text1
stringlengths 11
17.4k
| text2
stringlengths 11
8.2k
| label
class label 2
classes |
|---|---|---|
Paromomycin is an antimicrobial used to treat a number of parasitic infections including amebiasis, giardiasis, leishmaniasis, and tapeworm infection. It is a first-line treatment for amebiasis or giardiasis during pregnancy. Otherwise it is generally a second line treatment option. It is taken by mouth, applied to the skin, or by injection into a muscle.Common side effects when taken by mouth include loss of appetite, vomiting, abdominal pain, and diarrhea. When applied to the skin side effects include itchiness, redness, and blisters. When given by injection there may be fever, liver problems, or hearing loss. Use during breastfeeding appears to be safe. Paromomycin is in the aminoglycoside family of medications and causes microbe death by stopping the creation of bacterial proteins.Paromomycin was discovered in the 1950s from a type of streptomyces and came into medical use in 1960. It is on the World Health Organizations List of Essential Medicines. Paromomycin is available as a generic medication. Medical uses
It is an antimicrobial used to treat intestinal parasitic infections such as cryptosporidiosis and amoebiasis, and other diseases such as leishmaniasis. Paromomycin was demonstrated to be effective against cutaneous leishmaniasis in clinical studies in the USSR in the 1960s, and in trials with visceral leishmaniasis in the early 1990s.The route of administration is intramuscular injection and capsule. Paromomycin topical cream with or without gentamicin is an effective treatment for ulcerative cutaneous leishmaniasis, according to the results of a phase-3, randomized, double-blind, parallel group–controlled trial. Pregnancy and breastfeeding
The medication is poorly absorbed. | Band keratopathy is a corneal disease derived from the appearance of calcium on the central cornea. This is an example of metastatic calcification, which by definition, occurs in the presence of hypercalcemia. Signs and symptoms
Signs and symptoms of band keratopathy include eye pain and decreased visual acuity. Causes
Band keratopathy has several causes. These causes include uveitis, interstitial keratitis, superficial keratitis, phthisis, sarcoidosis, trauma, intraocular silicone oil, systemic diseases (high levels of calcium in the blood, vitamin D intoxication, Fanconis Syndrome, low levels of phosphorus in the blood, gout, milk-alkali syndrome, myotonic dystrophy, and chronic mercury exposure). Pathology
Band keratopathy is seen when there is calcification of the epithelial basement membrane, Bowmans membrane, and the anterior stroma with destruction of Bowmans membrane. The calcium salts are intracellular when the process is due to alteration of systemic calcium metabolism, whereas they are extracellular when due to local disease. Diagnosis
Band keratopathy is diagnosed by slit lamp examination of the eye. Treatment
Treatment is indicated if vision is threatened or if the eye is uncomfortable. It is important to recognize and treat any underlying condition. Improvement of vision may be obtained by scraping off the opacity. Topical disodium EDTA can be used as a chelating agent. It can also be removed with the excimer laser (phototherapeutic keratectomy). Other modalities include diamond burr and lamellar keratoplasty. References
== External links == | 0-1
|
In advanced airway management, rapid sequence induction (RSI) – also referred to as rapid sequence intubation or as rapid sequence induction and intubation (RSII) or as crash induction – is a special process for endotracheal intubation that is used where the patient is at a high risk of pulmonary aspiration. It differs from other techniques for inducing general anesthesia in that several extra precautions are taken to minimize the time between giving the induction drugs and securing the tube, during which period the patients airway is essentially unprotected.First described by William Stept and Peter Safar in 1970, "classical" or "traditional" RSI involves pre-filling the patients lungs with a high concentration of oxygen gas; applying cricoid pressure to occlude the esophagus; administering pre-determined doses of rapid-onset sedative and neuromuscular-blocking drugs (traditionally thiopentone and suxamethonium) that induce prompt unconsciousness and paralysis; avoiding any artificial positive-pressure ventilation by mask after the patient stops breathing (to minimize insufflation of air into the stomach, which might otherwise provoke regurgitation); inserting a cuffed endotracheal tube with minimal delay; and then releasing the cricoid pressure after the cuff is inflated, with ventilation being started through the tube. There is no consensus around the precise definition of the term "modified RSI", but it is used to refer to various modifications that deviate from the classic sequence – usually to improve the patients physiological stability during the procedure, at the expense of theoretically increasing the risk of regurgitation. | Drug Information Portal. U.S. National Library of Medicine. "Umbralisib". NCI Drug Dictionary. National Cancer Institute. | 0-1
|
Over a five- to ten-year period, this vasculopathy (blood vessel pathology) results in vision loss and destructive brain lesions with neurologic deficits and death. Although brain and eye disease are universally present in patients with RVCL, the disease is truly a multi-system disorder characterized by chronic kidney disease, liver disease, and frequently other manifestations including gastrointestinal disease, osteonecrosis, and hypothyroidism. Diagnosis
Differential diagnosis
Treatment
Currently, there is no therapy that is proven to prevent the blood vessel deterioration. In 2021, Dr. Jonathan Miner of the University of Pennsylvania (Penn) in Philadelphia, and the Hospital of the University of Pennsylvania (Penn Medicine), initiated a clinical trial of crizanlizumab for RVCL. The University of Pennsylvania also sponsors RVCL patient support groups, and collaborates with the RVCL Research Center at Washington University (WashU) on clinical trials and longitudinal studies. Miner also established a large RVCL Research Center, which is conducting clinical trials, performing basic research, and developing novel personalized therapies for RVCL. This includes small molecular inhibitors (to be taken as pills) and gene therapy approaches, with the goal of fully correcting the disease-causing mutation. Developing these personalized therapies is a challenging process that will take years. History
1985–1988: CRV (Cerebral Retinal Vasculopathy) was discovered by multiple investigators including Dr. Gil Grand, Dr. John P. Atkinson, and colleagues at Washington University School of Medicine (WashU). | Brand names
Aeronide (TH); Aquacort (DE); B Cort (CO); Bronex (PH); Budair (MY); Budecort DP (MY); Budenofalk (DE, GB, HK, KP, PH, SG); Budeson (AR); Budeson Aqua (AR); BudeSpray (TH); Budiair (KP); Budicort Respules (IL); Budinide (KSA); Bunase (TH); Clebudan (CN); Cortiment (CA, GB, AU); Cycortide (HK); Denecort (PH); Duasma (TW); Eltair (MY); Entocort (AR, AT, BE, BR, CH, CZ, DK, FI, FR, GB, HK, IE, IL, IT, KP, NL, NO, PL, PT, SE, TR);: 13 Giona Easyhaler (MY, SG, TH); Inflammide (PE); Miflonid (CZ); Miflonide (BE, DE, IL, IT, NZ, PT); Neumocort (PY); Novopulmon (DE, FR); Pulmicon Susp for Nebulizer (KP); Pulmicort (AT, BE, BG, BR, CH, CL, CN, CO, CR, CZ, DE, DK, DO, EE, FI, FR, GB, GR, GT, HN, ID, IN, NI, NL, NO, PA, PK, PL, PT, RU, SE, SV, TR, TW, UY, VE, ZA);: 13 Pulmicort Nasal Turbohaler (CL, KE, MU, NG); Pulmicort Turbuhaler (KE, MU, NG); Rafton (FR); Rhinocort (AU); Rhinocort Aqua (HK); Rhinoside (GR); Symbicort (FR, UK, US, ZA) Uceris (US). Research
COVID-19
Budesonide was recommended in April 2021 by the UKs NHS to treat COVID-19 on a case-by-case basis for those aged 50 years of age and older. After a University of Oxford research team found in a trial with 1,700 patients that budesonide could benefit many people over 50 with COVID-19 symptoms, it was recommended from 12 April 2021, by the National Health Service in the UK for general practitioners (GPs) to treat COVID-19 on a case-by-case basis. | 0-1
|
Alveolar soft part sarcoma, abbreviated ASPS, is a very rare type of soft-tissue sarcoma, that grows slowly and whose cell of origin is unknown. ASPS arises mainly in children and young adults and can migrate (metastasize) into other parts of the body, typically the lungs and the brain. Typically, ASPS arises in muscles and deep soft tissue of the thigh or the leg (lower extremities), but can also appear in the upper extremities (hands, neck, and head). While ASPS is a soft tissue sarcoma, it can also spread and grow inside the bones. Etymology
The term alveolar comes from the microscopic pattern, visible during the analysis of slides of ASPS under the microscope in histopathology. The tumor cells seem to be arranged in the same pattern as the cells of the small air sacks (alveoli) in the lungs. However, this is just a structural similarity. ASPS was first described and characterized in 1952.ASPS is a sarcoma, and that indicates that this cancer initially arises from tissue of embryonic mesenchymal origin. (The fertilized egg divides and redivides forming a sphere. Early in embryogenesis, dimples appear in the poles of the sphere and burrow through the sphere forming an inner passage that will ultimately form the gut. Malignancies arising from cells that were originally part of the outer layer of the sphere and those that were part of the embryonic tunnel are termed carcinomas; malignancies arising from the cells between the outer layer and the inner burrow are termed sarcomas.) | References
== External links == | 11
|
If necessary, a decompression surgery that is minimally destructive of normal structures may be used to treat spinal stenosis.Non-surgical treatments for this condition are very similar to the non-surgical methods described above for spinal stenosis. Spinal fusion surgery may be required to treat this condition, with many patients improving their function and experiencing less pain. Nearly half of all vertebral fractures occur without any significant back pain. If pain medication, progressive activity, or a brace or support does not help with the fracture, two minimally invasive procedures - vertebroplasty or kyphoplasty - may be options. Ankles can be stabilized by lightweight orthoses, available in molded plastics as well as softer materials that use elastic properties to prevent foot drop. Additionally, shoes can be fitted with traditional spring-loaded braces to prevent foot drop while walking. Regular exercise is usually prescribed.Functional electrical stimulation (FES) is a technique that uses electrical currents to activate nerves innervating extremities affected by paralysis resulting from spinal cord injury (SCI), head injury, stroke and other neurological disorders. FES is primarily used to restore function in people with disabilities. It is sometimes referred to as Neuromuscular electrical stimulation (NMES)
The latest treatments include stimulation of the peroneal nerve, which lifts the foot when you step. Many stroke and multiple sclerosis patients with foot drop have had success with it. Often, individuals with foot drop prefer to use a compensatory technique like steppage gait or hip hiking as opposed to a brace or splint. | The effect it may have on the baby is still unknown.There is limited data regarding the safety of taking paromomycin while breastfeeding but because the drug is poorly absorbed minimal amounts of drug will be secreted in breastmilk. HIV/AIDS
There is limited evidence that paromomycin can be used in persons coinfected with HIV and Cryptosporidium. A few small trials have showed a reduction in oocyst shedding after treatment with paromomycin. Adverse effects
The most common adverse effects associated with paromomycin sulfate are abdominal cramps, diarrhea, heartburn, nausea, and vomiting. Long-term use of paromomycin increases the risk for bacterial or fungal infection. Signs of overgrowth include white patches in the oral cavities. Other less common adverse events include myasthenia gravis, kidney damage, enterocolitis, malabsorption syndrome, eosinophilia, headache, hearing loss, ringing in the ear, itching, severe dizziness, and pancreatitis. Interactions
Paromomycin belongs to the aminoglycoside drug class and therefore are toxic to the kidneys and to ears. These toxicities are additive and are more likely to occur when used with other drugs that cause ear and kidney toxicity. Concurrent use of foscarnet increases the risk of kidney toxicity. Concurrent use of colistimethate and paromomycin can cause a dangerous slowing of breathing known as respiratory depression, and should be done with extreme caution if necessary. When used with systemic antibiotics such as paromomycin, the cholera vaccine can cause an immune response. Use with strong diuretics, which can also harm hearing, should be avoided. | 0-1
|
Prior to its onset, patients may present with the neurological signs and symptoms of hyponatraemic encephalopathy such as nausea and vomiting, confusion, headache and seizures. These symptoms may resolve with normalisation of the serum sodium concentration. Three to five days later, a second phase of neurological manifestations occurs correlating with the onset of myelinolysis. Observable immediate precursors may include seizures, disturbed consciousness, gait changes, and decrease or cessation of respiratory function.The classical clinical presentation is the progressive development of spastic quadriparesis, pseudobulbar palsy, and emotional lability (pseudobulbar affect), with other more variable neurological features associated with brainstem damage. These result from a rapid myelinolysis of the corticobulbar and corticospinal tracts in the brainstem.In about ten per cent of people with central pontine myelinolysis, extrapontine myelinolysis is also found. In these cases symptoms of Parkinsons disease may be generated. Causes
The most common cause is overly-rapid correction of low blood sodium levels (hyponatremia). Apart from rapid correction of hyponatraemia, there are case reports of central pontine myelinolysis in association with hypokalaemia, anorexia nervosa when feeding is started, patients undergoing dialysis and burn victims. There is a case report of central pontine myelinolysis occurring in the context of refeeding syndrome, in the absence of hyponatremia.It has also been known to occur in patients suffering withdrawal symptoms of chronic alcoholism. In these instances, occurrence may be entirely unrelated to hyponatremia or rapid correction of hyponatremia. | Guaifenesin was studied as a method to improve the possibility of conception, by thinning and increasing the stretchability (improved spinnbarkeit) of the cervical mucus, during the few days before ovulation, thus facilitating sperm penetration.Results from a 2014 study by the Virginia Commonwealth Universitys Department of Pediatrics indicated that guaifenesin did not have significant impact on sputum production or clearance in upper respiratory infections. This was consistent with a 2014 study involving 378 adult and adolescent participants, which indicated guaifenesin had no significant effect as either mucolytic or expectorant compared to placebo: "Although the upper respiratory quality of life improved over the course of the study in both the [guaifenesin] and placebo groups, this improvement was not accompanied by changes in sputum properties". See also
Guaiacol
Guaifenesin/phenylephrine
Methocarbamol
Mephenoxalone
Oxomemazine/guaifenesin
Plastic bronchitis
References
External links
"Guaifenesin". Drug Information Portal. U.S. National Library of Medicine. "F.D.A. Study Worries Makers of Drugs". The New York Times. 20 October 1981. | 0-1
|
Medications
Appetite stimulant medications are used to treat cachexia to increase food intake, but are not effective in stopping muscle wasting and may have detrimental side effects. Appetite stimulants include glucocorticoids, cannabinoids, or progestins such as megestrol acetate. Anti-emetics such as 5-HT3 antagonists are also commonly used in cancer cachexia if nausea is a prominent symptom.Anabolic-androgenic steroids like oxandrolone may be beneficial in cachexia but their use is recommended for a maximum of two weeks since a longer duration of treatment increases side effects. Whilst preliminary studies have suggested thalidomide may be useful, a Cochrane review found no evidence to make an informed decision about the use of this drug in cancer patients with cachexia. Nutrition
The increased metabolic rate and appetite suppression common in cachexia can compound muscle loss. Studies using a calorie-dense protein supplementation have suggested at least weight stabilization can be achieved, although improvements in lean body mass have not been observed in these studies. Supplements
Administration of exogenous amino acids have been investigated to serve as a protein-sparing metabolic fuel by providing substrates for both muscle metabolism and gluconeogenesis. The branched-chain amino acids leucine and valine may have potential in inhibiting overexpression of protein breakdown pathways. The amino acid glutamine has been used as a component of oral supplementation to reverse cachexia in people with advanced cancer or HIV/AIDS.β-hydroxy β-methylbutyrate (HMB) is a metabolite of leucine that acts as a signaling molecule to stimulate protein synthesis. Studies showed positive results for chronic pulmonary disease, hip fracture, and in AIDS‐related and cancer‐related cachexia. | Additionally, it can be diagnosed through sarcopenia, or loss of skeletal muscle mass.Laboratory markers are used in evaluation of people with cachexia, including albumin, prealbumin, C-reactive protein, or hemoglobin. However, laboratory metrics and cut-off values are not standardized across different diagnostic criteria. Acute phase reactants (IL-6, IL-1b, tumor necrosis factor-a, IL-8, interferon-g) are sometimes measured but correlate poorly with outcomes. There are no biomarkers to identify people with cancer who may develop cachexia.In the effort to better classify cachexia severity, several scoring systems have been proposed including the Cachexia Staging Score (CSS) and Cachexia Score (CASCO). The CSS takes into account weight loss, subjective reporting of muscle function, performance status, appetite loss, and laboratory changes to categorize patients into non-cachexia, pre-cachexia, cachexia, and refractory cachexia. The Cachexia SCOre (CASCO) is another validated score that includes evaluation of body weight loss and composition, inflammation, metabolic disturbances, immunosuppression, physical performance, anorexia, and quality of life.Evaluation of changes in body composition is limited by the difficulty in measuring muscle mass and health in a non-invasive and cost-effective way. Imaging with quantification of muscle mass has been investigated including bioelectrical impedance analysis, computed tomography, dual-energy X-ray absorptiometry (DEXA), and magnetic resonance imaging but are not widely used. Definition
Identification, treatment, and research of cachexia have historically been limited by the lack of a widely accepted definition of cachexia. | 11
|
In the opinion of Public Citizen, an advocacy group, the FDA erroneously allowed it to be labelled without stating that it is only approved for use by totally blind people. However, FDA updated its press release on Oct. 2, 2014 to clarify the approved use of Hetlioz, which includes both sighted and blind individuals. The update did not change the drug labeling (prescribing information). Toxicity
Experiments with rodents revealed fertility impairments, an increase in certain cancers, and serious adverse events during pregnancy at dosages in excess of what is considered the "human dose". See also
Discovery and development of melatonin receptor agonists
References
External links
"Tasimelteon". Drug Information Portal. U.S. National Library of Medicine. | A small assessment of sustained release of 0.4 mg belladonna alkaloids and ~60 mg phenobarbital (1 grain) was performed in patients with functional GI disorders (i.e., gastric hyperacidity, dyspepsia, pyrosis, gas pains and epigastric distress. Overall, 23 of 25 patients reported complete or significant relief of symptoms. Hock also examined the effect of 0.25 mg belladonna alkaloids and 50 mg phenobarbital sustained release formulation in 82 clinical practice patients with various gastrointestinal disturbances including “functional distress” over 27 months. Of the 82 total patients, 33 patients reported a 50-75% and 20 reported a 75-100% improvement. There were specific reports of improvements in pain and bowel habits in over half of the patients. One of the earliest randomized double-blind clinical trials of belladonna alkaloids + phenobarbital was in 1959 by Lichstein et al. The study involved 75 patients with unstable bowel (whose symptoms are typical or similar to a current diagnosis of IBS) to investigate the combination therapy of an anticholinergic with the addition of phenobarbital against placebo over 15 months. Of these patients, 20 were treated with placebo, 43 were treated with 50 mg phenobarbital in combination with 0.25 mg Belladonna alkaloids, and 12 received both therapies (patients who lacked a response were switched therapy). Of those receiving the belladonna alkaloids / phenobarbital only, 75.6% reported a mean improvement (2+ or better) in all categories. Among the placebo patients only, only 29.8% reported mean improvement in symptoms. | 0-1
|
