziness, headaches, vertigo, confusion, and perceived difficulty in breathing. At the first stages of unconsciousness, breathing is often sufficient or even rapid, although the state of the person progresses towards a deep coma, sometimes accompanied by pulmonary edema, and finally cardiac arrest. A cherry red skin color that darkens may be present as the result of increased venous hemoglobin oxygen saturation. Despite the similar name, cyanide does not directly cause cyanosis. A fatal dose for humans can be as low as 1.5 mg/kg body weight. Other sources claim a lethal dose is 1–3 mg per kg body weight for vertebrates. Chronic exposure
Exposure to lower levels of cyanide over a long period (e.g., after use of improperly processed cassava roots, which are a primary food source in tropical Africa) results in increased blood cyanide levels, which can result in weakness and a variety of symptoms, including permanent paralysis, nervous lesions, hypothyroidism, and miscarriages. Other effects include mild liver and kidney damage. Causes
Cyanide poisoning can result from the ingestion of cyanide salts; imbibing pure liquid prussic acid; skin absorption of prussic acid; intravenous infusion of nitroprusside for hypertensive crisis; or the inhalation of hydrogen cyanide gas. | 0-1
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It is a combination of three medical conditions: hemolytic anemia, elevated liver enzymes and low platelet count. Eclampsia
This is when tonic-clonic seizures appear in a pregnant woman with high blood pressure and proteinuria.Pre-eclampsia and eclampsia are sometimes treated as components of a common syndrome. Treatment
There is no specific treatment, but is monitored closely to rapidly identify pre-eclampsia and its life-threatening complications (HELLP syndrome and eclampsia).Drug treatment options are limited, as many antihypertensives may negatively affect the fetus. Methyldopa, hydralazine, and labetalol are most commonly used for severe pregnancy hypertension.The fetus is at increased risk for a variety of life-threatening conditions, including pulmonary hypoplasia (immature lungs). If the dangerous complications appear after the fetus has reached a point of viability, even though still immature, then an early delivery may be warranted to save the lives of both mother and baby. An appropriate plan for labor and delivery includes selection of a hospital with provisions for advanced life support of newborn babies. Evolutionary considerations
Humans
Gestational hypertension is one of the most common disorders seen in human pregnancies. Though relatively benign on its own, in roughly half of the cases of gestational hypertension the disorder progresses into pre-eclampsia, a dangerous condition that can prove fatal to expectant mothers. However, gestational hypertension is a condition that is fairly rare to see in other animals. | Stroke
A combination of dipyridamole and aspirin (acetylsalicylic acid/dipyridamole) is FDA-approved for the secondary prevention of stroke and has a bleeding risk equal to that of aspirin use alone. Dipyridamole absorption is pH-dependent and concomitant treatment with gastric acid suppressors (such as a proton pump inhibitor) will inhibit the absorption of liquid and plain tablets. Modified release preparations are buffered and absorption is not affected.However, it is not licensed as monotherapy for stroke prophylaxis, although a Cochrane review suggested that dipyridamole may reduce the risk of further vascular events in patients presenting after cerebral ischemia.A triple therapy of aspirin, clopidogrel, and dipyridamole has been investigated, but this combination led to an increase in adverse bleeding events. Vasodilation occurs in healthy arteries, whereas stenosed arteries remain narrowed. This creates a "steal" phenomenon where the coronary blood supply will increase to the dilated healthy vessels compared to the stenosed arteries which can then be detected by clinical symptoms of chest pain, electrocardiogram and echocardiography when it causes ischemia. Flow heterogeneity (a necessary precursor to ischemia) can be detected with gamma cameras and SPECT using nuclear imaging agents such as Thallium-201, Tc99m-Tetrofosmin and Tc99m-Sestamibi. However, relative differences in perfusion do not necessarily imply any absolute decrease in blood supply in the tissue supplied by a stenosed artery. Other uses
Dipyridamole also has non-medicinal uses in a laboratory context, such as the inhibition of cardiovirus growth in cell culture. Drug interactions