A 2005 study by the Mayo Clinic noted that individuals with RA have a doubled risk of heart disease, independent of other risk factors such as diabetes, excessive alcohol use, and elevated cholesterol, blood pressure and body mass index. The mechanism by which RA causes this increased risk remains unknown; the presence of chronic inflammation has been proposed as a contributing factor. It is possible that the use of new biologic drug therapies extend the lifespan of people with RA and reduce the risk and progression of atherosclerosis. This is based on cohort and registry studies, and still remains hypothetical. It is still uncertain whether biologics improve vascular function in RA or not. There was an increase in total cholesterol and HDLc levels and no improvement of the atherogenic index. Epidemiology
RA affects between 0.5 and 1% of adults in the developed world with between 5 and 50 per 100,000 people newly developing the condition each year. In 2010 it resulted in about 49,000 deaths globally.Onset is uncommon under the age of 15 and from then on the incidence rises with age until the age of 80. Women are affected three to five times as often as men.The age at which the disease most commonly starts is in women between 40 and 50 years of age, and for men somewhat later. | Pathological specimens to be obtained for investigations include:
Sputum for culture, AAFBS and gram stain
Blood for full hemogram/complete blood count, ESR and other acute phase reactants
Procalcitonin test, more specificOn a posteroanterior and lateral chest radiograph, an entire lobe will be radiopaque, which is indicative of lobar pneumonia. The identification of the infectious organism (or other cause) is an important part of modern treatment of pneumonia. The anatomical patterns of distribution can be associated with certain organisms, and can help in selection of an antibiotic while waiting for the pathogen to be cultured. References
External links
Media related to Lobar pneumonia at Wikimedia Commons | 0-1
|
Medication
If the separation alone is not working, antipsychotics are often prescribed for a short time to prevent the delusions. Antipsychotics are medications that reduce or relieve symptoms of psychosis such as delusions or hallucinations (seeing or hearing something that is not there). Other uses of antipsychotics include stabilizing moods for people with mood swings and mood disorders ( i.e. in bipolar patients), reducing anxiety in anxiety disorders, and lessening tics in people with Tourettes. Antipsychotics do not cure psychosis, but they do help reduce symptoms; when paired with therapy, the person with the condition has the best chance of recovering. While antipsychotics are powerful, and often effective, they do have side effects, such as inducing involuntary movements. They should only be taken if absolutely required, and under the supervision of a psychiatrist. Therapy
The two most common forms of therapy for people with shared delusional disorder are personal and family therapy.Personal therapy is one-on-one counseling that focuses on building a relationship between the counselor and the patient, and aims to create a positive environment where the patient feels that they can speak freely and truthfully. This is advantageous, as the counselor can usually get more information out of the patient to get a better idea of how to help them. Additionally, if the patient trusts what the counselor says, disproving the delusion will be easier.Family therapy is a technique in which the entire family comes into therapy together to work on their relationships, and to find ways to eliminate the delusion within the family dynamic. | Enchondromatosis is a form of osteochondrodysplasia characterized by a proliferation of enchondromas. Ollier disease can be considered a synonym for enchondromatosis. Maffucci syndrome is enchondromatosis with hemangiomatosis. References
== External links == | 0-1
|
Each innominate bone (ilium) joins the femur (thigh bone) to form the hip joint; thus the sacroiliac joint moves with walking and movement of the torso.In this joint, hyaline cartilage on the sacral side moves against fibrocartilage on the iliac side. The sacroiliac joint contains numerous ridges and depressions that function in stability. Studies have documented that motion does occur at the joint; therefore, slightly subluxed and even locked positions can occur.Muscles and ligaments surround and attach to the SI joint in the front and back, primarily on the ilial or sacral surfaces. These can all be a source of pain and inflammation if the SI joint is dysfunctional. The sacroiliac joint is highly dependent on its strong ligamentous structure for support and stability. The most commonly disrupted and/or torn ligaments are the iliolumbar ligament and the posterior sacroiliac ligament. The ligamentous structures offer resistance to shear and loading. The deep anterior, posterior, and interosseous ligaments resist the load of the sacrum relative to the ilium. More superficial ligaments (e.g., the sacrotuberous ligament) react to dynamic motions (such as straight-leg raising during physical motion). The long dorsal sacroiliac ligament can become stretched in periods of increased lumbar lordosis (e.g., during pregnancy). Affected muscle groups
Many large and small muscles have relationships with the ligaments of the sacroiliac joint including the piriformis (see "piriformis syndrome", a condition often related with sacroiliac joint dysfunction), rectus femoris, gluteus maximus and minimus, erector spinae, latissimus dorsi, thoracolumbar fascia, and iliacus. | In the acute phase (first 1–2 weeks) for a mild sprain of the sacroiliac, it is typical for the patient to be prescribed rest, ice/heat, spinal manipulation, and physical therapy; anti-inflammatory medicine can also be helpful.If the pain does not resolve in the first 1–2 weeks, then the patient may benefit from a steroid and anesthetic mixture fluoroscopically injected into the joint (this also serves in confirming diagnosis), as well as manipulative or manual therapy. For the most severe and chronic forms of sacroiliac dysfunction, treatment should proceed with the support of a sacroiliac belt, injection therapy, and finally, surgery. The anti-inflammatory effect of injection therapy is not permanent, and the injections do not offer an opportunity to stabilize an incompetent joint. Surgery is often considered a last resort, but for some patients, it is the only method of effectively stabilizing the loose joint. A fixation of the joint (screws or similar hardware only, without the use of bone grafting) is more common than a spinal fusion, as it is much less invasive, surgically straightforward, and results in a quicker recovery time for the patient. Some experts in the field believe that it is important to make sure the sacroiliac joint is in an anatomically correct position prior to fixation or fusion, but published research contradicts this belief.Platelet-rich plasma (PRP) injections have shown positive results as a treatment for Sacroiliac Joint Dysfunction, with randomized trials & case reports showing them to be more effective over periods of 3 months than steroid injections. | 11
|
Although it has not been fully explored, one investigation of lower limb amputation observed that as stump length decreased, there was a greater incidence of moderate and severe phantom pain. See also
Phantom limb
Phantom eye syndrome
References
External links
MacLachlan, Malcolm; McDonald, Dympna; Waloch, Justine (2004), "Mirror Treatment of Lower Limb Phantom Pain: A Case Study", Disability and Rehabilitation, 26 (14/15): 901–904, doi:10.1080/09638280410001708913, PMID 15497919, S2CID 36325980Richardson, Cliff; Glenn, Sheila; Horgan, Maureen; Nurmikko, Turo (October 2007), "A Prospective Study of Factors Associated with the Presence of Phantom Limb Pain Six Months After Major Lower Limb Amputation in Patients with Peripheral Vascular Disease", The Journal of Pain, 8 (10): 793–801, doi:10.1016/j.jpain.2007.05.007, PMID 17631056Targeted Muscle Reinnervation | He was brought over by Operation Smile to receive the major surgery in Virginia, at the Childrens Hospital of the Kings Daughters. The other facial defects within the fifty-minute programme consisted of children with facial cleft and cleft lip and palate which may be associated with encephalocele. In November 2006, there was an hour-long documentary on the British television network Channel 4 about Facing the World, an organization that helps children with severe facial disfigurements in developing countries. One of the children featured on the documentary was Ney, a Cambodian boy who had a severe form of encephalocele, wherein part of his brain protruded through his face. On December 4, 2012, Dr. Meara again led a cranio-facial surgical team to remove the encephalocele of an infant, Dominic Gundrum, the son of a Wisconsin Court of Appeals judge and his wife. The surgery also closed the babys skull, repaired a Tessier facial cleft, and brought the babys facial features together. See also
Cephalic disorder
Knobloch syndrome
References
9. Chaturvedi J, Goyal N, Arora RK, Govil N. Giant occipitocervical encephalocele. J Neurosci Rural Pract 2018;9:414-6
External links
encephaloceles at NINDS | 0-1
|
Stomatitis is inflammation of the mouth and lips. It refers to any inflammatory process affecting the mucous membranes of the mouth and lips, with or without oral ulceration.In its widest meaning, stomatitis can have a multitude of different causes and appearances. Common causes include infections, nutritional deficiencies, allergic reactions, radiotherapy, and many others. When inflammation of the gums and the mouth generally presents itself, sometimes the term gingivostomatitis is used, though this is also sometimes used as a synonym for herpetic gingivostomatitis. The term is derived from the Greek stoma (στόμα), meaning "mouth", and the suffix -itis (-ῖτις), meaning "inflammation". Causes
Nutritional deficiency
Malnutrition (improper dietary intake) or malabsorption (poor absorption of nutrients into the body) can lead to nutritional deficiency states, several of which can lead to stomatitis. For example, deficiencies of iron, vitamin B2 (riboflavin),: 490 vitamin B3 (niacin), vitamin B6 (pyridoxine), vitamin B9 (folic acid) or vitamin B12 (cobalamine) may all manifest as stomatitis. Iron is necessary for the upregulation of transcriptional elements for cell replication and repair. Lack of iron can cause genetic downregulation of these elements, leading to ineffective repair and regeneration of epithelial cells, especially in the mouth and lips. Many disorders which cause malabsorption can cause deficiencies, which in turn causes stomatitis. Examples include tropical sprue. : 49
Aphthous stomatitis
Aphthous stomatitis (canker sores) is the recurrent appearance of mouth ulcers in otherwise healthy individuals. The cause is not completely understood, but it is thought that the condition represents a T cell mediated immune response which is triggered by a variety of factors. | Apart from not being restricted to the tongue, migratory stomatitis is an identical condition in every regard to geographic tongue. Another synonym for geographic tongue which uses the term stomatitis is "stomatitis areata migrans". Herpetic gingivostomatitis
This is inflammation of the mouth caused by herpes simplex virus. Irradiation and chemotherapy
Stomatitis may also be caused by chemotherapy, or radiation therapy of the oropharyngeal area. The term mucositis is sometimes used synonymously with stomatitis, however the former usually refers to mucosal reactions to radiotherapy or chemotherapy, and may occur anywhere in the gastrointestinal tract and not just in the mouth. Necrotizing ulcerative gingivostomatitis
The term necrotizing ulcerative gingivostomatitis is sometimes used as a synonym of the necrotizing periodontal disease more commonly termed necrotizing ulcerative gingivitis, or a more severe form (also termed necrotizing stomatitis). The term necrotizing gingivostomatitis is also sometimes used. Stomatitis nicotina
Also called smokers palatal keratosis,: 176 this condition may occur in smokers, especially pipe smokers. The palate appears dry and cracked, and white from keratosis. The minor salivary glands appear as small, red and swollen bumps. It is not a premalignant condition, and the appearance reverses if the smoking is stopped. : 176
Chronic ulcerative stomatitis
Chronic ulcerative stomatitis is a condition with specific immunopathologic features, which was first described in 1990. It is characterized by erosions and ulcerations which relapse and remit. Lesions are located on the buccal mucosa (inside of the cheeks) or on the gingiva (gums). The condition resembles oral lichen planus when biopsied. | 11
|
Although this scale is currently seen as the gold standard to evaluate patients with DCP in research, substantial time and experience with the current CA and DYS definitions is needed from the rater in scoring dystonia and choreoathetosis.Both the DIS and BFMS can be used as outcome measure in intervention studies such as deep brain stimulation (DBS) or intrathecal baclofen. Aim of treatment interventions
Dyskinetic cerebral palsy is a non-progressive, non-reversible disease. The current management is symptomatic, since there is no cure. The main goal is to improve daily activity, quality of life and autonomy of the children by creating a timed and targeted management. The many management options for patients with DCP are not appropriate as standalone treatment but must be seen within an individualized multidisciplinary rehabilitation program. Medical and rehabilitation interventions
Management options can be subdivided into medical treatment and rehabilitation interventions. Medical treatment consists of oral medication and surgery. Before using oral drugs, it is important to differentiate between spasticity, dystonia and choreoathetosis since each motor disorder has a specific approach. In general, many oral drugs have low efficacy, unwanted side-effects and variable effects. Oral baclofen and trihexyphenidyl are commonly used to decrease dystonia, although its efficacy is relatively low in most patients. Adverse effects of the latter can include worsening of choreoathetosis. Since dystonia predominates over choreoathetosis in most patients, reducing dystonia allows the possibility of a full expression of choreoathetosis. | It is instead hypothesized that women with PMDD are more sensitive to normal levels of hormone fluctuations, predominantly estrogen and progesterone, which produces biochemical events in the nervous system that cause the premenstrual symptoms. These symptoms are more predominant in women who have a predisposition to the disorder.It is apparent that the premenstrual disorders are biologically driven and are not only psychological or cultural phenomena. PMDD has been reported by menstruating women worldwide, indicating a biological basis that is not geographically selective. Most psychologists infer that this disorder is caused by both a reaction to hormone flux and also genetic components. There is evidence of heritability of (retrospectively-reported) premenstrual symptoms from several twin and family studies done in the 1990s, with the heritability of PMDD proving to be about 56%. Genetic factors
While whether or not this disorder has a specific genetic basis is still being discussed in the academic community and the possible genetic factors contributing to PMDD have yet to be thoroughly researched, there has recently been multiple genetic factors identified that contribute to the moodiness, depression, irritability, increased appetite, trouble sleeping, acne, fluid retention, headaches, nausea, and other symptoms that are all associated with this disorder. Many studies have noted that a polymorphism of the brain-derived neurotrophic factor gene (BDNF), a gene that helps support neurons in their function and survival in the brain by creating a protein that helps in the growth, maturation, and maintenance of these cells, may play a role in causing PMDD symptoms. | 0-1
|
Furthermore, according to ASHA, organic dysphonia can be subdivided into structural and neurogenic; neurogenic dysphonia is defined as impaired functioning of the vocal structure due to a neurological problem (in the central nervous system or peripheral nervous system); in contrast, structural dysphonia is defined as impaired functioning of the vocal mechanism that is caused by some sort of physical change (e.g. a lesion on the vocal folds). Notably, an additional subcategory of functional dysphonia recognized by professionals is psychogenic dysphonia, which can be defined as a type of voice disorder that has no known cause and can be presumed to be a product of some sort of psychological stressors in ones environment. It is important to note that these types are not mutually exclusive and much overlap occurs. For example, Muscle Tension Dysphonia (MTD) has been found to be a result of many different causes including the following: MTD in the presence of an organic pathology (i.e. organic type), MTD stemming from vocal use (i.e. functional type), and MTD as a result of personality and/or psychological factors (i.e. psychogenic type). Causes
The most common causes of hoarseness is laryngitis (acute 42.1%; chronic 9.7%) and functional dysphonia (30%). Hoarseness can also be caused by laryngeal tumours (benign 10.7 - 31%; malignant 2.2 - 3.0%). Causes that are overall less common include neurogenic conditions (2.8 - 8.0%), psychogenic conditions (2.0 - 2.2%), and aging (2%).A variety of different causes, which result in abnormal vibrations of the vocal folds, can cause dysphonia. | Palatal myoclonus is a rare condition in which there are rhythmic jerky movements or a rapid spasm of the palatal (roof of the mouth) muscles. Chronic clonus is often due to lesions of the central tegmental tract (which connects the red nucleus to the ipsilateral inferior olivary nucleus).When associated with eye movements, it is known as oculopalatal myoclonus. Signs and symptoms
Signs and symptoms of Palatal Myoclonus include:
- A rhythmic clicking sound in the ear due to the opening and closing of the Eustachian tube.- Rhythmic, jerky movements in the face, eyeballs, tongue, jaw, vocal cord or extremities (mostly hands). Diagnosis
Classifications
physiologic, essential, epileptic, and symptomatic
Treatment
Drugs
Drugs used to treat palatal myoclonus include clonazepam, carbamazepine, baclofen, anticholinergics, tetrabenazine, valproic acid, phenytoin, lamotrigine, sumatriptan, and PIR. A rare case of palatal myoclonus that associated with orofacial buccal dystonia has been treated with Botulinum toxin A (Dysport) injection and counseling. Notes
http://www.tchain.com/otoneurology/disorders/central/opm.html
https://www.hindawi.com/journals/criot/2013/231505/
"NINDS Tremor Information Page". National Institute of Neurological Disorders and Stroke. July 20, 2007. Archived from the original on October 6, 2007. Retrieved 2007-10-08. References
== External links == | 0-1
|
An avalanche is a rapid flow of snow down a slope, such as a hill or mountain.Avalanches can be set off spontaneously, by such factors as increased precipitation or snowpack weakening, or by external means such as humans, animals, and earthquakes. Primarily composed of flowing snow and air, large avalanches have the capability to capture and move ice, rocks, and trees. Avalanches occur in two general forms, or combinations thereof: slab avalanches made of tightly packed snow, triggered by a collapse of an underlying weak snow layer, and loose snow avalanches made of looser snow. After being set off, avalanches usually accelerate rapidly and grow in mass and volume as they capture more snow. If an avalanche moves fast enough, some of the snow may mix with the air, forming a powder snow avalanche. Though they appear to share similarities, avalanches are distinct from slush flows, mudslides, rock slides, and serac collapses. They are also different from large scale movements of ice. Avalanches can happen in any mountain range that has an enduring snowpack. They are most frequent in winter or spring, but may occur at any time of year. In mountainous areas, avalanches are among the most serious natural hazards to life and property, so great efforts are made in avalanche control. There are many classification systems for the different forms of avalanches, which vary according to their users needs. Avalanches can be described by their size, destructive potential, initiation mechanism, composition, and dynamics. | The severity of the condition is not linked to the duration of tamsulosin intake. References
== External links == | 0-1
|
Beckwith–Wiedemann syndrome (; abbreviated BWS) is an overgrowth disorder usually present at birth, characterized by an increased risk of childhood cancer and certain congenital features. A minority (<15%) cases of BWS are familial, meaning that a close relative may also have BWS, and parents of an affected child may be at increased risk of having other children with BWS. While children with BWS are at increased risk of childhood cancer, most children with BWS do not develop cancer and the vast majority of children who do develop cancer can be treated successfully. Presentation
No consensus clinical diagnostic criteria for Beckwith–Wiedemann syndrome (BWS) exist. Beckwith–Wiedemann syndrome (BWS) should be suspected in individuals who have one or more of the following major and/or minor findings.Major findings associated with BWS
Macrosomia (traditionally defined as weight and length/height >97th centile)
Macroglossia
Hemihyperplasia (asymmetric overgrowth of one or more regions of the body)
Omphalocele (also called exomphalos) or umbilical hernia
Embryonal tumor (e.g., Wilms tumor, hepatoblastoma, neuroblastoma, rhabdomyosarcoma) in childhood
Visceromegaly involving one or more intra-abdominal organs including liver, spleen, kidneys, adrenal glands, and/or pancreas
Cytomegaly of the fetal adrenal cortex (pathognomonic)
Renal abnormalities including structural abnormalities, nephromegaly, nephrocalcinosis, and/or later development of medullary sponge kidney