Due to its action as a phosphodiesterase inhibitor, dipyridamole is likely to potentiate the effects of adenosine. | 0-1
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While Goldestein and Gelb believed the patient had damaged the lateral and medial parts of the left occipital lobe, it was later indicated that both occipital lobes were probably affected, due to the bilateral, concentric loss of his visual field. He lost his visual field beyond a 30-degree eccentricity and could not identify visual objects by their proper names. "LM"
Most of what is known about akinetopsia was learned from LM, a 43-year-old female admitted into the hospital October 1978 complaining of headache and vertigo. LM was diagnosed with thrombosis of the superior sagittal sinus which resulted in bilateral, symmetrical lesions posterior of the visual cortex. These lesions were verified by PET and MRI in 1994. LM had minimal motion perception that was preserved as perhaps a function of V1, as a function of a "higher" order visual cortical area, or some functional sparing of V5.LM found no effective treatment, so she learned to avoid conditions with multiple visual motion stimuli, i.e. by not looking at or fixating them. She developed very efficient coping strategies to do this and nevertheless lived her life. In addition, she estimated the distance of moving vehicles by means of sound detection in order to continue to cross the street.LM was tested in three areas against a 24-year-old female subject with normal vision:
Visual functions other than movement vision
LM had no evidence of a color discrimination deficit in either center or periphery of visual fields. Her recognition time for visual objects and words was slightly higher than the control, but not statistically significant. | Erythrocyanosis crurum is a skin condition, a variant of acrocyanosis caused by chronic exposure to cold. See also
Chilblains
List of cutaneous conditions
References
Otto Braun-Falco; G. Plewig; H. H. Wolff; Walter H. C. Burgdorf (2000). Dermatology. Springer Verlag. p. 889. ISBN 3-540-59452-3. James, William D.; Berger, Timothy G.; et al. (2006). Andrews Diseases of the Skin: Clinical Dermatology. Saunders Elsevier. ISBN 0-7216-2921-0. == External links == | 0-1
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In particular, five is the first congruent number, since it is the length of the hypotenuse of the smallest integer-sided right triangle.Five is the second Fermat prime of the form
2
2
n
{\displaystyle 2^{2^{n}}}
+
1
{\displaystyle 1}
, and more generally the second Sierpiński number of the first kind,
n
n
{\displaystyle n^{n}}
+
1
{\displaystyle 1}
. | There are exclusively twelve complex aperiotopes in
C
n
{\displaystyle \mathbb {C} ^{n}}
complex spaces of dimensions
n
{\displaystyle n}
⩾
5
{\displaystyle 5}
, with fifteen in
C
4
{\displaystyle \mathbb {C} ^{4}}
and sixteen in
C
3
{\displaystyle \mathbb {C} ^{3}}
; alongside complex polytopes in
C
5
{\displaystyle \mathbb {C} ^{5}}
and higher under simplex, hypercubic and orthoplex groups, the latter with van Oss polytopes. | 11
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Gynecologic hemorrhage represents excessive bleeding of the female reproductive system. Such bleeding could be visible or external, namely bleeding from the vagina, or it could be internal into the pelvic cavity or form a hematoma. Normal menstruation is not considered a gynecologic hemorrhage, as it is not excessive. Hemorrhage associated with a pregnant state or during delivery is an obstetrical hemorrhage. Types
Metrorrhagia (metro = womb, -rrhagia = excessive flow) is uterine bleeding at irregular intervals, particularly between the expected menstrual periods. Postcoital bleeding is vaginal bleeding after sexual intercourse. Causes
Causes of gynecologic bleeding include:
Hormonal
Anovulation is a common cause of gynecological hemorrhage. Under the influence of estrogen the endometrium (uterine lining) is stimulated and eventually such lining will be shed off (estrogen breakthrough bleeding). The anovulation chapter discusses its multiple possible causes. Longstanding anovulation can also lead to endometrial hyperplasia and facilitate the development of endometrial cancer. Neoplasm
Cancer of the uterus is always a concern, specifically when the bleeding occurs after menopause. Other types of cancer include cervical cancer; bleeding in that case can sometimes be triggered by postcoital bleeding. Cancers of the vagina or fallopian tubes are rare causes of hemorrhage. Uterine fibroids represent a common, benign condition that may lead to bleeding, specifically if the lesion affects the uterine cavity. Polyps of the uterine lining are a common cause of bleeding, but such bleeding tends to be light. Trauma
Sexual assault and rape can lead to injury and gynecological hemorrhage. Accidents to the lower abdomen may lead to internal or external bleeding. | Some trauma specialists suggest increasing inquiry into information about childrens trauma history and exposure to violence, since the majority of people (about 80%) responsible for child maltreatment are the childs own parents. Trauma-specific intervention for children may be useful in preventing future symptoms. Treatment
Treatment of DPDR is often difficult and refractory. Some clinicians speculate that this could be due to a delay in diagnosis by which point symptoms tend to be constant and less responsive to treatment. Additionally, symptoms tend to overlap with other diagnoses. Some results have been promising, but are hard to evaluate with confidence due to the small size of trials. However, recognizing and diagnosing the condition may in itself have therapeutic benefits, considering many patients express their problems as baffling and unique to them, but are in fact: one, recognized and described by psychiatry; and two, those affected by it are not the only individuals to be affected from the condition. However, symptoms are often transient and can remit on their own without treatment.Treatment is primarily non-pharmacological and can include paradoxical intention, record keeping, positive reward, flooding, psychotherapy, cognitive-behavioral therapy, psychoeducation, self-hypnosis, and meditation. Meditation with the focus on the body has been used to achieve self awareness as it allows feelings, which otherwise are put aside or neutralized by the DPD condition. Self-hypnosis training can be helpful and entails training patients to induce dissociative symptoms and respond in an alternative manner. | 0-1
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The variation in joint configuration results in some sacroiliac joints being inherently weaker or more prone to misalignment.Certain biomechanical or muscle length imbalances may ultimately predispose a person to sacroiliac dysfunction and pain. Likely, this is a result of altered gait patterns and repetitive stress to the SI joint and related structures. These conditions exist in persons with leg-length inequality, scoliosis, a history of polio, poor-quality footwear, and hip osteoarthritis.There is also a notable incidence of lumbar spinal fusion patients that present with sacroiliac pain and hypermobility, potentially due to the adjacent lumbar joints being fixed and unable to move. Clinical studies have found up to 75% of post-lumbar fusion patients develop SI joint degeneration within five years of surgery. Hypomobility
Pathological hypomobility (too little movement) of the sacroiliac joint is an intra-articular disorder in which the joint locks due to wearing down with age or degenerative joint disease.Hypomobility of this kind can also occur with an inflammatory disease such as ankylosing spondylitis, rheumatoid arthritis, or an infection. Pathophysiology
The sacroiliac joint is a true diarthrodial joint that joins the sacrum to the pelvis. The sacrum connects on the right and left sides to the ilia (pelvic bones) to form the sacroiliac joints. The pelvic girdle is made up of two innominate bones (the iliac bones) and the sacrum. The innominate bones join in the front of the pelvis to form the pubic symphysis, and at back of the sacrum to form the sacroiliac (SI) joints. | Nevus spilus, also known as speckled lentiginous nevus, is a light brown or tan birth mark, speckled with small, dark spots or small bumps. If it occurs in a segmental pattern then it is sometimes referred to as a Zosteriform speckled lentiginous nevus.It may be associated with types of phakomatosis pigmentovascularis.Prevalence is between 0.2% and 2.8%. See also
Phakomatosis pigmentokeratotica
Skin lesion
List of cutaneous conditions
References
== External links == | 0-1
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This clot tends to stick the parietal and visceral pleura together and has the potential to lead to scarring within the pleura, which if extensive leads to the condition known as a fibrothorax. Following the initial loss of blood, a small hemothorax may irritate the pleura, causing additional fluid to seep out, leading to a bloodstained pleural effusion. Furthermore, as enzymes in the pleural fluid begin to break down the clot, the protein concentration of the pleural fluid increases. As a result, the osmotic pressure of the pleural cavity increases, causing fluid to leak into the pleural cavity from the surrounding tissues. Diagnosis