Anterior linear ear lobe creases and/or posterior helical ear pits
Placental mesenchymal dysplasia
Cleft palate (rare in BWS)
Cardiomyopathy (rare in BWS)
Positive family history (≥1 family members with a clinical diagnosis of BWS or a history or features suggestive of BWS)Minor findings associated with BWS
Pregnancy-related findings including polyhydramnios and prematurity in fetuses with the condition. | With proper testing and diagnosis, the mortality rate falls to less than 1%. Antibiotics such as azithromycin are particularly effective in treating the disease. Epidemiology
Factors outside the household, such as unclean food from street vendors and flooding, help distribute the disease from person to person. Because of poverty and poor hygiene and insanitary conditions, the disease is more common in less-industrialized countries, principally owing to the problem of unsafe drinking water, inadequate sewage disposal, and flooding. Occasionally causing epidemics, paratyphoid fever is found in large parts of Asia, Africa, and Central and South America. Many of those infected get the disease in Asian countries. About 16 million cases occur a year, which result in about 25,000 deaths worldwide. References
Further reading
"Typhoid and Paratyphoid Fever." Communicable Disease Management Protocol. November 2001 https://www.gov.mb.ca/health/publichealth/cdc/protocol/typhoid.pdf. "Typhoid and Paratyphoid Fever." Public Health Notifiable Disease Management Guidelines. Disease Control and Prevention. Alberta Health and Wellness: June 2013 https://web.archive.org/web/20130925214850/http://www.health.alberta.ca/documents/Guidelines-Paratyphoid-Fever-2013.pdf | 0-1
|
Aspirational biopsy of odontogenic keratocysts contains a greasy fluid which is pale in colour and contains keratotic squames. Protein content of cyst fluid below 4g% is diagnostic of odontogenic keratocysts. Smaller and unilocular lesions resembling other types of cysts may require a biopsy to confirm the diagnosis. On a CT scan, the radiodensity of a keratocystic odontogenic tumour is about 30 Hounsfield units, which is about the same as ameloblastomas. However, ameloblastomas show more bone expansion and seldom show high density areas.Radiographs of odontogenic keratocysts show well-defined radiolucent areas with rounded or scalloped margins which are well demarcated. These areas can be multilocular or unilocular. The growth pattern of the lesion is very characteristic from which a diagnosis can be made as there is growth and spread both forward and backward along the medullary cavity with little expansion. No resorption of teeth or inferior dental canal and minimal displacement of teeth is seen. Due to lack of expansion of the odontogenic keratocyst, the lesion can be very large when radiographically discovered. Differential diagnosis
Radiologically
Odontogenic myxoma
Ameloblastoma
Central giant-cell granuloma
Adenomatoid odontogenic tumor
Dentigerous cyst (follicular cyst)Histologically
Orthokeratocyst
Radicular cyst (particularly if the OKC is very inflamed)
Ameloblastoma
Histology
Odontogenic keratocysts have a diagnostic histological appearance. Under the microscope, OKCs vaguely resemble keratinized squamous epithelium; however, they lack rete ridges and often have an artifactual separation from their basement membrane.The fibrous wall of the cyst is usually thin and uninflamed. | The epithelial lining is thin with even thickness and parakeratinised with columnar cells in the basal layer which have focal reverse polarisation (nuclei are on the opposite pole of the cell). The basal cells are an indication of the odontogenic origin as they resemble pre-ameloblasts. The epithelium can separate from the wall, resulting in islands of epithelium. These can go on to form satellite or daughter cysts, leading to an overall multilocular cyst. Presence of daughter cysts is particularly seen in those with NBCCS. Inflamed cysts show hyperplastic epithelium which is no longer characteristic of OKCs and can have resemblance to radicular cysts instead. Due to areas of focal inflammation, a larger biopsy is required for correct diagnosis of odontogenic keratocysts. Treatment
As the condition is quite rare, opinions among experts about how to treat OKCs differ. A 2015 Cochrane review found that there is currently no high quality evidence to suggest the effectiveness of specific treatments for the treatment of odontogenic keratocysts. Treatment depends on extent of multilocularity and cyst. Small multilocular and unilocular cysts can be treated more conservatively through enucleation and curretage. Treatment options for KTOC may vary according to its size, extent, site, and adjacent structures. Treatment options:
Surgical enucleation: surgical removal of the entire epithelial lining of the cyst. Marsupialisation followed by enucleation: this method is carried out by surgeons for larger cysts. Curettage involving simple excision and scraping-out of cavity. Carnoys solution fixative (ethanol, chloroform and acetic acid) which is usually used in conjunction with excision and curretage. | 11
|
However, there was a higher incidence of injection site pain with subcutaneous autoinjection than with intramuscular injection (37.3% vs. 8.2%). Distribution
OHPC is extensively bound to plasma proteins, of which include albumin. Unlike progesterone and 17α-hydroxyprogesterone, OHPC has very low affinity for corticosteroid-binding globulin (less than 0.01% of that of cortisol). Progesterone and 17α-hydroxyprogesterone have low affinity for sex hormone-binding globulin, and for this reason, only a very small fraction of them (less than 0.5%) is bound to this protein in the circulation. Metabolism
OHPC appears to be metabolized primarily by the cytochrome P450 enzymes CYP3A4 and CYP3A5. It may also be metabolized by CYP3A7 in fetal liver and the placenta. Unlike progesterone, OHPC is not metabolized by traditional steroid-transforming enzymes and does not form similar metabolites. The metabolism of OHPC is by reduction, hydroxylation, and conjugation, including glucuronidation, sulfation, and acetylation. The caproate ester of OHPC is not cleaved during metabolism, so 17α-hydroxyprogesterone is not formed from OHPC. As such, OHPC is not a prodrug of 17α-hydroxyprogesterone, nor of progesterone.OHPC has been found to have an elimination half-life of 7.8 days when given by intramuscular injection in an oil-based formulation to non-pregnant women. Its total duration is said to be 10 to 14 days, which is much longer than the duration of intramuscularly administered progesterone in an oil formulation (2 to 3 days). In pregnant women, the elimination half-life of OHPC appears to be longer, about 16 or 17 days. | OHPC has been studied in humans at doses as high as 5,000 mg per week by intramuscular injection, with safety and without glucocorticoid effects observed. The medication does interact with the glucocorticoid receptor however; it has about 4% of the affinity of dexamethasone for the rabbit glucocorticoid receptor. But it acts as a partial agonist of the receptor and has no greater efficacy than progesterone in activating the receptor and eliciting associated gene expression in vitro. Other activities
As a pure progestogen, OHPC has no androgenic, antiandrogenic, estrogenic, or glucocorticoid activity. The absence of androgenic and antiandrogenic activity with OHPC is in contrast to most other 17α-hydroxyprogesterone-derivative progestins. Due to its lack of androgenic properties, similarly to progesterone, OHPC does not have any teratogenic effects on the fetus, making it safe for use during pregnancy. Although OHPC has been described as a pure progestogen, there is evidence that it possesses some antimineralocorticoid activity, similarly to progesterone and 17α-hydroxyprogesterone. This includes clinically important diuretic effects and reversal of estrogen-induced fluid retention and edema. Unlike progesterone, OHPC and its metabolites are not anticipated to interact with non-genomic receptors such as membrane progesterone receptors or the GABAA receptor. In accordance, OHPC is not thought to possess the neurosteroid activities of progesterone or its associated sedative effects.In relation to cytochrome P450 enzymes, OHPC has no effect on CYP1A, CYP2D6, CYP2C9, or CYP3A4, but is a modest inducer of CYP2C19. Differences from progesterone
There are pharmacodynamic differences between progesterone and OHPC, which may have implications for obstetrical use. | 11
|
paliperidone palmitate), which do not appear to carry the same risk. PDSS is characterized by symptoms of delirium (e.g. confusion, difficulty speaking, and uncoordinated movements) and sedation. Most people with PDSS exhibit both delirium and sedation (83%). Although less specific to PDSS, a majority of cases (67%) involved a feeling of general discomfort. PDSS may occur due to accidental injection and absorption of olanzapine pamoate into the bloodstream, where it can act more rapidly, as opposed to slowly distributing out from muscle tissue. Using the proper, intramuscular-injection technique for olanzapine pamoate helps to decrease the risk of PDSS, though it does not eliminate it entirely. This is why the FDA advises that people who are injected with olanzapine pamoate be watched for 3 hours after administration, in the event that PDSS occurs. Animal toxicology
Olanzapine has demonstrated carcinogenic effects in multiple studies when exposed chronically to female mice and rats, but not male mice and rats. The tumors found were in either the liver or mammary glands of the animals. Discontinuation
The British National Formulary recommends a gradual withdrawal when discontinuing antipsychotics to avoid acute withdrawal syndrome or rapid relapse. Symptoms of withdrawal commonly include nausea, vomiting, and loss of appetite. Other symptoms may include restlessness, increased sweating, and trouble sleeping. Less commonly, vertigo, numbness, or muscle pains may occur. Symptoms generally resolve after a short time.Tentative evidence indicates that discontinuation of antipsychotics can result in psychosis. It may also result in reoccurrence of the condition that is being treated. | In contrast, pooled second-generation antipsychotics showed superiority to first-generation antipsychotics only against the discontinuation, negative symptoms (with a much larger effect seen among industry- compared to government-sponsored studies), and cognition scores. Olanzapine caused less extrapyramidal side effects and less akathisia, but caused significantly more weight gain, serum cholesterol increase, and triglyceride increase than haloperidol.A 2012 review concluded that among ten atypical antipsychotics, only clozapine, olanzapine, and risperidone were better than first-generation antipsychotics. A 2011 review concluded that neither first- nor second-generation antipsychotics produce clinically meaningful changes in CGI scores, but found that olanzapine and amisulpride produce larger effects on the PANSS and BPRS batteries than five other second-generation antipsychotics or pooled first-generation antipsychotics. A 2010 Cochrane systematic review found that olanzapine may have a slight advantage in effectiveness when compared to aripiprazole, quetiapine, risperidone, and ziprasidone. No differences in effectiveness were detected when comparing olanzapine to amisulpride and clozapine. A 2014 meta-analysis of nine published trials having minimum duration six months and median duration 52 weeks concluded that olanzapine, quetiapine, and risperidone had better effects on cognitive function than amisulpride and haloperidol. Bipolar disorder
Olanzapine is recommended by the National Institute for Health and Care Excellence as a first-line therapy for the treatment of acute mania in bipolar disorder. Other recommended first-line treatments are haloperidol, quetiapine, and risperidone. | 11
|
With erythromycin treatment, individuals with DPB now have a much longer life expectancy due to better management of symptoms, delay of progression, and prevention of associated infections like P. aeruginosa. The 10-year survival rate for treated DPB is about 90%. In DPB cases where treatment has resulted in significant improvement, which sometimes happens after about two years, treatment has been allowed to end for a while. However, individuals allowed to stop treatment during this time are closely monitored. As DPB has been proven to recur, erythromycin therapy must be promptly resumed once disease symptoms begin to reappear. In spite of the improved prognosis when treated, DPB currently has no known cure. Epidemiology
DPB has its highest prevalence among the Japanese, at 11 per 100,000 population. Korean, Chinese, and Thai individuals with the disease have been reported as well. A genetic predisposition among East Asians is suggested. The disease is more common in males, with the male to female ratio at 1.4–2:1 (or about 5 men to 3 women). The average onset of the disease is around age 40, and two-thirds of those affected are non-smokers, although smoking is not believed to be a cause. The presence of HLA-Bw54 increases the risk of diffuse panbronchiolitis 13.3-fold.In Europe and the Americas, a relatively small number of DPB cases have been reported in Asian immigrants and residents, as well as in individuals of non-Asian ancestry. Misdiagnosis has occurred in the West owing to less recognition of the disease than in Asian countries. | Elevated levels of IgG and IgA (classes of immunoglobulins) may be seen, as well as the presence of rheumatoid factor (an indicator of autoimmunity). Hemagglutination, a clumping of red blood cells in response to the presence of antibodies in the blood, may also occur. Neutrophils, beta-defensins, leukotrienes, and chemokines can also be detected in bronchoalveolar lavage fluid injected then removed from the bronchiolar airways of individuals with DPB, for evaluation. Differential diagnosis
In the differential diagnosis (finding the correct diagnosis between diseases that have overlapping features) of some obstructive lung diseases, DPB is often considered. A number of DPB symptoms resemble those found with other obstructive lung diseases such as asthma, chronic bronchitis, and emphysema. Wheezing, coughing with sputum production, and shortness of breath are common symptoms in such diseases, and obstructive respiratory functional impairment is found on pulmonary function testing. Cystic fibrosis, like DPB, causes severe lung inflammation, excess mucus production, and infection; but DPB does not cause disturbances of the pancreas nor the electrolytes, as does CF, so the two diseases are different and probably unrelated. DPB is distinguished by the presence of lesions that appear on X-rays as nodules in the bronchioles of both lungs; inflammation in all tissue layers of the respiratory bronchioles; and its higher prevalence among individuals with East Asian lineage.DPB and bronchiolitis obliterans are two forms of primary bronchiolitis. Specific overlapping features of both diseases include strong cough with large amounts of often pus-filled sputum; nodules viewable on lung X-rays in the lower bronchi and bronchiolar area; and chronic sinusitis. | 11
|
Lymphangiosarcoma is a rare cancer which occurs in long-standing cases of primary or secondary lymphedema. It involves either the upper or lower lymphedematous extremities but is most common in upper extremities. Although its name implies lymphatic origin, it is believed to arise from endothelial cells and may be more accurately referred to as angiosarcoma. Signs and symptoms
Lymphangiosarcoma may present as a purple discoloration or a tender skin nodule in the extremity, typically on the anterior surface. It progresses to an ulcer with crusting to an extensive necrotic focus involving the skin and subcutaneous tissue. It metastasizes quickly. Causes
It was previously a relatively common complication of the massive lymphedema of the arm which followed removal of axillary (arm pit) lymph nodes and lymphatic channels as part of the classical Halstedian radical mastectomy, as a treatment for breast cancer. The classical radical mastectomy was abandoned in most areas of the world in the late 1960s to early 1970s, being replaced by the much more conservative modified radical mastectomy and, more recently, by segmental breast tissue excision and radiation therapy. Because of this change in clinical practice lymphedema is now a rarity following breast cancer treatment—and post-mastectomy lymphangiosarcoma is now vanishingly rare. When it occurs following mastectomy it is known as Stewart–Treves syndrome. The pathogenesis of lymphangiosarcoma has not been resolved, however several vague mechanisms have been proposed. Stewart and Treves, proposed that a cancer causing agent is present in lymphedematous limbs. Schreiber et al. | proposed that local immunodeficiency as a result of lymphedema results in a "immunologically privileged site" in which the sarcoma is able to develop. Diagnosis
Treatment
The most successful treatment for lymphangiosarcoma is amputation of the affected limb if possible. Chemotherapy may be administered if there is evidence or suspicion of metastatic disease. Evidence supporting the effectiveness of chemotherapy is, in many cases, unclear due to a wide variety of prognostic factors and small sample size. However, there is some evidence to suggest that drugs such as paclitaxel, doxorubicin, ifosfamide, and gemcitabine exhibit antitumor activity. See also
Angiosarcoma
Hemangiosarcoma
Lymphangioma
Sarcoma
Stewart–Treves syndrome
References
== External links == | 11
|
Chorionic hematoma is the pooling of blood (hematoma) between the chorion, a membrane surrounding the embryo, and the uterine wall. It occurs in about 3.1% of all pregnancies, it is the most common sonographic abnormality and the most common cause of first trimester bleeding. Cause and diagnosis
Chorionic hematomas can be caused by the separation of the chorion from the endometrium (inner membrane of the uterus). Hematomas are classified by their location between tissue layers:
Subchorionic hematomas, the most common type, are between the chorion and endometrium. Retroplacental hematomas are entirely behind the placenta and not touching the gestational sac. Subamniotic or preplacental hematomas are contained within amnion and chorion. Rare.Most patients with a small subchorionic hematoma are asymptomatic. Symptoms include vaginal bleeding, abdominal pain, premature labor and threatened miscarriage.Ultrasonography is the preferred method of diagnosis. A chorionic hematoma appears on ultrasound as a hypoechoic crescent adjacent to the gestational sac. The hematoma is considered small if it is under 20% of the size of the sac and large if it is over 50%. Prognosis and treatment
The presence of subchorionic bleeding around the gestational sac does not have a significant association with miscarriage overall. However, the case of intrauterine hematoma observed before 9 weeks of gestational age has been associated with an increased risk of miscarriage. In one study women who complied with instructions for bed rest for the duration of bleeding had a lower rate of miscarriage and a higher rate of term pregnancy than non-compliant women. | The study had several limitations; results were severely confounded by inherent differences between compliant and non-compliant women. == References == | 11
|
Patients will be monitored over time, and treatments will be provided based on the complications that arise. Surgical
Spinal Fusion for patients with severe kyphoscoliosis
Extension Osteotomy to help treat progressive joint limitation
Surgical realignment
Retinal Detachment repair
Myringotomy (surgical procedure to relieve pressure by draining fluid from the eardrum)
Non-surgical
Routine monitoring
Oxygen support, CPAP, Bipap, Mechanical Ventilation
Physical therapy
BracingLike treatment options, the prognosis is dependent on the severity of the symptoms. Despite the various symptoms and limitations, most individuals have normal intelligence and can lead a normal life. Recent research
A recent article in 2015 reported a persistent notochord in a fetus at 23 weeks of gestation. The fetus had an abnormal spine, shortened long bones and a left clubfoot. After running postmortem tests and ultrasound, the researchers believed that the fetus suffered from hypochondrogenesis. Hypochondrogenesis is caused when type II collagen is abnormally formed due to a mutation in the COL2A1 gene. Normally, the cartilaginous notochord develops into the bony vertebrae in a human body. The COL2A1 gene results in malformed type II collagen, which is essential in the transition from collagen to bone. This is the first time that researchers found a persistent notochord in a human body due to a COL2A1 mutation. Eponym
It is named for Wilhelm Kniest. == References == | Orthokeratosis is hyperkeratosis without parakeratosis. No nucleus is seen in the cells. There is also formation of an anuclear keratin layer, as in the normal epidermis. == References == | 0-1
|
HPA-1a is present in 98% of the population of the United States, suggesting that approximately 2% of women who are HPA-1a negative may be at risk for NAIT during pregnancy. Of course, the antigen expression of the father must also be taken into account - in most cases the father is HPA-1a/1a or 1a/1b and the mother is HPA-1b/1b with anti-HPA-1a antibodies. In women of Asian descent, HPA-4 antigens are the most frequently implicated.Studies have shown a relationship between maternal HLA type DRw52a (DRB3* 0101) and the development of anti-HPA-1a.The offending antibodies are IgG subtype and therefore capable of crossing the placenta and entering the fetal circulation.Unlike hemolytic disease of the fetus and newborn, NAIT occurs during the first pregnancy in up to 50% of cases, and the affected fetuses may develop severe thrombocytopenia (<50,000 μL−1) very early during pregnancy (as early as 20 weeks gestation, consistent with the development of platelet antigens, and the majority of the time in utero). Usually, the thrombocytopenia increases as gestation progresses. During the first pregnancy, NAIT is often not detected until birth when the newborn presents with classic symptoms of thrombocytopenia including petechiae, bruising or intracranial hemorrhage. In utero intracranial hemorrhage occurs in about 10% to 30% of affected cases (and NAIT is thought to be the underlying cause in the majority of cases of intracranial hemorrhage due to thrombocytopenia- greater than all other causes of thrombocytopenia combined). | Other conditions causing a low platelet count