Hemothoraces are most commonly detected using a chest X-ray, although ultrasound is sometimes used in an emergency setting. It can be suspected in any person with any form of chest trauma. However, plain X-rays may miss smaller hemothoraces while other imaging modalities such as computed tomography (CT), or magnetic resonance imaging may be more sensitive. In cases where the nature of an effusion is in doubt, a sample of fluid can be aspirated and analysed in a procedure called thoracentesis. Physical examination is used initially. Auscultation has been reported to have an accuracy of nearly 100% in diagnosing hemopneumothorax. Chest X-ray
A chest X-ray is the most common technique used to diagnosis a hemothorax. X-rays should ideally be taken in an upright position (an erect chest X-ray), but may be performed with the person lying on their back (supine) if an erect chest X-ray is not feasible. | Tebentafusp, sold under the brand name Kimmtrak, is an anti-cancer medication used to treat uveal melanoma (eye cancer).The most common side effects include cytokine release syndrome, rash, pyrexia (fever), pruritus (itching), fatigue, nausea, chills, abdominal pain, edema, hypotension, dry skin, headache, and vomiting.Tebentafusp is a bispecific gp100 peptide-HLA-directed CD3 T cell engager. It was approved for medical use in the United States in January 2022. Medical uses
Tebentafusp is indicated for HLA-A*02:01-positive adults with unresectable or metastatic uveal melanoma. History
Efficacy was evaluated in IMCgp100-202 (NCT03070392), a randomized, open-label, multicenter trial of 378 participants with metastatic uveal melanoma. Participants were required to be HLA-A*02:01 genotype positive identified by a central assay. Participants were excluded if prior systemic therapy or localized liver-directed therapy were administered. Prior surgical resection of oligometastatic disease was permitted. Participants with clinically significant cardiac disease or symptomatic, untreated brain metastases were excluded.The U.S. Food and Drug Administration (FDA) granted Immunocores application for tebentafusp priority review, breakthrough therapy, and orphan drug designations. Society and culture
Legal status
On 24 February 2022, the Committee for Medicinal Products for Human Use (CHMP) of the European Medicines Agency (EMA) adopted a positive opinion, recommending the granting of a marketing authorization for the medicinal product Kimmtrak, intended for the treatment of uveal melanoma. The applicant for this medicinal product is Immunocore Ireland Limited. Tebentafusp was approved for medical use in the European Union in April 2022. References
External links
"Tebentafusp". Drug Information Portal. U.S. National Library of Medicine. | 0-1
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Opsoclonus myoclonus syndrome (OMS), also known as opsoclonus-myoclonus-ataxia (OMA), is a rare neurological disorder of unknown cause which appears to be the result of an autoimmune process involving the nervous system. It is an extremely rare condition, affecting as few as 1 in 10,000,000 people per year. It affects 2 to 3% of children with neuroblastoma and has been reported to occur with celiac disease and diseases of neurologic and autonomic dysfunction. Signs and symptoms
Symptoms include:
opsoclonus (rapid, involuntary, multivectorial (horizontal and vertical), unpredictable, conjugate fast eye movements without intersaccadic [quick rotation of the eyes] intervals)
myoclonus (brief, involuntary twitching of a muscle or a group of muscles)
cerebellar ataxia, both truncal and appendicular
aphasia (a language disorder in which there is an impairment of speech and of comprehension of speech, caused by brain damage)
mutism (a language disorder in which a person does not speak despite evidence of speech ability in the past, often part of a larger neurological or psychiatric disorder)
lethargy
irritability or malaise
drooling
strabismus (a condition in which the eyes are not properly aligned with each other)
vomiting
sleep disturbances
emotional disturbances (including fits of rage)About half of all OMS cases occur in association with neuroblastoma (a cancer of the sympathetic nervous system usually occurring in infants and children). Disease course and clinical subtypes
In most cases, OMS starts with an acute flare-up of physical symptoms within days or weeks, but some less obvious symptoms such as irritability and malaise may begin weeks or months earlier. | Since neurologic and developmental difficulties have not been reported as a consequence of neuroblastoma or its treatment, it is thought that these are exclusively due to the immune mechanism underlying OMS.One study concludes that: "Patients with OMA and neuroblastoma have excellent survival but a high risk of neurologic sequelae. Favourable disease stage correlates with a higher risk for development of neurologic sequelae. The role of anti-neuronal antibodies in late sequelae of OMA needs further clarification".Another study states that: "Residual behavioral, language, and cognitive problems occurred in the majority". Research
The National Institute of Neurological Disorders and Stroke (NINDS) conducts and supports research on various movement disorders, including opsoclonus myoclonus. These studies are focused on finding ways to prevent, treat, and cure these disorders, as well as increasing knowledge about them. Nomenclature
OMS was first described by Marcel Kinsbourne in 1962. (The term Opsoclonus was coined by Orzechowski in 1913, but it was classically described and associated with neuroblastoma by Kinsbourne). Other names for OMS include:
Dancing Eyes-Dancing Feet syndrome
Dancing Eyes syndrome (see also Nystagmus)
Kinsbourne syndrome
Myoclonic Encephalopathy of Infants (MEI), not to be confused with Early myoclonic encephalopathy (EME)
Opsoclonic Cerebellopathy
Opsoclonus-Myoclonus-Ataxia (OMA)
Paraneoplastic Opsoclonus-Myoclonus Ataxia (POMA)
References
Further reading
== External links == | 11
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Although lung biopsy is usually not necessary, the presence of asbestos bodies in association with pulmonary fibrosis establishes the diagnosis. Conversely, interstitial pulmonary fibrosis in the absence of asbestos bodies is most likely not asbestosis. Asbestos bodies in the absence of fibrosis indicate exposure, not disease. Treatment
There is no cure available for asbestosis. Oxygen therapy at home is often necessary to relieve the shortness of breath and correct underlying low blood oxygen levels. Supportive treatment of symptoms includes respiratory physiotherapy to remove secretions from the lungs by postural drainage, chest percussion, and vibration. Nebulized medications may be prescribed in order to loosen secretions or treat underlying chronic obstructive pulmonary disease. Immunization against pneumococcal pneumonia and annual influenza vaccination is administered due to increased sensitivity to the diseases. Those with asbestosis are at increased risk for certain cancers. If the person smokes, quitting the habit reduces further damage. Periodic pulmonary function tests, chest x-rays, and clinical evaluations, including cancer screening/evaluations, are given to detect additional hazards. Society and culture
Legal issues
The death of English textile worker Nellie Kershaw in 1924 from pulmonary asbestosis was the first case to be described in medical literature, and the first published account of disease attributed to occupational asbestos exposure. However, her former employers (Turner Brothers Asbestos) denied that asbestosis even existed because the medical condition was not officially recognised at the time. As a result, they accepted no liability for her injuries and paid no compensation, either to Kershaw during her final illness or to her family after her death. | The organization of the modules necessary to biosynthesize Vancomycin is shown in Figure 1. In the biosynthesis of Vancomycin, additional modification domains are present, such as the epimerization (E) domain, which isomerizes the amino acid from one stereochemistry to another, and a thioesterase domain (TE) is used as a catalyst for cyclization and releases of the molecule via a thioesterase scission. A set of NRPS enzymes (peptide synthase VpsA, VpsB, and VpsC) are responsible for assembling the heptapeptide. (Figure 2). VpsA codes for modules 1, 2, and 3. VpsB codes for modules 4, 5, and 6, and VpsC codes for module 7. The vancomycin aglycone contains 4 D-amino acids, although the NRPSs only contain 3 epimerization domains. The origin of D-Leu at residue 1 is not known. The three peptide syntheses are located at the start of the region of the bacterial genome linked with antibiotic biosynthesis, and span 27 kb.β-hydroxytyrosine (β-HT) is synthesized prior to incorporation into the heptapeptide backbone. L-tyrosine is activated and loaded on the NRPS VpsD, hydroxylated by OxyD, and released by the thioesterase Vhp. The timing of the chlorination by halogenase VhaA during biosynthesis is currently undetermined, but is proposed to occur before the complete assembly of the heptapeptide.After the linear heptapeptide molecule is synthesized, vancomycin has to undergo further modifications, such as oxidative cross-linking and glycosylation, in trans by distinct enzymes, referred to as tailoring enzymes, to become biologically active (Figure 3). To convert the linear heptapeptide to cross-linked, glycosylated vancomycin, six enzymes, are required. | 0-1