Other conditions that can cause a low platelet count in the neonate include bacterial and viral infection, disseminated intravascular coagulation and other rare congenital conditions associated with a low platelet count. Pathophysiology
Platelets have many proteins on their surface. Each person has a different set of proteins, which are inherited from their parents. These different platelet proteins make different platelet groups, just like different proteins on red blood cells make different blood groups. These differences do not affect how the platelets work. However, if a baby inherits a protein that is found on the fathers platelets but is absent from the mothers platelets, the mother may respond to this foreign protein by developing an antibody that fights against it.This antibody may pass from the mothers blood into the babys blood and attach to the babys platelets. This antibody destroys the babys platelets and suppresses production of fetal platelets, they are also thought to weaken the blood vessel walls (vascular integrity) and affect production of new blood vessels (angiogenesis). This results in an increased risk of bleeding for the baby, and this can lead to the babys death. The mothers antibodies can remain in the babys bloodstream for weeks, and bleeding can occur in the baby before birth (fetal), during birth or after birth (neonatal).A number of different proteins can cause NAIT, about 80% of cases are caused by antibodies against platelet antigen HPA-1a, 15% by anti-HPA-5b, and 5% by other antibodies (e.g. HPA-1b, HPA-15, HPA-3 and HPA-9b). | 11
|
If overdose of opium tincture is suspected, rapid professional intervention is required. The primary concern is re-establishing a viable airway and institution of assisted or controlled ventilation if the patient is unable to breathe on their own. Other supportive measures such as the use of vasopressors and oxygen may be indicated to treat cardiac and/or pulmonary failure. Cardiac arrhythmias or arrest will require advanced life-saving measures. Intravenous naloxone or nalmefene, quick-acting opioid antagonists, are the first-line treatment to reverse respiratory depression caused by an overdose of opium tincture. Gastric lavage may be of some use in certain cases. In fiction
In Mary Shelleys novel Frankenstein (1818), Victor Frankenstein takes laudanum as his only means of sleeping and thus preserving his life while in recovery from months of fever and a series of horrible events. In Charles Dickenss novel Oliver Twist (1837), Nancy gave William "Bill" Sikes laudanum to keep him asleep while she ran away to meet Rose Maylie. In Uncle Toms Cabin (1852), an anti-slavery novel by Harriet Beecher Stowe, an enslaved woman named Cassy talks about how she killed her newborn by laudanum overdose to spare him from experiencing the horrors of slavery. In the novel Silas Marner: The Weaver of Raveloe by George Eliot (Mary Ann Evans) (1861), Silas finds and adopts a two-year old girl who had wandered into his house. The girl had been abandoned while walking with her opium-addicted mother, Molly Farren, who had fallen asleep in the snow and died. | In the eighteenth century several physicians published work about it, including John Jones, who wrote The Mysteries of Opium Revealed (1700), which was described by one commentator as "extraordinary and perfectly unintelligible." The Scottish physician John Brown, creator of the Brunonian system of medicine, recommended opium for what he termed asthenic conditions, but his system was discredited by the time of his death. The most influential work was by George Young, who published a comprehensive medical text entitled Treatise on Opium (1753). Young, an Edinburgh surgeon and physician, wrote this to counter an essay on opium by his contemporary Charles Alston, professor of botany and materia medica at Edinburgh who had recommended the use of opium for a wide variety of conditions. Young countered this by emphasising the risks ...that I may prevent such mischief as I can, I here give it as my sincere opinion... that opium is a poison by which great numbers are daily destroyed. Young gives a comprehensive account of the indications for the drug including its complications. He is critical about writers whose knowledge of the drug is based on chemical or animal experiments rather than clinical practice. The treatise is a detailed, balanced and valuable guide to prevailing knowledge and practice. As it gained popularity, opium, and after 1820, morphine, was mixed with a wide variety of agents, drugs and chemicals including mercury, hashish, cayenne pepper, ether, chloroform, belladonna, whiskey, wine and brandy. | 11
|
If someone is at risk for either a mass or raised ICP (recent head injury, a known immune system problem, localizing neurological signs, or evidence on examination of a raised ICP), a CT or MRI scan is recommended prior to the lumbar puncture. This applies in 45% of all adult cases.There are no physical tests that can rule out or determine if a person has meningitis. The jolt accentuation test is not specific or sensitive enough to completely rule out meningitis.If someone is suspected of having meningitis, blood tests are performed for markers of inflammation (e.g. C-reactive protein, complete blood count), as well as blood cultures. If a CT or MRI is required before LP, or if LP proves difficult, professional guidelines suggest that antibiotics should be administered first to prevent delay in treatment, especially if this may be longer than 30 minutes. Often, CT or MRI scans are performed at a later stage to assess for complications of meningitis.In severe forms of meningitis, monitoring of blood electrolytes may be important; for example, hyponatremia is common in bacterial meningitis. The cause of hyponatremia, however, is controversial and may include dehydration, the inappropriate secretion of the antidiuretic hormone (SIADH), or overly aggressive intravenous fluid administration. Lumbar puncture
A lumbar puncture is done by positioning the person, usually lying on the side, applying local anesthetic, and inserting a needle into the dural sac (a sac around the spinal cord) to collect cerebrospinal fluid (CSF). | An alternative is scintigraphy with red blood cells labeled with a radioactive marker; this shows the site of the bleeding on a gamma camera but tends to be unhelpful unless the bleeding is continuous and significant. Treatment
If the anemia is severe, blood transfusion is required before any other intervention is considered. Endoscopic treatment is an initial possibility, where cautery or argon plasma coagulation (APC) treatment is applied through the endoscope. Failing this, angiography and embolization with particles is another microinvasive treatment option, which avoids the need for surgery and bowel resection. Here, the vessel supplying the angiodysplasia is selectively catheterized and embolized with microparticles. Resection of the affected part of the bowel may be needed if the other modalities fail. However, the lesions may be widespread, making such treatment impractical.If the bleeding is from multiple or inaccessible sites, systemic therapy with medication may be necessary. First-line options include the antifibrinolytics tranexamic acid or aminocaproic acid. Estrogens can be used to stop bleeding from angiodysplasia. Estrogens cause mild hypercoagulability of the blood. Estrogen side effects can be dangerous and unpleasant in both sexes. Changes in voice and breast swelling is bothersome in men, but older women often report improvement of libido and perimenopausal symptoms. (The worries about hormone replacement therapy/HRT, however, apply here as well. )In difficult cases, there have been positive reports about octreotide and thalidomide.In severe cases or cases not responsive to either endoscopic or medical treatment, surgery may be necessary to arrest the bleeding. References
== External links == | 0-1
|
False-positive Aspergillus galactomannan tests have been found in patients on intravenous treatment with some antibiotics or fluids containing gluconate or citric acid such as some transfusion platelets, parenteral nutrition, or PlasmaLyte.On microscopy, Aspergillus species are reliably demonstrated by silver stains, e.g., Gridley stain or Gomori methenamine-silver. These give the fungal walls a gray-black colour. The hyphae of Aspergillus species range in diameter from 2.5 to 4.5 μm. They have septate hyphae, but these are not always apparent, and in such cases they may be mistaken for Zygomycota. Aspergillus hyphae tend to have dichotomous branching that is progressive and primarily at acute angles of around 45°. Prevention
Prevention of aspergillosis involves a reduction of mold exposure via environmental infection-control. Antifungal prophylaxis can be given to high-risk patients. Posaconazole is often given as prophylaxis in severely immunocompromised patients. Screening
A systematic review has evaluated the diagnostic accuracy of polymerase chain reaction (PCR) tests in people with defective immune systems from medical treatment such as chemotherapy. Evidence suggests PCR tests have moderate diagnostic accuracy when used for screening for invasive aspergillosis in high risk groups. CT and MRI are vital to diagnosis, however it is always highly recommended to under go a biopsy of the area to confirm a diagnosis. Treatment
The current medical treatments for aggressive invasive aspergillosis include voriconazole and liposomal amphotericin B in combination with surgical debridement. For the less aggressive allergic bronchopulmonary aspergillosis, findings suggest the use of oral steroids for a prolonged period of time, preferably for 6–9 months in allergic aspergillosis of the lungs. | Risk factors
People who are immunocompromised — such as patients undergoing hematopoietic stem cell transplantation, chemotherapy for leukaemia, or AIDS — are at an increased risk for invasive aspergillosis infections. These people may have neutropenia or corticoid-induced immunosuppression as a result of medical treatments. Neutropenia is often caused by extremely cytotoxic medications such as cyclophosphamide. Cyclophosphamide interferes with cellular replication including that of white blood cells such as neutrophils. A decreased neutrophil count inhibits the ability of the body to mount immune responses against pathogens. Although tumor necrosis factor alpha (TNF-α) — a signaling molecule related to acute inflammation responses — is produced, the abnormally low number of neutrophils present in neutropenic patients leads to a depressed inflammatory response. If the underlying neutropenia is not fixed, rapid and uncontrolled hyphal growth of the invasive fungi will occur and result in negative health outcomes. In addition to decreased neutrophil degranulation, the antiviral response against Flu and SARS-CoV-2 viruses, mediated by type I and type II interferon, is diminished jointly with the local antifungal immune response measured in the lungs of patients with IAPA (Influenza-Associated Pulmonary Aspergillosis) and CAPA (COVID-19-Associated Pulmonary Aspergillosis). Diagnosis
On chest X-ray and CT, pulmonary aspergillosis classically manifests as a halo sign, and later, an air crescent sign. In hematologic patients with invasive aspergillosis, the galactomannan test can make the diagnosis in a noninvasive way. | 11
|
There is a suspicion that ADE is not the only mechanism underlying severe dengue-related complications, and various lines of research have implied a role for T cells and soluble factors such as cytokines and the complement system.Severe disease is marked by the problems of capillary permeability (an allowance of fluid and protein normally contained within the blood to pass) and disordered blood clotting. These changes appear associated with a disordered state of the endothelial glycocalyx, which acts as a molecular filter of blood components. Leaky capillaries (and the critical phase) are thought to be caused by an immune system response. Other processes of interest include infected cells that become necrotic—which affect both coagulation and fibrinolysis (the opposing systems of blood clotting and clot degradation)—and low platelets in the blood, also a factor in normal clotting. Diagnosis
The diagnosis of dengue is typically made clinically, on the basis of reported symptoms and physical examination; this applies especially in endemic areas. However, early disease can be difficult to differentiate from other viral infections. A probable diagnosis is based on the findings of fever plus two of the following: nausea and vomiting, rash, generalized pains, low white blood cell count, positive tourniquet test, or any warning sign (see table) in someone who lives in an endemic area. Warning signs typically occur before the onset of severe dengue. | If a person with an IUD develops cervicitis, it usually does not need to be removed, if the person wants to continue using it.There are also certain behaviors that can place individuals at a higher risk for contracting cervicitis. High-risk sexual behavior, a history of STIs, many sexual partners, sex at an early age, and sexual partners who engage in high-risk sexual behavior or have had an STI can increase the likelihood of contracting cervicitis. Diagnosis
To diagnose cervicitis, a clinician will perform a pelvic exam. This exam includes a speculum exam with visual inspection of the cervix for abnormal discharge, which is usually purulent or bleeding from the cervix with little provocation. Swabs can be used to collect a sample of this discharge for inspection under a microscope and/or lab testing for gonorrhea, chlamydia, and Trichomonas vaginalis. A bimanual exam in which the clinician palpates the cervix to see if there is any associated pain should be done to assess for pelvic inflammatory disease. Prevention
The risk of contracting cervicitis from STIs can be reduced by using condoms during every sexual encounter. Condoms are effective against the spread of STIs like chlamydia and gonorrhea that cause cervicitis. Also, being in a long-term monogamous relationship with an uninfected partner can lower the risk of an STI.Ensuring that foreign objects like tampons are properly placed in the vagina and following instructions how long to leave it inside, how often to change it, and/or how often to clean it can reduce the risk of cervicitis. | 0-1
|
Tobacco smoking is the practice of burning tobacco and ingesting the resulting smoke. The smoke may be inhaled, as is done with cigarettes, or simply released from the mouth, as is generally done with pipes and cigars. The practice is believed to have begun as early as 5000–3000 BC in Mesoamerica and South America. Tobacco was introduced to Eurasia in the late 17th century by European colonists, where it followed common trade routes. The practice encountered criticism from its first import into the Western world onwards but embedded itself in certain strata of a number of societies before becoming widespread upon the introduction of automated cigarette-rolling apparatus.Smoking is the most common method of consuming tobacco, and tobacco is the most common substance smoked. The agricultural product is often mixed with additives and then combusted. The resulting smoke is then inhaled and the active substances absorbed through the alveoli in the lungs or the oral mucosa. Many substances in cigarette smoke trigger chemical reactions in nerve endings, which heighten heart rate, alertness and reaction time, among other things. Dopamine and endorphins are released, which are often associated with pleasure.German scientists identified a link between smoking and lung cancer in the late 1920s, leading to the first anti-smoking campaign in modern history, albeit one truncated by the collapse of Nazi Germany at the end of World War II. In 1950, British researchers demonstrated a clear relationship between smoking and cancer. Evidence continued to mount in the 1960s, which prompted political action against the practice. | By 2030, the World Health Organization (WHO) forecasts that 10 million people a year will die of smoking-related illness, making it the single biggest cause of death worldwide, with the largest increase to be among women. WHO forecasts the 21st centurys death rate from smoking to be ten times the 20th centurys rate ("Washingtonian" magazine, December 2007). The tobacco industry is known to be one of the largest global enterprises in the world. The six biggest tobacco companies made a combined profit of $35.1 billion (Jha et al., 2014) in 2010. Social
Famous smokers of the past used cigarettes or pipes as part of their image, such as Jean-Paul Sartres Gauloises-brand cigarettes; Albert Einsteins, Douglas MacArthurs, Bertrand Russells, and Bing Crosbys pipes; or the news broadcaster Edward R. Murrows cigarette. Writers in particular seem to be known for smoking, for example, Cornell Professor Richard Kleins book Cigarettes are Sublime for the analysis, by this professor of French literature, of the role smoking plays in 19th and 20th century letters. The popular author Kurt Vonnegut addressed his addiction to cigarettes within his novels. British Prime Minister Harold Wilson was well known for smoking a pipe in public as was Winston Churchill for his cigars. Sherlock Holmes, the fictional detective created by Sir Arthur Conan Doyle, smoked a pipe, cigarettes, and cigars. The DC Vertigo comic book character John Constantine, created by Alan Moore, is synonymous with smoking, so much so that the first storyline by Preacher creator Garth Ennis centered around John Constantine contracting lung cancer. | 11
|
When glutamine-rich polypeptides are in a β-sheet conformation, glutamines can brace the structure by forming inter-strand hydrogen bonding between its amide carbonyls and nitrogens of both the backbone and side chains. The onset age for Huntingtons disease shows an inverse correlation with the length of the polyglutamine sequence, with analogous findings in a C. elegans model system with engineered polyglutamine peptides.Other polypeptides and proteins such as amylin and the β amyloid peptide do not have a simple consensus sequence and are thought to aggregate through the sequence segments enriched with hydrophobic residues, or residues with high propensity to form β-sheet structure. Among the hydrophobic residues, aromatic amino-acids are found to have the highest amyloidogenic propensity.Cross-polymerization (fibrils of one polypeptide sequence causing other fibrils of another sequence to form) is observed in vitro and possibly in vivo. This phenomenon is important, since it would explain interspecies prion propagation and differential rates of prion propagation, as well as a statistical link between Alzheimers and type 2 diabetes. In general, the more similar the peptide sequence the more efficient cross-polymerization is, though entirely dissimilar sequences can cross-polymerize and highly similar sequences can even be "blockers" that prevent polymerization. Amyloid toxicity
The reasons why amyloid cause diseases are unclear. In some cases, the deposits physically disrupt tissue architecture, suggesting disruption of function by some bulk process. An emerging consensus implicates prefibrillar intermediates, rather than mature amyloid fibers, in causing cell death, particularly in neurodegenerative diseases. | A chest injury, also known as chest trauma, is any form of physical injury to the chest including the ribs, heart and lungs. Chest injuries account for 25% of all deaths from traumatic injury. Typically chest injuries are caused by blunt mechanisms such as direct, indirect, compression, contusion, deceleration, or blasts- caused by motor vehicle collisions or penetrating mechanisms such as stabbings. Classification
Chest injuries can be classified as blunt or penetrating. Blunt and penetrating injuries have different pathophysiologies and clinical courses. Specific types of injuries include:
Injuries to the chest wall
Chest wall contusions or hematomas. Rib fractures
Flail chest
Sternal fractures
Fractures of the shoulder girdle
Pulmonary injury (injury to the lung) and injuries involving the pleural space
Pulmonary contusion
Pulmonary laceration
Pneumothorax
Hemothorax
Hemopneumothorax
Injury to the airways
Tracheobronchial tear
Cardiac injury
Pericardial tamponade
Myocardial contusion
Traumatic arrest
Hemopericardium
Blood vessel injuries
Traumatic aortic rupture
Thoracic aorta injury
Aortic dissection
And injuries to other structures within the torso
Esophageal injury (Boerhaave syndrome)
Diaphragm injury
Diagnosis
Most blunt injuries are managed with relatively simple interventions like tracheal intubation and mechanical ventilation and chest tube insertion. Diagnosis of blunt injuries may be more difficult and require additional investigations such as CT scanning. Penetrating injuries often require surgery, and complex investigations are usually not needed to come to a diagnosis. Patients with penetrating trauma may deteriorate rapidly, but may also recover much faster than patients with blunt injury. See also
Transmediastinal gunshot wound
Commotio thoracis
References
== External links == | 0-1
|