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Thiazolidinediones have been strongly linked to new cases of heart failure and worsening of pre-existing congestive heart failure due to their association with weight gain and fluid retention. Certain calcium channel blockers, such as diltiazem and verapamil, are known to decrease the force with which the heart ejects blood, thus are not recommended in people with heart failure with a reduced ejection fraction. Supplements
Certain alternative medicines carry a risk of exacerbating existing heart failure, and are not recommended. This includes aconite, ginseng, gossypol, gynura, licorice, lily of the valley, tetrandrine, and yohimbine. Aconite can cause abnormally slow heart rates and abnormal heart rhythms such as ventricular tachycardia. Ginseng can cause abnormally low or high blood pressure, and may interfere with the effects of diuretic medications. Gossypol can increase the effects of diuretics, leading to toxicity. Gynura can cause low blood pressure. Licorice can worsen heart failure by increasing blood pressure and promoting fluid retention. Lily of the valley can cause abnormally slow heart rates with mechanisms similar to those of digoxin. Tetrandrine can lead to low blood pressure through inhibition of L-type calcium channels. Yohimbine can exacerbate heart failure by increasing blood pressure through alpha-2 adrenergic receptor antagonism. Pathophysiology
Heart failure is caused by any condition that reduces the efficiency of the heart muscle, through damage or overloading. | This high prevalence in these ethnic minority populations has been linked to high incidence of diabetes and hypertension. In many new immigrants to the U.S., the high prevalence of heart failure has largely been attributed to lack of preventive health care or substandard treatment. Nearly one of every four people (24.7%) hospitalized in the U.S. with congestive heart failure are readmitted within 30 days. Additionally, more than 50% of people seek readmission within 6 months after treatment and the average duration of hospital stay is 6 days. Heart failure is a leading cause of hospital readmissions in the U.S. People aged 65 and older were readmitted at a rate of 24.5 per 100 admissions in 2011. In the same year, people under Medicaid were readmitted at a rate of 30.4 per 100 admissions, and uninsured people were readmitted at a rate of 16.8 per 100 admissions. These are the highest readmission rates for both categories. Notably, heart failure was not among the top-10 conditions with the most 30-day readmissions among the privately insured. United Kingdom
In the UK, despite moderate improvements in prevention, heart failure rates have increased due to population growth and ageing. Overall heart failure rates are similar to the four most common causes of cancer (breast, lung, prostate, and colon) combined. People from deprived backgrounds are more likely to be diagnosed with heart failure and at a younger age. Developing world
In tropical countries, the most common cause of HF is valvular heart disease or some type of cardiomyopathy. | 11
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Tonsillitis is inflammation of the tonsils in the upper part of the throat. Tonsillitis is a type of pharyngitis that typically comes on fast (rapid onset). Symptoms may include sore throat, fever, enlargement of the tonsils, trouble swallowing, and large lymph nodes around the neck. Complications include peritonsillar abscess.Tonsillitis is most commonly caused by a viral infection and about 5% to 40% of cases are caused by a bacterial infection. When caused by the bacterium group A streptococcus, it is referred to as strep throat. Rarely bacteria such as Neisseria gonorrhoeae, Corynebacterium diphtheriae, or Haemophilus influenzae may be the cause. Typically the infection is spread between people through the air. A scoring system, such as the Centor score, may help separate possible causes. Confirmation may be by a throat swab or rapid strep test.Treatment efforts involve improving symptoms and decreasing complications. Paracetamol (acetaminophen) and ibuprofen may be used to help with pain. If strep throat is present the antibiotic penicillin by mouth is generally recommended. In those who are allergic to penicillin, cephalosporins or macrolides may be used. In children with frequent episodes of tonsillitis, tonsillectomy modestly decreases the risk of future episodes.About 7.5% of people have a sore throat in any three-month period and 2% of people visit a doctor for tonsillitis each year. It is most common in school-aged children and typically occurs in the colder months of autumn and winter. The majority of people recover with or without medication. | Clinical pharmacology
As is evident with the parent molecule, atracurium, cisatracurium is also susceptible to degradation by Hofmann elimination and ester hydrolysis as components of the in vivo metabolic processes. See the atracurium page for information on Hofmann elimination in vivo versus the Hofmann degradation chemical reaction. Because Hofmann elimination is a temperature- and plasma pH-dependent process, cisatracuriums rate of degradation in vivo is highly influenced by body pH and temperature just as it is with the parent molecule, atracurium: thus, an increase in body pH favors the elimination process, whereas a decrease in temperature slows down the process. One of the metabolites of cisatracurium via Hofmann elimination is laudanosine – see the atracurium page for further discussion of the issue regarding this metabolite. 80% of cisatracurium is metabolized eventually to laudanosine and 20% is metabolized hepatically or excreted renally. 10-15% of the dose is excreted unchanged in the urine.Since Hofmann elimination is an organ-independent chemodegradative mechanism, there is little or no risk to the use of cisatracurium in patients with liver or renal disease when compared with other neuromuscular-blocking agents.The two reverse ester linkages in the bridge between the two isoquinolinium groups make atracurium and cisatracurium poor targets for plasma cholinesterase, unlike mivacurium which has two conventional ester linkages. Adverse effects
Histamine release – hypotension, reflex tachycardia and cutaneous flush
Bronchospasm – Pulmonary compliance
To date, cisatracurium has not been reported to elicit bronchospasm at doses that are clinically prescribed. | 0-1
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Chimera – sometimes depicted with the heads of a goat and a lion. The Lernaean Hydra – an ancient nameless serpent-like chthonic water beast that possessed numerous heads. Orthrus – a two-headed dog owned by Geryon. Scylla – sometimes described as a six-headed sea monster.Other multi-headed creatures in Greek mythology include:
The Hecatonchires – giants with fifty heads and one hundred arms. The word "Hecatonchire" means "hundred arms". They were the sons of Gaia, and Uranus. Hecate – Greek goddess of witches, nightmares, crossroads, and one of the Moon deities; sometimes represented with three heads. Iranian mythology
Zahhak, an evil figure in Iranian mythology - also evident in ancient Iranian folklore as Aži Dahāka (Azh dahak) - is the most significant and long-lasting of the ažis of the Avesta, the earliest religious texts of Zoroastrianism. He is described as a monster with three mouths, six eyes, and three heads (presumably meaning three heads with one mouth and two eyes each), cunning, strong and demonic. But in other respects Aži Dahāka has human qualities, and is never a mere animal. Hinduism
Hindu deities are often depicted with multiple arms or heads. The fire-god Agni has two heads
Dattatreya: three
The creator-god Brahma: four
The goddess Gayatri: five
The war-god Kartikeya: sixThough usually depicted with one head, some deities like Ganesha (in Heramba form) and Shiva (Sadashiva) have aspects where they are depicted with multiple heads; five in this case. The Vishvarupa form of Vishnu is described as having infinite heads. Besides deities, demons (asura and rakshasa) may be depicted with multiple heads. | Focal dystonia, or focal task specific dystonia, is a neurological condition, a type of dystonia, that affects a muscle or group of muscles in a specific part of the body during specific activities, causing involuntary muscular contractions and abnormal postures. There are many different types of focal dystonia, each effecting a different region of the body. For example, in focal hand dystonia, or writers cramp, the fingers either curl into the palm or extend outward without control. In musicians, the condition is called musicians focal dystonia, or simply, musicians dystonia. In sports, it may be involved in what is commonly referred to as the yips. The condition appears to be associated with over-training, and individualized treatment strategies may involve medications, retraining techniques, and procedures. Signs and Symptoms