External links
This article incorporates public domain text from The U.S. National Library of Medicine | A myxoid liposarcoma is a malignant adipose tissue neoplasm of myxoid appearance histologically. Myxoid liposarcomas are the second-most common type of liposarcoma, representing 30–40% of all liposarcomas in the limbs, occurring most commonly in the legs, particularly the thigh, followed by the buttocks, retroperitoneum, trunk, ankle, proximal limb girdle, head and neck, and wrist. They occur in the intermuscular fascial planes or deep-seated areas. They present as a large, slow-growing, painless mass.The neoplastic cells in these neoplasms contain chromosomal translocations which create one of two fusion genes: the FUS-DDIT3 in ~90% and the EWSR1-DDIT3 fusion gene in up to 10% of myxoid liposarcoma cases. The FUS-DDIT3 fusion gene forms by a merger of part of the FUS FET gene family gene normally located at band 11.2 on the short (or "p") arm of chromosome 16 with part of the DDIT3 ETS transcription factor family gene normally located at band 13.3 on the long (or "q") arm of chromosome 12. This fusion gene is notated as t(12;16)(q13;p11). Preclinical studies, i.e. laboratory studies, suggest that the FUS-DDIT3 fusion gene may act as an oncogene to promote the development of myxoid liposarmas. The EWSR1-DDIT3 fusion gene forms by a merger of the EWSR1 FET gene family gene located at band 12.2 on the q arm of chromosome 22 with part of the DDIT3 gene. This fusion gene is notated as t(12;22)(q13;12). Additional images
See also
Lipoma
Trabectedin
References
External links
Myxoid liposarcoma: a rare soft-tissue tumor with a misleading benign appearance
Efficacy of first-line doxorubicin and ifosfamide in myxoid liposarcoma. 2012 | 0-1
|
The multipotent stem cells in the bone marrow reconstitute all three blood cell lines, giving the patient a new immune system, red blood cells, and platelets. However, besides the risk of graft failure, there is also a risk that the newly created white blood cells may attack the rest of the body ("graft-versus-host disease"). In young patients with an HLA-matched sibling donor, bone marrow transplant can be considered as a first-line treatment. Patients lacking a matched sibling donor typically pursue immunosuppression as a first-line treatment, and matched, unrelated donor transplants are considered second-line therapy. Treatment often includes a course of antithymocyte globulin (ATG) and several months of treatment with ciclosporin to modulate the immune system. Chemotherapy with agents such as cyclophosphamide may also be effective but is more toxic than ATG. Antibody therapy such as ATG targets T cells, which are believed to attack the bone marrow. Corticosteroids are generally ineffective, though they are used to ameliorate serum sickness caused by ATG. Normally, success is judged by bone marrow biopsy six months after initial treatment with ATG.One prospective study involving cyclophosphamide was terminated early due to a high incidence of mortality from severe infections as a result of prolonged neutropenia.Before the above treatments became available, patients with low leukocyte counts were often confined to a sterile room or bubble (to reduce risk of infection), as in the case of Ted DeVita. Follow-up
Full blood counts are required on a regular basis to determine whether the patient is still in remission. | Rajani may refer to:
Rajani (name), people named Rajani
Rajani (actress) (born 1965), Indian film actress
Rajani (TV series), a 1980s Indian TV series
Rajani (film), a 2009 Indian Kannada romantic comedy
Rajani, an 1877 novel by Bankimchandra Chattopadhyay
See also
Rajini (disambiguation) | 0-1
|
Amblyopia, also called lazy eye, is a disorder of sight in which the brain fails to fully process input from one eye and over time favors the other eye. It results in decreased vision in an eye that typically appears normal in other respects. Amblyopia is the most common cause of decreased vision in a single eye among children and younger adults.The cause of amblyopia can be any condition that interferes with focusing during early childhood. This can occur from poor alignment of the eyes (strabismic), an eye being irregularly shaped such that focusing is difficult, one eye being more nearsighted or farsighted than the other (refractive), or clouding of the lens of an eye (deprivational). After the underlying cause is addressed, vision is not restored right away, as the mechanism also involves the brain. Amblyopia can be difficult to detect, so vision testing is recommended for all children around the ages of four to five.Early detection improves treatment success. Glasses may be all the treatment needed for some children. If this is not sufficient, treatments which encourage or force the child to use the weaker eye are used. This is done by either using a patch or putting atropine in the stronger eye. Without treatment, amblyopia typically persists. Treatment in adulthood is usually much less effective.Amblyopia begins by the age of five. In adults, the disorder is estimated to affect 1–5% of the population. While treatment improves vision, it does not typically restore it to normal in the affected eye. | Given that the refractive correction of anisometropia by means of spectacles typically leads to different image magnification for the two eyes, which may in turn prevent binocular vision, a refractive correction using contact lenses is to be considered. Also pediatric refractive surgery is a treatment option, in particular if conventional approaches have failed due to aniseikonia or lack of compliance or both.Frequently, amblyopia is associated with a combination of anisometropia and strabismus. In some cases, the vision between the eyes can differ to the point where one eye has twice average vision while the other eye is completely blind. Deprivation and occlusion
Deprivation amblyopia (amblyopia ex anopsia) results when the ocular media become opaque, such as is the case with congenital cataract or corneal haziness. These opacities prevent adequate visual input from reaching the eye, and disrupt development. If not treated in a timely fashion, amblyopia may persist even after the cause of the opacity is removed. Sometimes, drooping of the eyelid (ptosis) or some other problem causes the upper eyelid to physically occlude a childs vision, which may cause amblyopia quickly. Occlusion amblyopia may be a complication of a hemangioma that blocks some or all of the eye. Other possible causes of deprivation and occlusion amblyopia include obstruction in the vitreous and aphakia. Deprivation amblyopia accounts for less than 3% of all individuals affected by amblyopia. | 11
|
Leucomatous corneal opacity (leucoma simplex)
Leucomatous corneal opacity is a dense white opacity which results due to scarring of more than half of the stroma. A number of different presentations of leucomatous corneal opacity exist:
Adherent leucoma: results when healing occurs after perforation of cornea with incarceration of iris. The iris is adherent to the back of a leucomatous cornea. One of the major complication of adherent leucoma is Secondary glaucoma
Corneoiridic scar: if iris tissue is incarcerated and incorporated within the scar tissue, as occurs in healing of a large sloughed corneal ulcer, it is called a corneoiridic scar. Corneal facet: corneal surface depressed at the site of healing (due to less fibrous tissue); such a scar is called facet. Kerectasia: In this condition, corneal curvature is increased at the site of opacity (bulge due to weak scar). Presentation
Signs and symptoms include the following:
Loss of vision or blindness (when dense opacity covers the pupillary area)
Blurred vision (due to astigmatic effect and light scattering)
Glare
Complications
Congenital corneal opacity that affecting vision will cause amblyopia. That type of amblyopia is known as form-deprivation amblyopia (or amblyopia ex anopsia). Secondary changes in corneal opacity
Secondary changes may be seen in long-standing cases include: hyaline degeneration, calcareous degeneration, pigmentation and atheromatous ulceration. Causes
Congenital opacities may occur as developmental anomalies or following birth trauma. Causes of congenital corneal opacities include sclerocornea, trauma, ulcer, mucopolysaccharidosis, Peter’s anomaly, congenital hereditary endothelial dystrophy. Ocular trauma
Corneal ulceration
Xerophthalmia, caused by Vitamin A deficiency
Trachoma
Onchocerciasis
Mucous membrane pemphigoid: Ocular form of mucous membrane pemphigoid may cause corneal opacity and loss of vision. | Transient neonatal diabetes mellitus (TNDM) is a form of neonatal diabetes presenting at birth that is not permanent. This disease is considered to be a type of maturity onset diabetes of the young (MODY). Types
Cause
This condition has to do with genetics and is often associated with having an added Chromosome 7 gene (mostly from the paternal side).The form on chromosome 6 can involve imprinting. Diagnosis
Management
See also
Permanent neonatal diabetes mellitus
References
Further reading
GeneReview/NIH/UW entry on 6q24-Related Transient Neonatal Diabetes Mellitus
== External links == | 0-1
|
Though the supraspinatus is the most commonly injured tendon in the rotator cuff, the other three can also be injured at the same time. Acute tears
The amount of stress needed to acutely tear a rotator cuff tendon will depend on the underlying condition of the tendon. If healthy, the stress needed will be high, such as with a fall on the outstretched arm. This stress may occur coincidentally with other injuries such as a dislocation of the shoulder or separation of the acromioclavicular joint. In the case of a tendon with pre-existing degeneration, the force may be more modest, such as with a sudden lift, particularly with the arm above the horizontal position. The type of loading involved with injury is usually eccentric, such as when two people are carrying a load and one lets go, forcing the other to maintain force while the muscle elongates. Chronic tears
Chronic tears are indicative of extended use in conjunction with other factors such as poor biomechanics or muscular imbalance. Ultimately, most are the result of wear that occurs slowly over time as a natural part of aging. They are more common in the dominant arm, but a tear in one shoulder signals an increased risk of a tear in the opposing shoulder.Several factors contribute to degenerative, or chronic, rotator cuff tears of which repetitive stress is the most significant. This stress consists of repeating the same shoulder motions frequently, such as overhead throwing, rowing, and weightlifting. | Repair can be performed through an open incision, again requiring detachment of a portion of the deltoid, while a mini-open technique approaches the tear through a deltoid-splitting approach. The latter may cause less injury to muscle and produce better results. Contemporary techniques now use an all arthroscopic approach. Recovery can take as long as three–six months, with a sling being worn for the first one–six weeks. In the case of partial thickness tears, if surgery is undertaken, tear completion (converting the partial tear to a full tear) and then repair, is associated with better early outcomes than transtendinous repairs (where the intact fibres are preserved) and no difference in failure rates.Biceps tenotomy and tenodesis are often performed concomitantly with rotator cuff repair or as separate procedures, and can also cause shoulder pain. Tenodesis, which may be performed as an arthroscopic or open procedure, generally restores pain free motion it the biceps tendon, or attached portion of the labrum, but can cause pain. Tenotomy is a shorter surgery requiring less rehabilitation, that is more often performed in older patients, though after surgery there can be a cosmetic popeye sign visible in thin arms.In a small minority of cases where extensive arthritis has developed, an option is shoulder joint replacement (arthroplasty). Specifically, this is a reverse shoulder replacement, a more constrained form of shoulder arthroplasty that allows the shoulder to function well even in the presence of large full thickness rotator cuff tears. | 11
|
Alcoholic neuropathy
Cerebral palsy
Leprosy
Syphilis (tabes dorsalis), caused by the organism Treponema pallidum
Spinal cord injury
Myelomeningocele
Syringomyelia
Intra-articular steroid injections
Congenital insensitivity to pain
Peroneal muscular atrophy
Underlying mechanisms
Two primary theories have been advanced:
Neurotrauma: Loss of peripheral sensation and proprioception leads to repetitive microtrauma to the joint in question; this damage goes unnoticed by the neuropathic patient, and the resultant inflammatory resorption of traumatized bone renders that region weak and susceptible to further trauma. In addition, poor fine motor control generates unnatural pressure on certain joints, leading to additional microtrauma. Neurovascular: Neuropathic patients have dysregulated autonomic nervous system reflexes, and de-sensitized joints receive significantly greater blood flow. The resulting hyperemia leads to increased osteoclastic resorption of bone, and this, in concert with mechanical stress, leads to bony destruction.In reality, both of these mechanisms probably play a role in the development of a Charcot joint. Joint involvement
Diabetes is the foremost cause in America today for neuropathic joint disease, and the foot is the most affected region. In those with foot deformity, approximately 60% are in the tarsometatarsal joints (medial joints affected more than lateral), 30% Metatarsophalangeal joints and 10% have ankle disease. Over half of diabetic patients with neuropathic joints can recall some kind of precipitating trauma, usually minor. Patients with neurosyphilis tend to have knee involvement, and patients with syringomyelia of the spinal cord may demonstrate shoulder deformity.Hip joint destruction is also seen in neuropathic patients. Diagnosis
Clinical findings
Clinical findings include erythema, edema and increased temperature in the affected joint. In neuropathic foot joints, plantar ulcers may be present. | Duration and aggressiveness of offloading (non-weight-bearing vs. weight-bearing, non-removable vs. removable device) should be guided by clinical assessment of healing of neuropathic arthropathy based on edema, erythema, and skin temperature changes. It can take six to nine months for the edema and erythema of the affected joint to recede. Outcome
Outcomes vary depending on the location of the disease, the degree of damage to the joint, and whether surgical repair was necessary. Average healing times vary from 55 to 97 days, depending on location. Up to one to two years may be required for complete healing. Further reading
Neuropathic osteoarthropathy by Monica Bhargava, M.D., University of Washington Department of Radiology
John R. Crockarell; Daugherty, Kay; Jones, Linda Winstead; Frederick M. Azar; Beaty, James H; James H. Calandruccio; Peter G. Carnesale; Kevin B. Cleveland; Andrew H. Crenshaw (2003). Campbells Operative Orthopedics (10th ed.). Saint Louis, MO: C.V. Mosby. ISBN 0-323-01248-5. Gupta R (November 1993). "A short history of neuropathic arthropathy". Clinical Orthopaedics and Related Research (296): 43–9. PMID 8222448. Sommer TC, Lee TH (November 2001). "Charcot foot: the diagnostic dilemma". American Family Physician. 64 (9): 1591–8. PMID 11730314. References
== External links == | 11
|
Both drugs are also potent inhibitors of CYP2D6.The extremely slow elimination of fluoxetine and its active metabolite norfluoxetine from the body distinguishes it from other antidepressants. With time, fluoxetine and norfluoxetine inhibit their own metabolism, so fluoxetine elimination half-life increases from 1 to 3 days, after a single dose, to 4 to 6 days, after long-term use. Similarly, the half-life of norfluoxetine is longer (16 days) after long-term use. Therefore, the concentration of the drug and its active metabolite in the blood continues to grow through the first few weeks of treatment, and their steady concentration in the blood is achieved only after four weeks. Moreover, the brain concentration of fluoxetine and its metabolites keeps increasing through at least the first five weeks of treatment. For major depressive disorder, while onset of antidepressant action may be felt as early as 1-2 weeks, the full benefit of the current dose a patient receives is not realized for at least a month following ingestion. For example, in one 6-week study, the median time to achieving consistent response was 29 days. Likewise, complete excretion of the drug may take several weeks. During the first week after treatment discontinuation, the brain concentration of fluoxetine decreases by only 50%, The blood level of norfluoxetine four weeks after treatment discontinuation is about 80% of the level registered by the end of the first treatment week, and, seven weeks after discontinuation, norfluoxetine is still detectable in the blood. | Taking fluoxetine 20 mg/d can be effective in treating PMDD, though doses of 10 mg/d have also been prescribed effectively. Impulsive aggression
Fluoxetine is considered a first-line medication for the treatment of impulsive aggression of low intensity. Fluoxetine reduced low intensity aggressive behavior in patients in intermittent aggressive disorder and borderline personality disorder. Fluoxetine also reduced acts of domestic violence in alcoholics with a history of such behavior. Special populations
In children and adolescents, fluoxetine is the antidepressant of choice due to tentative evidence favoring its efficacy and tolerability. In pregnancy, fluoxetine is considered a category C drug by the US Food and Drug Administration (FDA). Evidence supporting an increased risk of major fetal malformations resulting from fluoxetine exposure is limited, although the Medicines and Healthcare products Regulatory Agency (MHRA) of the UK has warned prescribers and patients of the potential for fluoxetine exposure in the first trimester (during organogenesis, formation of the fetal organs) to cause a slight increase in the risk of congenital cardiac malformations in the newborn. Furthermore, an association between fluoxetine use during the first trimester and an increased risk of minor fetal malformations was observed in one study.However, a systematic review and meta-analysis of 21 studies – published in the Journal of Obstetrics and Gynaecology Canada – concluded, "the apparent increased risk of fetal cardiac malformations associated with maternal use of fluoxetine has recently been shown also in depressed women who deferred SSRI therapy in pregnancy, and therefore most probably reflects an ascertainment bias. | 11
|
the subject must never have met the criteria for any other type of schizophrenia, or any other psychotic disorder. 3. Absence of evidence of dementia or any other organic mental disorder. The ICD is currently in revision 10, and the ICD-11 was accepted in May 2019 will come into effect in 2022. In the ICD-11, there is no longer a diagnostic category of simple schizophrenia, and all subtypes of schizophrenia have been eliminated. DSM
Simple-type schizophrenia also appeared in the first two editions of the DSM as an official diagnosis:
This psychosis is characterized chiefly by a slow and insidious reduction of external attachments and interests and by apathy and indifference leading to impoverishment of interpersonal relations, mental deterioration, and adjustment on a lower level of functioning. In general, the condition is less dramatically psychotic than are the hebephrenic, catatonic, and paranoid types of schizophrenia. Also, it contrasts with schizoid personality, in which there is little or no progression of the disorder. But after that, it was omitted in later versions and has since then never returned as a formal diagnosis in any DSM. However, DSM-IV (1994) and DSM-IV-TR (2000) included Simple Schizophrenia in the appendix under the proposed category of simple deteriorative disorder. | A carbuncle is a cluster of boils caused by bacterial infection, most commonly with Staphylococcus aureus or Streptococcus pyogenes. The presence of a carbuncle is a sign that the immune system is active and fighting the infection. The infection is contagious and may spread to other areas of the body, or other people; those living in the same residence may develop carbuncles at the same time. In the early 21st century, infection involving methicillin-resistant Staphylococcus aureus (MRSA) has become more common. Signs and symptoms
A carbuncle is a cluster of several boils, which is typically filled with purulent exudate (dead neutrophils, phagocytized bacteria, and other cellular components). Fluid may drain freely from the carbuncle, or intervention involving an incision and drainage procedure may be needed. Carbuncles may develop anywhere, but they are most common on the back and the nape of the neck.A carbuncle is palpable and can range in size to be as small as a pea or as large as a golf ball. The surrounding area is indurated. Later, skin on the centre of the carbuncle softens and peripheral satellite vesicles appear, which rupture discharging pus and give rise to cribriform appearance. As the impending infection develops, itching may occur. There may be localized erythema, skin irritation, and the area may be painful when touched. Sometimes more severe symptoms may occur, such as fatigue, fever, chills, and general malaise as the body fights the infection. Cause
The initial cause of a carbuncle can often not be determined. | 0-1
|
However, when acne is caused by mechanical obstruction it is not considered a form of acne vulgaris when being very technical. It would be an other acneiform eruption known as Acne mechanica. Several medications can also worsen pre-existing acne; this condition is the acne medicamentosa
form of acne. Examples of such medications include lithium, hydantoin, isoniazid, glucocorticoids, iodides, bromides, and testosterone. When acne medicamentosa is specifically caused by anabolic–androgenic steroids it can simply be referred to as steroid acne. Genetically susceptible individuals can get acne breakouts as a result of polymorphous light eruption; a condition triggered by sunlight and artificial UV light exposure. This form of acne is called Acne aestivalis and its specifically caused by intense UVA light exposure. Affected individuals usually experience seasonal acne breakouts on their upper arms, shoulder girdle, back, and chest. The breakouts typically occur one-to-three days after theyve been exposed to intese UVA radiation. Unlike other forms of acne the condition spares the face; this could possibly be a result of the pathogenesis of polymorphous light eruption, in which areas of the skin that are newly exposed to intense ultraviolet radiation are affected. Since faces are typically left uncovered at all stages of life theres little-to-no likelihood for an eruption to appear there. Studies show that both polymorphous light eruption outbreaks and the acne aestivalis breakout response can be prevented by topical antioxidants combined with the application of a broad spectrum sunscreen. | It is unclear whether eradication of the mite improves acne. Diet
High-glycemic-load diets have been found to have different degrees of effect on acne severity. Multiple randomized controlled trials and nonrandomized studies have found a lower-glycemic-load diet to be effective in reducing acne. There is weak observational evidence suggesting that dairy milk consumption is positively associated with a higher frequency and severity of acne. Milk contains whey protein and hormones such as bovine IGF-1 and precursors of dihydrotestosterone. Studies suggest these components promote the effects of insulin and IGF-1 and thereby increase the production of androgen hormones, sebum, and promote the formation of comedones. Available evidence does not support a link between eating chocolate or salt and acne severity. Few studies have examined the relationship between obesity and acne. Vitamin B12 may trigger skin outbreaks similar to acne (acneiform eruptions), or worsen existing acne when taken in doses exceeding the recommended daily intake. Stress
There are few high-quality studies to demonstrate that stress causes or worsens acne. Despite being controversial, some research indicates that increased acne severity is associated with high stress levels in certain contexts, such as hormonal changes seen in premenstrual syndrome. Other
Some individuals experience severe intensification of their acne when they are exposed to hot humid climates; this is due to bacteria and fungus thriving in warm, moist environments. This climate induced acne exacerbation has been termed tropical acne. Mechanical obstruction of skin follicles with helmets or chinstraps can worsen pre-existing acne. | 11
|
Vogt–Koyanagi–Harada disease (VKH) is a multisystem disease of presumed autoimmune cause that affects melanin-pigmented tissues. The most significant manifestation is bilateral, diffuse uveitis, which affects the eyes. VKH may variably also involve the inner ear, with effects on hearing, the skin, and the meninges of the central nervous system. Signs and symptoms
Overview
The disease is characterised by bilateral diffuse uveitis, with pain, redness and blurring of vision. The eye symptoms may be accompanied by a varying constellation of systemic symptoms, such as auditory (tinnitus, vertigo, and hypoacusis), neurological (meningismus, with malaise, fever, headache, nausea, abdominal pain, stiffness of the neck and back, or a combination of these factors; meningitis, CSF pleocytosis, cranial nerve palsies, hemiparesis, transverse myelitis and ciliary ganglionitis), and cutaneous manifestations, including poliosis, vitiligo, and alopecia. The vitiligo often is found at the sacral region. Phases
The sequence of clinical events in VKH is divided into four phases - prodromal, acute uveitic, convalescent, and chronic recurrent.The prodromal phase may have no symptoms, or may mimic a nonspecific viral infection, marked by flu-like symptoms that typically last for a few days. Fever, headache, nausea, meningismus, dysacusia (discomfort caused by loud noises or a distortion in the quality of the sounds being heard), tinnitus, and/or vertigo may occur. Eye symptoms can include orbital pain, photophobia, and tearing. The skin and hair may be sensitive to touch. Cranial nerve palsies and optic neuritis are uncommon.The acute uveitic phase occurs a few days later and typically lasts for several weeks. | Friedel-Crafts cyclization of the olefin with the equivalent of PPA then gives the thiopyran (3). Acylation with acetyl chloride in the presence of aluminium chloride gives the methyl ketone (4). Reaction of the enolate of that ketone with diethyl chlorophosphate gives the enol phosphate 5 as a transient intermediate. This eliminates diethyl phosphite in the presence of excess base to give the corresponding acetylene 6. The anion from the reaction of the acetylene with base is then used to displace chlorine from Ethyl 6-chloronicotinate (7). This reaction affords the coupling product tazarotene (8). == References == | 0-1
|
The use of electroencephalography (EEG) can exclude temporal status epilepticus from consideration. EEGs are normal in about 70% of KLS patients, but background slowing may sometimes be detected. In addition, low-frequency high-amplitude waves can be observed during waking hours.Initially, KLS appears similar to bipolar depression. Patients with frontal-lobe syndromes and Klüver–Bucy syndrome also display similar symptoms, but these conditions can be differentiated by the presence of brain lesions. KLS should also be distinguished from very rare cases of menstruation-caused hypersomnia. Prevention
Lithium is the only drug that appears to have a preventive effect. In two studies of more than 100 patients, lithium helped prevent recurrence of symptoms in 20% to 40% of cases. The recommended blood level of lithium for KLS patients is 0.8–1.2 mEq/ml. It is not known if other mood stabilizers have an effect on the condition. Anti-depressants do not prevent recurrence. Treatment
Several drug therapies have been used on patients with KLS, but none of them have been subject to randomized controlled trials. A 2016 Cochrane Review concluded that "No evidence indicates that pharmacological treatment for Kleine–Levin syndrome is effective and safe".In several cases, stimulants, including modafinil, have been reported to have a limited effect on patients, often alleviating sleepiness. They can cause behavioral problems, but they may pose fewer issues if used in older patients with mild symptoms. In some case reports, lithium has been reported to decrease the length of episodes and the severity of their symptoms and to increase the time between episodes. | Kleine–Levin syndrome (KLS) is a rare disorder characterized by persistent episodic hypersomnia and cognitive or mood changes. Many patients also experience hyperphagia, hypersexuality and other symptoms. Patients generally experience recurrent episodes of the condition for more than a decade and may return at a later age. Individual episodes generally last more than a week, sometimes lasting for months. The condition greatly affects the personal, professional, and social lives of those with KLS. The severity of symptoms and the course of the syndrome vary between those with KLS. Patients commonly have about 20 episodes over about a decade. Several months generally elapse between episodes. The onset of the condition usually follows a viral infection; several different viruses have been observed to trigger KLS. It is generally only diagnosed after similar conditions have been excluded; MRI, CT scans, lumbar puncture, and toxicology tests are used to rule out other possibilities. The syndromes mechanism is not known, but the thalamus is thought to possibly play a role. SPECT has shown thalamic hypoperfusion of patients during episodes. KLS is very rare, occurring at a rate of 1 in 1 million, which limits research into genetic factors. The condition primarily affects adolescent males, though females can also be affected and the age of onset varies. There is no known cure, and there is little evidence supporting drug treatment. Lithium has been reported to have limited effects in case reports, decreasing the length of episodes and duration between them in some patients. | 11
|
The build up of fluid in the lungs increases the pressure in the lungs leading to vasoconstriction. The coupled vasoconstriction and pulmonary hypertension causes the right side of the heart to fail which in turn, increases the venous hydrostatic pressure in the body. The summation of these effects ultimately leads to peripheral edema and ascites. All in all, the left side failure of the heart will lead to pulmonary edema whereas right side failure will lead to peripheral edema and ascites. The non-immune form of hydrops fetalis has many causes including:
Iron deficiency anemia
Paroxysmal supraventricular tachycardia resulting in heart failure
Deficiency of the enzyme beta-glucuronidase. This enzyme deficiency is the cause of the lysosomal storage disease called mucopolysaccharidosis type VII. Congenital disorders of glycosylation
Parvovirus B19 (fifth disease) infection of the pregnant woman (most common cause)
Cytomegalovirus in mother
Congenital pulmonary airway malformation (formerly called congenital cystic adenomatoid malformation)
Maternal syphilis and maternal diabetes mellitus
Alpha-thalassemia can also cause hydrops fetalis when all four of the genetic loci for α globin are deleted or affected by mutation. This is termed Hb Barts (consists of y-4 tetramers). Uncommonly, Niemann-Pick disease Type C (NPC) and Gaucher disease type 2 can present with hydrops fetalis. Turner syndrome
Tumors, the most common type of fetal tumor being teratoma, particularly a sacrococcygeal teratoma. | Fetal hydrothorax, chylothorax, or large pleural effusion associated with bronchopulmonary sequestration should be treated using a Fetal needle drainage of effusion or placement of thoracoamniotic shunt. This procedure can be performed prior to delivery if gestational age is advanced. Hydrops Fetalis resulting from fetal CPAM can be treated using either a fetal needle drainage of effusion or placement of thoracoamniotic shunt or a maternal administration of corticosteroids, betamethasone 12.5 mg IM q24 h × 2 doses or dexamethasone 6.25 mg IM q12 h × 4 doses. Therapy for hydrops fetalis derived from TTTS or TAPS requires laser ablation of placental anastomoses or selective termination. Therapy for hydrops fetalis derived from TRAPS requires percutaneous radio frequency ablation. See also
Mirror syndrome
== References == | 11
|
Cause
Selective IgA deficiency is inherited and has been associated with differences in chromosomes 18, 14 and 6. Selective IgA deficiency is often inherited, but has been associated with some congenital intrauterine infections. Pathophysiology
Pathogenesis of IgA Deficiency
‘In IgA-deficient patients, the common finding is a maturation defect in B cells to produce IgA’. ‘In IgA deficiency, B cells express IgA; however, they are of immature phenotype with the coexpression of IgM and IgD, and they cannot fully develop into IgA-secreting plasma cells’. There is an inherited inability to produce immunoglobulin A (IgA), a part of the bodys defenses against infection at the bodys surfaces (mainly the surfaces of the respiratory and digestive systems). As a result, bacteria at these locations are somewhat more able to cause disease.Types include:
Diagnosis
When suspected, the diagnosis can be confirmed by laboratory measurement of IgA level in the blood. SIgAD is an IgA level < 7 mg/dL with normal IgG and IgM levels (reference range 70–400 mg/dL for adults; children somewhat less). Treatment
The treatment consists of identification of co-morbid conditions, preventive measures to reduce the risk of infection, and prompt and effective treatment of infections. Infections in an IgA-deficient person are treated as usual (i.e., with antibiotics). There is no treatment for the underlying disorder. Use of IVIG as treatment
There is a historical popularity in using intravenous immunoglobulin (IVIG) to treat SIgAD, but the consensus is that there is no evidence that IVIG treats this condition. | An accident is an unintended, normally unwanted event that was not directly caused by humans. The term accident implies that nobody should be blamed, but the event may have been caused by unrecognized or unaddressed risks. Most researchers who study unintentional injury avoid using the term accident and focus on factors that increase risk of severe injury and that reduce injury incidence and severity. For example, when a tree falls down during a wind storm, its fall may not have been caused by humans, but the trees type, size, health, location, or improper maintenance may have contributed to the result. Most car wrecks are not true accidents; however English speakers started using that word in the mid-20th century as a result of media manipulation by the US automobile industry. Types
Physical and non-physical
Physical examples of accidents include unintended motor vehicle collisions, falls, being injured by touching something sharp or hot, or bumping into something while walking. Non-physical examples are unintentionally revealing a secret or otherwise saying something incorrectly, accidental deletion of data, or forgetting an appointment. Accidents by activity
Accidents during the execution of work or arising out of it are called work accidents. According to the International Labour Organization (ILO), more than 337 million accidents happen on the job each year, resulting, together with occupational diseases, in more than 2.3 million deaths annually. In contrast, leisure-related accidents are mainly sports injuries. Accidents by vehicle
Vehicle collisions are not usually accidents; they are mostly caused by preventable causes such as drunk driving and intentionally driving too fast. | 0-1
|
Symptoms management
The following are some of the most common potential problems that can arise in the last days and hours of a patients life:
Pain
Typically controlled with opioids, like morphine, fentanyl, hydromorphone or, in the United Kingdom, diamorphine. High doses of opioids can cause respiratory depression, and this risk increases with concomitant use of alcohol and other sedatives. Careful use of opioids is important to improve the patients quality of life while avoiding overdoses. Agitation
Delirium, terminal anguish, restlessness (e.g. thrashing, plucking, or twitching). Typically controlled using clonazepam or midazolam,antipsychotics such as haloperidol or levomepromazine may also be used instead of, or concomitantly with benzodiazepines. Symptoms may also sometimes be alleviated by rehydration, which may reduce the effects of some toxic drug metabolites. Respiratory tract secretions
Saliva and other fluids can accumulate in the oropharynx and upper airways when patients become too weak to clear their throats, leading to a characteristic gurgling or rattle-like sound ("death rattle"). While apparently not painful for the patient, the association of this symptom with impending death can create fear and uncertainty for those at the bedside. The secretions may be controlled using drugs such as hyoscine butylbromide, glycopyrronium, or atropine. Rattle may not be controllable if caused by deeper fluid accumulation in the bronchi or the lungs, such as occurs with pneumonia or some tumours. Nausea and vomiting
Typically controlled using haloperidol, metoclopramide, ondansetron, cyclizine; or other anti-emetics. | Non-medical
Family and friends
Family members are often uncertain as to what they should be doing when a person is dying. Many gentle, familiar daily tasks, such as combing hair, putting lotion on delicate skin, and holding hands, are comforting and provide a meaningful method of communicating love to a dying person.Family members may be suffering emotionally due to the impending death. Their own fear of death may affect their behavior. They may feel guilty about past events in their relationship with the dying person or feel that they have been neglectful. These common emotions can result in tension, fights between family members over decisions, worsened care, and sometimes (in what medical professionals call the "Daughter from California syndrome") a long-absent family member arrives while a patient is dying to demand inappropriately aggressive care. Family members may also be coping with unrelated problems, such as physical or mental illness, emotional and relationship issues, or legal difficulties. These problems can limit their ability to be involved, civil, helpful, or present. Spirituality and Religion
Spirituality is thought to be of increased importance to an individuals wellbeing during a terminal illness or toward the end-of-life. Pastoral/spiritual care has a particular significance in end of life care, and is considered an essential part of palliative care by the WHO. In palliative care, responsibility for spiritual care is shared by the whole team, with leadership given by specialist practitioners such as pastoral care workers. | 11
|
Amastia refers to a rare clinical anomaly in which both internal breast tissue and the visible nipple are absent on one or both sides. It affects both men and women. Amastia can be either isolated (the only medical condition) or comorbid with other syndromes, such as ectodermal dysplasia, syndactaly (Polands syndrome) and lipoatrophic diabetes. This abnormality can be classified into various types, and each could result from different pathologies. Amastia differs from amazia and athelia. Amazia is the absence of one or both mammary glands but the nipples remain present, and athelia is the absence of one or both nipples, but the mammary gland remains.Amastia is presumably due to failure of embryologic (before birth) mammary ridge development or incomplete involution. People with amastia often suffer from ectodermal defects, which include various syndromes such as cleft palate, isolated pectoral muscle and abnormal formation of the arms.Treatment for female amastia particularly includes psychological guidance and breast reconstruction. Because there is no breast tissue, breastfeeding is not possible. If amastia only appears on one side, then it is possible to breastfeed on the other side. Often, people with amastia decide against treatment. Classification
Amastia can be either iatrogenic or congenital. The congenital amastia are further divided into syndromic type and non-syndromic type respectively. As the definition suggests, syndromic amastia is often associated with obvious symptoms. The common case is hypoplasia of ectodermal tissue, such as hair and skin defects. On the other hand, non-syndromic amastia, shows no defects in body parts other than breast. | Homozygous frameshift mutation in PTPRF may cause amastia, which suggests the causative relationship between PTPRF defect and syndromic amastia. Management
Since bilateral and unilateral amastia may be attributed to different pathologies, appropriate managements should be adopted accordingly. Bilateral amastia can occur in isolation or associated with other disorders. This case is less understood and difficult to treat. On the other hand, Polands syndrome is the most common cause of unilateral amastia. Managements such as muscle/breast reconstruction and nipple areola relocation should be provided to these patients. Breast reconstruction
Surgical treatment for breast defects such as mastectomy is also applicable to treat patients with amastia. Tissue expansion is the most common technique and can be done by using either autologous or prosthetic tissue. For autologous reconstruction, different tissues may be chosen according to patients’ physical condition or their preferences. Prosthetic reconstruction may follow the same principles. Flap reconstruction is another method to rebuild the breast surgically. There are various kinds of flaps to choose depending on different situation. Nipple areola relocation
Amastia is often associated with Polands syndrome, which requires appropriate reconstructive procedure to stabilize chest wall, transfer dynamic muscle and reposition nipple areola region. The treatment of nipple areola relocation provides space for secondary breast enlargement. In this treatment, the tissue expander can be inserted either beforehand or delayed. It can be placed in different parts of body depending on how many overlying soft tissues the patient has. | 11
|
Pramlintide (trade name Symlin) is an injectable amylin analogue drug for diabetes (both type 1 and 2), developed by Amylin Pharmaceuticals (now a wholly owned subsidiary of AstraZeneca). Pramlintide is sold as an acetate salt. Pharmacology
Pramlintide is an analogue of amylin, a small peptide hormone that is released into the bloodstream by the β cells of the pancreas along with insulin after a meal. Like insulin, amylin is completely absent in individuals with Type I diabetes.In synergy with endogenous amylin, pramlintide aids in the regulation of blood glucose by slowing gastric emptying, promoting satiety via hypothalamic receptors (different receptors than for GLP-1), and inhibiting inappropriate secretion of glucagon, a catabolic hormone that opposes the effects of insulin and amylin. Pramlintide also has effects in raising the acute first-phase insulin response threshold following a meal. Both a reduction in glycated hemoglobin and weight loss have been shown in insulin-treated patients with type 2 diabetes taking pramlintide as an adjunctive therapy. Research Applications