People with dystonia experience tightness, cramping, fatigue, involuntary sustained or repetitive muscle contractions that can be painless or painful and resulting in abnormal posturing, twisting motions, and even tremors. Focal dystonia typically presents in adults, more commonly in women ranging from ages 30 through 40. Major types of focal dystonia affect the limbs, face, mouth, neck, and larynx. These focal dystonias can be exclusive to specific tasks, like writing or playing an instrument. Focal dystonia will typically have a subtle and slow onset before slowly worsening over years. During the beginning stages, symptoms can be intermittent and without clear associations and provocation. The progression of this disease can vary from person to person. | 0-1
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The Sar1B gene is located at position 5q31.1 in the fifth chromosome and is composed of eight exons. Alternative splicing of the second exon results into two different splice isoforms for the Sar1B transcript RNA. In CMRD, a mutation of this genomic sequence affects the Sar1B enzymes ability to interact with Guanine Exchange Factors (GEFs) and GTP-Activating Proteins (GAPs). The mutation of exon 6 of the sequence can eliminate the critical chain that is responsible for recognizing guanine. This strips the GTPase of its capability to hydrolyze GTP, its hallmark trait. This overall affects the ability of Sar1B GTPase to control chylomicron release. A third mutant allele containing a missense mutation has also been reported to cause CMRD. All three of these alleles display recessive inheritance, suggesting that they loss-of-function mutations cause the symptoms of CMRD. Physiology
During digestion, fats, or triglycerides(TGs), are enzymatically catabolized by lipases into two fatty acids and a monoglyceride molecule. Those components are then transported across the enterocyte membrane as micelles and reformed into triglycerides once across the membrane.Once transported to the ER the triglycerides are incorporated into pre-chylomicrons which are made up of TGs, cholesterol, and phospholipids. The pre-chylomicrons are then packaged into PCTV in order to be transported to the Golgi apparatus for additional maturation prior to exocytosis into the lymphatic system. From the lymphatic system, they enter general circulation, where they are produced in various forms that can be absorbed by bodily tissues and metabolized or stored by adipose tissue. | Chylomicron retention disease is a disorder of fat absorption. It is associated with SAR1B. Mutations in SAR1B prevent the release of chylomicrons in the circulation which leads to nutritional and developmental problems. It is a rare autosomal recessive disorder with around 40 cases reported worldwide. Since the disease allele is recessive, parents usually do not show symptoms.Without functional chylomicrons, certain fat-soluble vitamins such as vitamin D and vitamin E cannot be absorbed. Chylomicrons have a crucial role in fat absorption and transport, thus a deficiency in chylomicron functioning reduces available levels of dietary fats and fat-soluble vitamins. History
Chylomicron Retention Disease, also called Andersons disease, is an autosomal recessive lipid malabsorption syndrome characterized by abnormally low amounts of cholesterol in the blood. This disease most frequently is diagnosed in infants. Charlotte Anderson first published a description of the disorder in 1961, where she observed a seven month old girl who developed intestinal mucosa filled with fat droplets. In 2003, Jones and colleagues identified mutations in the SAR1B gene, which transcripts the SAR1B protein involved in COPII transport and proposed this was the molecular defect of the disorder. To present day, 17 mutations of the SAR1B gene have been discovered. This disease is rare, with only 50 cases diagnosed worldwide. Genetics
The Sar1B GTPase is an enzyme located in epithelial cells of the gastrointestinal tract. These proteins are critical for release of chylomicrons in the body.Chylomicron retention disease is an autosomal homozygous recessive disorder arising from mutations in the gene encoding the Sar1B GTPase. | 11
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