In the research field, pramlintide has been experimented with and used as a potential treatment drug. Pramlintide has demonstrated its ability to decrease amyloid beta plaques in Alzheimers disease mouse models. Approval
Pramlintide has been approved by the FDA, for use by type 1 and type 2 diabetic patients who use insulin. Pramlintide allows patients to use less insulin, lowers average blood sugar levels, and substantially reduces what otherwise would be a large unhealthy rise in blood sugar that occurs in diabetics right after eating. | Pashayan syndrome, also known as Pashayan–Pruzansky Syndrome and blepharo-naso-facial syndrome, is a rare syndrome with Mendelian autosomal dominant inheritance with variable expression. An article describing a family with this syndrome was first published in 1973 in The American Journal of Diseases of Children by Drs Hermine Pashayan, Samuel Pruzansky and Allen Putterman from Abraham Lincoln School of Medicine, University of Illinois in Chicago. Facial abnormalities characterise this syndrome as well as malformation of extremities. Specific characteristics would be a bulky, flattened nose, where the face has a mask like appearance and the ears are also malformed.A subset of Pashayan syndrome has also been described, known as "cerebrofacioarticular syndrome", "Van Maldergem syndrome" or "Van Maldergem–Wetzburger–Verloes syndrome". Similar symptoms are noted in these cases as in Pashayan syndrome. References
== External links == | 0-1
|
Oxytocin was added to the Institute for Safe Medication Practicess list of High Alert Medications in Acute Care Settings in 2012. The list includes medications that have a high risk for harm if administered incorrectly.During pregnancy, increased uterine motility has led to decreased heart rate, cardiac arrhythmia, seizures, brain damage, and death in the fetus or neonate.Use is linked to an increased risk of postpartum depression in the mother.Certain learning and memory functions are impaired by centrally administered oxytocin. Also, systemic oxytocin administration can impair memory retrieval in certain aversive memory tasks. However, oxytocin does seem to facilitate learning and memory specifically for social information. Healthy males administered intranasal oxytocin show improved memory for human faces, in particular happy faces. Pharmacokinetics
Routes of administration
One IU of oxytocin is the equivalent of about 2 μg or mcg of pure peptide. Injection: Clinical doses of oxytocin are given by injection either into a muscle or into a vein to cause contraction of the uterus. Very small amounts (< 1%) do appear to enter the central nervous system in humans when peripherally administered. The compound has a half-life of typically about 3 minutes in the blood when given intravenously. Intravenous administration requires 40 minutes to reach a steady-state concentration and achieve maximum uterine contraction response. Buccal: Oxytocin was delivered in buccal tablets, but this is not common practice any more. Under the tongue: Oxytocin is poorly absorbed sublingually. | The lack of family history does not rule out CMT, but is helpful to rule out other causes of neuropathy, such as diabetes or exposure to certain chemicals or drugs.In 2010, CMT was one of the first diseases where the genetic cause of a particular patients disease was precisely determined by sequencing the whole genome of an affected individual. This was done by the scientists employed by the Charcot Marie Tooth Association (CMTA). Two mutations were identified in a gene, SH3TC2, known to cause CMT. Researchers then compared the affected patients genome to the genomes of the patients mother, father, and seven siblings with and without the disease. The mother and father each had one normal and one mutant copy of this gene, and had mild or no symptoms. The offspring who inherited two mutant genes presented fully with the disease. Histology
The constant cycle of demyelination and remyelination, which occurs in CMT, can lead to the formation of layers of myelin around some nerves, termed an "onion bulb". These are also seen in chronic inflammatory demyelinating polyneuropathy. Muscles show fiber type grouping, a similarly nonspecific finding that indicates a cycle of denervation/reinnervation. Normally, type I and type II muscle fibers show a checkerboard-like random distribution. However, when reinnervation occurs, the group of fibers associated with one nerve are of the same type. The standard for indicating fiber type is histoenzymatic adenosine triphosphatase (ATPase at pH 9.4). Management
Often, the most important goal for patients with CMT is to maintain movement, muscle strength, and flexibility. | 0-1
|
The symptoms included orthostatic headaches and other features that are now recognized as spontaneous intracranial hypotension. A few decades earlier, the same syndrome had been described in French literature as "hypotension of spinal fluid" and "ventricular collapse". In 1940, Henry Woltman of the Mayo Clinic wrote about "headaches associated with decreased intracranial pressure". The full clinical manifestations of intracranial hypotension and CSF leaks were described in several publications reported between the 1960s and early 1990s. Modern reports of spontaneous CSF leak have been reported to medical journals since the late 1980s. Research
Tetracosactide is a corticosteroid that causes the brain to produce additional spinal fluid to replace the volume of the lost CSF and alleviate symptoms, and has been given intravenously to treat CSF leaks.In three small studies of 1-2 patients with recurrent CSF leaks where repeated blood patches failed to form clots and relieve symptoms, the patients received temporary but complete resolution of symptoms with an epidural saline infusion. The saline infusion temporarily restores the volume necessary for a patient to avoid SIH until the leak can be repaired properly. Intrathecal saline infusion is used in urgent cases such as intractable pain or decreased consciousness.The gene TGFBR2 has been implicated in several connective tissue disorders including Marfan syndrome, arterial tortuosity, and thoracic aortic aneurysm. A study of patients with SCSFLS demonstrated no mutations in this gene. Minor features of Marfan syndrome have been found in 20% of CSF leak patients. | Complications
Several complications can occur as a result of SCSFLS including decreased cranial pressure, brain herniation, infection, blood pressure problems, transient paralysis, and coma. The primary and most serious complication of SCSFLS is spontaneous intracranial hypotension, where pressure in the brain is severely decreased. This complication leads to the hallmark symptom of severe orthostatic headaches.People with cranial CSF leaks, the rarer form, have a 10% risk of developing meningitis per year. If cranial leaks last more than seven days, the chances of developing meningitis are significantly higher. Spinal CSF leaks cannot result in meningitis due to the sterile conditions of the leak site. When a CSF leak occurs at the temporal bone, surgery becomes necessary in order to prevent infection and repair the leak. Orthostatic hypotension is another complication that occurs due to autonomic dysfunction when blood pressure drops significantly. The autonomic dysfunction is caused by compression of the brainstem, which controls breathing and circulation.Low CSF volume can cause the cerebellar tonsil position to descend, which can be mistaken for Chiari malformation; however when the CSF leak is repaired the tonsil position often returns to normal (as seen in upright MRI) in this "pseudo-Chiari" condition. A further, albeit rare, complication of CSF leak is transient quadriplegia due to a sudden and significant loss of CSF. This loss results in hindbrain herniation and causes major compression of the upper cervical spinal cord. The quadriplegia dissipates once the patient lies supine. | 11
|
Facial trauma, also called maxillofacial trauma, is any physical trauma to the face. Facial trauma can involve soft tissue injuries such as burns, lacerations and bruises, or fractures of the facial bones such as nasal fractures and fractures of the jaw, as well as trauma such as eye injuries. Symptoms are specific to the type of injury; for example, fractures may involve pain, swelling, loss of function, or changes in the shape of facial structures. Facial injuries have the potential to cause disfigurement and loss of function; for example, blindness or difficulty moving the jaw can result. Although it is seldom life-threatening, facial trauma can also be deadly, because it can cause severe bleeding or interference with the airway; thus a primary concern in treatment is ensuring that the airway is open and not threatened so that the patient can breathe. Depending on the type of facial injury, treatment may include bandaging and suturing of open wounds, administration of ice, antibiotics and pain killers, moving bones back into place, and surgery. When fractures are suspected, radiography is used for diagnosis. Treatment may also be necessary for other injuries such as traumatic brain injury, which commonly accompany severe facial trauma. In developed countries, the leading cause of facial trauma used to be motor vehicle accidents, but this mechanism has been replaced by interpersonal violence; however auto accidents still predominate as the cause in developing countries and are still a major cause elsewhere. | While seat belts reduce the number and severity of facial injuries that occur in crashes, airbags alone are not very effective at preventing the injuries. In sports, safety devices including helmets have been found to reduce the risk of severe facial injury. Additional attachments such as face guards may be added to sports helmets to prevent orofacial injury (injury to the mouth or face); mouth guards also used. In addition to factors listed above, correction of dental features that are associated with receiving more dental trauma also helps, such as increased overjet, Class II malocclusions, or correction of detofacal deformities with small mandible
Treatment
An immediate need in treatment is to ensure that the airway is open and not threatened (for example by tissues or foreign objects), because airway compromisation can occur rapidly and insidiously, and is potentially deadly. Material in the mouth that threatens the airway can be removed manually or using a suction tool for that purpose, and supplemental oxygen can be provided. Facial fractures that threaten to interfere with the airway can be reduced by moving the bones back into place; this both reduces bleeding and moves the bone out of the way of the airway. Tracheal intubation (inserting a tube into the airway to assist breathing) may be difficult or impossible due to swelling. | 11
|
Additionally, chronic increase in production of AII is associated with structural changes to the myocardium which reduces the functionality of the heart.In heart failure patients, fosinopril increases exercise tolerance and lowers the frequency of events associated with worsening heart failure, such as dyspnea, the need for supplemental diuretics, fatigue, and hospitalizations. Chemistry
Unlike other ACE inhibitors that are primarily excreted by the kidneys, fosinopril is eliminated from the body by both renal and hepatic pathways. This characteristic of fosinopril makes the drug a safer choice than other ACE inhibitors for heart failure patients with impaired kidney function resulting from poor perfusion as fosinopril can still be eliminated by the liver, preventing accumulation of the drug in the body.Fosinopril is de-esterified by the liver or gastrointestinal mucosa and is converted to its active form, fosinoprilat. Fosinoprilat competitively binds to ACE, preventing ACE from binding to and converting angiotensin I to angiotensin II. Inhibiting the production of AII lowers peripheral vascular resistance, decreases afterload, and decreases blood pressure, thus helping to alleviate the negative effects of AII on cardiac performance. == References == | A gumma (plural gummata or gummas) is a soft, non-cancerous growth resulting from the tertiary stage of syphilis (and yaws). It is a form of granuloma. Gummas are most commonly found in the liver (gumma hepatis), but can also be found in brain, heart, skin, bone, testis, and other tissues, leading to a variety of potential problems including neurological disorders or heart valve disease. Presentation
Gummas have a firm, necrotic center surrounded by inflamed tissue, which forms an amorphous proteinaceous mass. The center may become partly hyalinized. These central regions begin to die through coagulative necrosis, though they also retain some of the structural characteristics of previously normal tissues, enabling a distinction from the granulomas of tuberculosis where caseous necrosis obliterates preexisting structures. Other histological features of gummas include an intervening zone containing epithelioid cells with indistinct borders and multinucleated giant cells, and a peripheral zone of fibroblasts and capillaries. Infiltration of lymphocytes and plasma cells can be seen in the peripheral zone as well. With time, gummas eventually undergo fibrous degeneration, leaving behind an irregular scar or a round fibrous nodule.It is restricted to necrosis involving spirochaetal infections that cause syphilis. Growths that have the appearance of gummas are described as gummatous. Pathology
In syphilis, the gumma is caused by a reaction to spirochaete bacteria in the tissue. It appears to be the human bodys way to slow down the action of this bacteria; it is a unique immune response that develops in humans after the immune system fails to kill off syphilis. | 0-1
|
It suppresses the bone marrow by inhibiting the erythroid progenitor cells.Anti-M also recommends antigen testing to rule out the presence of HDN as the direct coombs can come back negative in a severely affected infant.Kidd antigens are also present on the endothelial cells of the kidneys.One study states that it would be unwise to routinely dismiss anti-E as being of little clinical consequence. It also found that the most severe case of anti-E HDFN occurred with titers 1:2, concluding that titers are not reliable for the diagnosis of the anti-E type. Diagnosis
The diagnosis of HDN is based on history and laboratory findings:Blood tests done on the newborn baby
Biochemistry tests for jaundice including total and direct bilirubin levels. Complete blood count (CBC) which may show a decreased hemoglobin and hematocrit due to red blood cell destruction
Reticulocyte count which will usually be increased as the bone marrow makes new red blood cells to replace the ones that are being destroyed, and a peripheral blood smear to look at cell morphology. In the presence of significant hemolysis the smear will show schistocytes (fragmented red blood cells), reticulocytosis, and in severe cases Erythroblasts (also known as nucleated red blood cells). Positive direct Coombs test (might be negative after fetal interuterine blood transfusion)Blood tests done on the mother
Positive indirect Coombs testBlood tests done on the father (rarely needed)
Erythrocyte antigen status
Prevention
In cases of Rho(D) incompatibility, Rho(D) immunoglobulin is given to prevent sensitization. However, there is no comparable immunotherapy available for other blood group incompatibilities.Early pregnancy
IVIG – IVIG stands for Intravenous Immunoglobulin. | There is some evidence that suggests that night terrors can occur if the individual does not eat a proper diet, does not get the appropriate amount or quality of sleep (e.g., because of sleep apnea), or is enduring stressful events. Adults who have experienced sexual abuse are more likely to receive a diagnosis of sleep disorders, including night terrors. Overall, though, adult night terrors are much less common and often respond best to treatments that rectify causes of poor quality or quantity of sleep. Diagnosis
The DSM-5 diagnostic criteria for sleep terror disorder requires:
Recurrent periods where the individual abruptly but not completely wakes from sleep, usually occurring during the first third major period of sleep. The individual experiences intense fear with a panicky scream at the beginning and symptoms of autonomic arousal, such as increased heart rate, heavy breathing, and increased perspiration. The individual cannot be soothed or comforted during the episode. The individual is unable or almost unable to remember images of the dream (only a single visual scene for example). The episode is completely forgotten. The occurrence of the sleep terror episode causes clinically significant distress or impairment in the individuals functioning. The disturbance is not due to the effects of a substance, general medical condition or medication. Coexisting mental or medical disorders do not explain the episodes of sleep terrors. Differential diagnosis
Night terrors are distinct from nightmares. In fact, in nightmares there are almost never vocalization or agitation, and if there are any, they are less strong in comparison to night terrors. | 0-1
|
A review showed substantial evidence for atypical processing of auditory information in children with autism. Dawes and Bishop noted how specialists in audiology and speech-language pathology often adopted different approaches to child assessment, and they concluded their review as follows: "We regard it as crucial that these different professional groups work together in carrying out assessment, treatment and management of children and undertaking cross-disciplinary research." In practice, this seems rare.To ensure that APD is correctly diagnosed, the examiners must differentiate APD from other disorders with similar symptoms. Factors that should be taken into account during the diagnosis are: attention, auditory neuropathy, fatigue, hearing and sensitivity, intellectual and developmental age, medications, motivation, motor skills, native language and language experience, response strategies and decision-making style, and visual acuity.It should also be noted that children under the age of seven cannot be evaluated correctly because their language and auditory processes are still developing. In addition, the presence of APD cannot be evaluated when a childs primary language is not English. | Dissociative symptoms include a sense of numbing or detachment from emotional reactions, a sense of physical detachment – such as seeing oneself from another perspective – decreased awareness of ones surroundings, the perception that ones environment is unreal or dreamlike, and the inability to recall critical aspects of the traumatic event (dissociative amnesia).In addition to these characteristics, ASD can be present in the following symptom clusters: intrusion, negative mood, disassociation, avoidance of distressing memories and emotional arousal. Intrusion symptoms include recurring and distressing dreams, flashbacks, or memories related to the traumatic event and related somatic symptoms. Emotional arousal symptoms include sleep disturbances, hypervigilance, difficulties with concentration, more common startle response, and irritability. Complications
There are a number of issues that can arise from acute stress. Depression, anxiety, mood disorders, and substance abuse problems can develop from acute stress. Untreated ASD can also lead to the development of post-traumatic stress disorder. Causes
There are several theoretical perspectives on trauma response, including cognitive, biological, and psycho-biological. While PTSD-specific, these theories are still useful in understanding acute stress disorder, as the two disorders share many symptoms. A recent study found that even a single stressful event may have long-term consequences on cognitive function. This result calls the traditional distinction between the effects of acute and chronic stress into question. Pathophysiology
Stress is characterised by specific physiological responses to adverse or noxious stimuli. | 0-1
|
Even levobupivacaine and ropivacaine (single-enantiomer derivatives), developed to ameliorate cardiovascular side effects, still harbor the potential to disrupt cardiac function. Toxicity from anesthetic combinations is additive. Endocrine
Endocrine and metabolic systems only have slightly adverse effects with most cases being without clinical repercussions. Immunologic allergy
Adverse reactions to local anesthetics (especially the esters) are not uncommon, but legitimate allergies are very rare. Allergic reactions to the esters is usually due to a sensitivity to their metabolite, para-aminobenzoic acid, and does not result in cross-allergy to amides. Therefore, amides can be used as alternatives in those patients. Nonallergic reactions may resemble allergy in their manifestations. In some cases, skin tests and provocative challenge may be necessary to establish a diagnosis of allergy. Also cases of allergy to paraben derivatives occur, which are often added as preservatives to local anesthetic solutions. Methemoglobinemia
Methemoglobinemia is a process where iron in hemoglobin is altered, reducing its oxygen-carrying capability, which produces cyanosis and symptoms of hypoxia. Exposure to aniline group chemicals such as benzocaine, lidocaine, and prilocaine can produce this effect, especially benzocaine. The systemic toxicity of prilocaine is comparatively low, but its metabolite, o-toluidine, is known to cause methemoglobinemia. Second-generation effects
Application of local anesthetics during oocyte removal during in vitro fertilisation has been up to debate. Pharmacological concentrations of anesthetic agents have been found in follicular fluid. Clinical trials have not concluded any effects on pregnant women. | Long-term therapy may lead to cognitive deficits, especially in the elderly, which may only be partially reversible. The elderly metabolize benzodiazepines more slowly than younger people and are more sensitive to the adverse effects of benzodiazepines compared to younger individuals even at similar plasma levels. Additionally, the elderly tend to take more drugs which may interact or enhance the effects of benzodiazepines. Benzodiazepines, including lorazepam, have been found to increase the risk of falls and fractures in the elderly. As a result, dosage recommendations for the elderly are about half of those used in younger individuals and used for no longer than two weeks. Lorazepam may also be slower to clear in the elderly, leading potentially to accumulation and enhanced effects. Lorazepam, similar to other benzodiazepines and nonbenzodiazepines, causes impairments in body balance and standing steadiness in individuals who wake up at night or the next morning. Falls and hip fractures are frequently reported. The combination with alcohol increases these impairments. Partial, but incomplete, tolerance develops to these impairments. Liver or kidney failure – Lorazepam may be safer than most benzodiazepines in people with impaired liver function. Like oxazepam, it does not require liver oxidation, but only liver glucuronidation into lorazepam-glucuronide. Therefore, impaired liver function is unlikely to result in lorazepam accumulation to an extent causing adverse reactions. Similarly kidney disease has minimal effects on lorazepam levels. Surgical premedication – Informed consent given only after receiving lorazepam premedication could have its validity challenged later. Staff must use chaperones to guard against allegations of abuse during treatment. | 0-1
|
This procedure is a way to destroy the area of the heart tissue that is causing the irregular contractions characteristic of PVCs using radio frequency energy. Implantable cardioverter-defibrillator
Lifestyle modification
Frequently stressed individuals should consider therapy, or joining a support group. Prognosis
PVCs are harmless, but frequent PVCs may increase the risk of developing cardiomyopathy, which can greatly impair heart function. On a more serious and severe scale, very frequent PVCs can accompany underlying heart disease.People who do not have heart disease (with ejection fractions greater than 40%) have the same long-term prognoses as the minorance of people without PVCs on the 24 hours. Emerging data also suggest that very frequent ventricular ectopy may be associated with cardiomyopathy through a mechanism thought to be similar to that of chronic right ventricular pacing associated cardiomyopathy. For patients with underlying chronic structural heart disease and complex ectopy, mortality is significantly increased.In meta-analysis of 11 studies, people with frequent PVCs (≥ once during a standard electrocardiographic recording or ≥30 times over a 1-hour recording) had risk of cardiac death twice as great as that of participants with occasional PVCs. Although most researchers attempted to exclude high-risk subjects, such as those with histories of cardiovascular disease, they did not test participants for underlying structural heart disease.In a study of 239 people with frequent PVCs (>1000 beats/day) and without structural heart disease (i.e. | A degloving injury is a type of avulsion in which an extensive section of skin is completely torn off the underlying tissue, severing its blood supply. It is named by analogy to the process of removing a glove. Effects
Typically, degloving injuries affect the extremities and limbs; in these cases, they are frequently associated with underlying fractures. Any injury which would induce degloving of the head or torso is likely to be lethal. However, controlled facial degloving is often featured in plastic surgery. Degloving injuries invariably require major surgical interventions. Treatment options include replantation or revascularization of the degloved skins, or when these are not possible, skin grafts or skin flaps. While the preservation of the extremities and limbs is normally preferred, in some cases amputations may be advised or required. Post-operative physiotherapy is of particular importance for degloving injuries involving the hand. Other animals
Many small mammals are able to induce degloving of their tails to escape capture; this is comparable to tail autotomy in reptiles. References
External links
Report on degloving injuries in children | 0-1
|
The procedure can be performed as an outpatient surgery using local anesthesia, and leaves substantially less scarring than open surgical procedures and no breast tissue deformation.The American Society of Breast Surgeons recommends the following criteria to establish a patient as a candidate for cryoablation of a fibroadenoma:
The lesion must be sonographically visible. The diagnosis of a fibroadenoma must be confirmed histologically. The lesion should be less than 4 cm in diameter. High Intensity Focused Ultrasound
High Intensity Focused Ultrasound (HIFU) is a newer technique for the treatment of malignant and benign tumors of the breast and has shown promising results in the form of complete radiological removal of tumors. An ultrasound beam is focused on a target in the breast and leads to tissue death and protein degradation by raising the temperature in that area. Currently, the use of radiation is recommended in some cases, but HIFU in particular is not part of treatment guidelines. Further research into the usefulness of HIFU, specifically in fibroadenoma, is required before more widespread use of the technique in fibroadenoma. Epidemiology
Of all breast tissue samples taken, fibroadenomas comprise about 50%, and this rate rises to 75% for tissue sample in women under the age of 20 years. Fibroadenomas are more frequent among women in higher socioeconomic classes and darker-skinned people. Body mass index and the number of full-term pregnancies were found to have a negative correlation with the risk of fibroadenomas. There are no known genetic factors that influence the rate of fibroadenomas. | A small amount of normal tissue must be removed in case the lesion turns out to be a phyllodes tumour on microscopic examination.Because needle biopsy is often a reliable diagnostic investigation, some doctors may decide not to operate to remove the lesion, and instead opt for clinical follow-up to observe the lesion over time using clinical examination and mammography to determine the rate of growth, if any, of the lesion. A growth rate of less than sixteen percent per month in women under fifty years of age, and a growth rate of less than thirteen percent per month in women over fifty years of age have been published as safe growth rates for continued non-operative treatment and clinical observation.Some fibroadenomas respond to treatment with ormeloxifene.Fibroadenomas have not been shown to recur following complete excision or transform into phyllodes tumours following partial or incomplete excision. Non-invasive Surgical Interventions
There are several non-invasive options for the treatment of fibroadenomas, including percutaneous radiofrequency ablation (RFA), cryoablation, and percutaneous microwave ablation. With the use of advanced medical imaging, these procedures do not require invasive surgery and have the potential for enhanced cosmetic results compared with conventional surgery. Cryoablation
The FDA approved cryoablation of a fibroadenoma as a safe, effective, and minimally-invasive alternative to open surgical removal in 2001. During cryoablation, ultrasound imaging is used to guide a probe into the mass of breast tissue. Extremely cold temperatures are then used to destroy the abnormal cells, and over time the cells are reabsorbed into the body. | 11
|
In descending perineum syndrome the anal canal is situated several cm below this imaginary line, or it descends 3–4 cm during straining. Defecography may also demonstrate abnormal perineal descent. Treatment
Surgical treatments may be used to treat the condition, and include retro-rectal levatorplasty, post-anal repair, retro-anal levator plate myorrhaphy. Epidemiology
The condition mainly occurs in women, and it is thought by some to be one of the main defects encountered problem in perineology. == References == | The glucuronidation of buprenorphine is primarily carried out by the UDP-glucuronosyltransferases (UGTs) UGT1A1 and UGT2B7, while norbuprenorphine is glucuronidated by UGT1A1 and UGT1A3. These glucuronides are then eliminated mainly through excretion into bile. The elimination half-life of buprenorphine is 20 to 73 hours (mean 37 hours). Due to the mainly hepatic elimination, there is no risk of accumulation in people with kidney problems. Naloxone
Naloxone is extensively inactivated by first-pass metabolism in the liver, meaning that use of buprenorphine/naloxone as prescribed should not lead to active naloxone in the blood (which, as an opioid antagonist, would reverse the effect of buprenorphine or other opioids). Society and culture
Cost
While the cost of the medication buprenorphine/naloxone is greater than buprenorphine alone, one analysis predicted the overall costs would be less in the United States due to a lower risk of misuse. Access in the United States
Before the Drug Addiction Treatment Act of 2000 (DATA), physicians were not allowed to prescribe narcotics to treat opioid dependence. People with narcotic dependence had to go to registered clinics to receive treatment. With DATA, Suboxone was the first medication approved for office-based treatment for opioid dependence. Suboxone has thus become widely used as a replacement for methadone as it can be prescribed by doctors in their offices, while methadone can only be provided at specialized addiction centers, of which there are a limited number, often making access difficult. Integrating Medication-Assisted Treatment into outpatient primary care practices improves patient access to Suboxone. | 0-1
|
Treatment
Depending on the severity of the disease, these steps can be taken, according to these guidelines:
Lifestyle
Stopping smoking (cigarettes promote PAD and are a risk factor for cardiovascular disease)
Regular exercise for those with claudication helps open up alternative small vessels (collateral flow) and the limitation in walking often improves. Treadmill exercise (35 to 50 minutes, three or four times per week) has been reviewed as another treatment with a number of positive outcomes, including reduction in cardiovascular events and improved quality of life. Supervised exercise programs increase pain-free walking time and the maximum walking distance in people with PAD. Medication
Management of diabetes
Management of hypertension
Management of high cholesterol, and antiplatelet drugs such as aspirin and clopidogrel. Statins reduce clot formation and cholesterol levels, respectively, can help with disease progression, and address the other cardiovascular risks that the affected person is likely to have.According to guidelines, taking aspirin or clopidogrel is recommended to reduce AMI ("heart attack"), stroke, and other causes of vascular death in people with symptomatic peripheral artery disease. It is recommended that aspirin and clopidogrel be taken alone and not in conjunction with one another (i.e., not as dual antiplatelet therapy). The recommended daily dosage of aspirin for treating PAD is between 75 and 325 mg, while the recommended daily dosage for clopidogrel is 75 mg. The effectiveness of both aspirin and clopidogrel to reduce risk of cardiovascular ischemic events in people with symptomatic PAD is not well established. | This system was created on the basis of ischemia and angiographic disease patterns not being the sole determinants of amputation risk. The WIfI classification system is broken up into two parts: wounds and ischemia. Wounds are graded 0 through 3 on the presence of ulceration and/or gangrene and ischemia. Grade 0: no ulcer, no gangrene
Grade 1: small, shallow ulcer; no gangrene
Grade 2: deep ulcer with exposed tendon or bone, gangrene limited to toes
Grade 3: extensive, full-thickness ulcer; gangrene extending to forefoot or midfootIschemia is graded 0 through 3 based on ABI, ankle systolic pressure, and toe pressure. Grade 0: ABI 0.8 or higher, ankle
Grade 1: arterial brachial index 0.6 to 0.79, ankle pressure 70 to 100 mm Hg, toe pressure 40 to 59 mm Hg
Grade 2: ABI 0.4–0.59, ankle pressure 50 to 70 mm Hg, toe pressure 30 to 39 mm HgThe TASC (and TASC II) classification suggested PAD treatment is based on the severity of disease seen on angiogram. Screening
It is not clear if screening for disease in the general population is useful as it has not been properly studied. This includes screening with the ankle-brachial index.Testing for coronary artery disease or carotid artery disease is of unclear benefit. While PAD is a risk factor for abdominal aortic aneurysms (AAA), there is no data on screening individuals with asymptomatic PAD for abdominal aortic aneurysms. In people with symptomatic PAD screening by ultrasound for AAA is not unreasonable. | 11
|
Pseudostrabismus is the false appearance of crossed eyes. When the eyes are actually crossed or not completely aligned with one another, it is called strabismus. Pseudostrabismus is more likely to be observed in East Asian or Native American infants, due to the presence of epicanthic folds obscuring the medial aspect of each eye. Pseudostrabismus generally occurs in infants and toddlers, whose facial features are not fully developed. The bridge of their nose is wide and flat, creating telecanthus (increased distance between medial canthus of both eyes). With age, the bridge will narrow, and the epicanthic folds in the corner of the eyes will go away. This will cause the eyes to appear wider and thus not have the appearance of strabismus. To detect the difference between strabismus and pseudostrabismus, clinicians use a flashlight to shine into the childs eyes. When the child is looking at the light, a reflection can be seen on the front surface of the pupil. If the eyes are aligned with one another, the reflection from the light will be in the same spot of each eye. If strabismus is present, the reflection from the light will not be in the same spot of each eye. Rakels Textbook of Family Medicine states, "A common misconception is that children with crossed eyes outgrow the condition, but this is generally not the case. This belief stems from the confusion between true strabismus and pseudostrabismus. | The World health organisation recommends giving amoxicillin-clavulanate along with meropenem as one of the therapeutic options in drug resistant tuberculosis, where clavulanate and not amoxicillin is being relied upon for anti TB activity. However, across the spectrum of dosage of amoxicillin-clavulanate combination, the dose of clavulanate is constant at 125 mg, whereas the dose of amoxicillin varies at 250 mg, 500 mg and 875 mg. Thus the use of low dose amoxicillin-clavulanate in combination with meropenem may be used in part of treatment regime for drug resistant TB and this has been demonstrated in a clinical setting also.This combination results in an antibiotic with an increased spectrum of action and restored efficacy against amoxicillin-resistant bacteria that produce β-lactamase. Adverse effects
Possible side effects include diarrhea, vomiting, nausea, thrush, and skin rash. These do not usually require medical attention. As with all antimicrobial agents, antibiotic-associated diarrhea due to Clostridium difficile infection—sometimes leading to pseudomembranous colitis—may occur during or after treatment with amoxicillin/clavulanic acid.Rarely, cholestatic jaundice (also referred to as cholestatic hepatitis, a form of liver toxicity) has been associated with amoxicillin/clavulanic acid. The reaction may occur up to several weeks after treatment has stopped, and usually takes weeks to resolve. It is more frequent in men, older people, and those who have taken long courses of treatment; the estimated overall incidence is one in 100,000 exposures. | 0-1
|
For example, neoplasms characterized by high-grade features, invasive glands and or signet ring cells, are termed adenocarcinoma in pathology literature. However, some pathologists (e.g., Odze and Goldblum, Surgical Pathology of the GI Tract, Liver, Biliary Tract and Pancreas, 2nd ed.) also use the term mucinous adenocarcinoma when referring to low-grade, well-differentiated tumors lacking high-grade features. Low-grade mucinous adenocarcinoma is used by the American Joint Committee on Cancer and World Health Organization and is nearly or completely synonymous with the DPAM designation. For low-grade mucinous adenocarcinoma, disease may be designated as "benign" because tumors do not invade deeply into tissue and rarely metastasize to parenchyma of organs; this designation may be misleading and confusing to the layperson because pseudomyxoma peritonei is not a harmless condition, fatal if untreated. High-grade or poorly differentiated mucinous adenocarcinoma has a generally poorer prognosis, though surgical treatment with heated intra-peritoneal chemotherapy (HIPEC) is yielding promising outcomes (see surgical treatment). Immunohistochemistry
Immunohistochemical features:
Diffuse expression of SATB2, CK20, CDX2, and mCEA
Sometimes patchy CK7; negative PAX8
High-grade neoplasms may show loss of DPC4 (10%)
Treatment
Treatment is variable, both due to its rarity and to its frequently slow-growing nature. Treatment ranges from watchful waiting to debulking and hyperthermic intraperitoneal chemotherapy (HIPEC, also called intraperitoneal hyperthermic chemotherapy, IPHC) with cytoreductive surgery. Surgical
The standard of care for mucinous adenocarcinoma with clinical condition PMP involves cytoreductive surgery (CRS) with hyperthermic intraperitoneal chemotherapy (HIPEC), performed by surgical oncologists who specialize in treating PMP. Some surgeons also apply early post-operative intraperitonial chemotherapy (EPIC), adjunct to surgical cytoreduction and HIPEC. | Classification
There is substantial debate regarding histopathologic classification of pseudomyxoma peritonei. In 1995, Ronnett et al. proposed separating pseudomyxoma peritonei cases into two diagnostic categories: adenoma (disseminated peritoneal adenomucinosis, DPAM) or carcinoma (peritoneal mucinous carcinomatosis, PMCA) with a third category reserved for cases with intermediate features. In this classification system, cases of DPAM were characterized by peritoneal lesions composed of abundant extracellular mucin containing scant simple to focally proliferative mucinous epithelium with little cytologic atypia or mitotic activity (in other words, most cells looked fairly normal and there was no evidence of mitosis which would indicate that cells were rapidly dividing), with or without an associated appendiceal mucinous adenoma. Cases of PMCA were characterized by peritoneal lesions composed of more abundant mucinous epithelium with the architectural and cytologic features of carcinoma (irregular cells, evidence that cells were rapidly dividing, and other criteria), with or without an associated primary mucinous adenocarcinoma. Bradley et al. (2007) argued that continued use of non-malignant terms, i.e., adenoma, for those frequent cases with low-grade features (such as DPAM), is misleading because pseudomyxoma peritonei is a disease state that results from invasion of the abdominal cavity by cells with uncontrolled growth. Bradley states that an adenoma, by definition, is a tumor confined to the appendiceal mucosa with absolutely no evidence of invasion beyond the muscularis mucosae. The term mucinous adenocarcinoma is used in different contexts depending on the reference material used by the pathologist for disease classification. | 11
|
They are produced by the dendritic, octopus-like melanocytes, seen between the epithelial cells situated closest to the epithelium/connective tissue border.In tobacco-users the melanocytes are stimulated to produce melanin granules and to distribute them out to the surrounding epithelial cells for further transport to the mucosal surface, like the mechanism in melanin-pigmented skin. Small amounts of melanin-like granules together with other electron-dense particles can also be seen within large melanosome complexes in the underlying connective tissue. If the granules derive from the epithelium, a phenomenon known as melanin incontinence, is not known. In Caucasians these granules are not expected to influence on the clinically observed degree of smokers melanosis. Causes
Smoking or the use of nicotine-containing drugs is the cause to Smokers melanosis,. Also tar-components (benzopyrenes) are known to stimulate melanocytes to melanin production, and other unknown toxic agents in tobacco may also be the cause. These chemical agents have a polycyclic, chain-like structure. Environmental tobacco smoke from parents is causing smokers melanosis in their children Swedish snuff causes a small elevation of oral melanin pigmented individuals from 3.0% to 4.7%. Nicotine tablets have shown to stimulate to melanin pigmentation of the oral mucosa. Treatment and prognosis
Lesions usually disappear between 3 months to 3 years for those who stop smoking. Smokers melanosis is a benign, normal physiological reaction, and does not develop into cancer. If it does not disappear, however, a biopsy can verify the diagnosis. | Symptoms
The most common symptoms of acquired and transient cortical blindness include:
A complete loss of visual sensation and of vision
Preservation/sparing of the abilities to perceive light and/or moving, but not static objects (Riddoch syndrome)
A lack of visual fixation and tracking
Denial of visual loss (Anton–Babinski syndrome)
Visual hallucinations
Macular sparing, in which vision in the fovea is spared from the blindness. Causes
The most common cause of cortical blindness is ischemia (oxygen deprivation) to the occipital lobes caused by blockage to one or both of the posterior cerebral arteries. However, other conditions have also been known to cause acquired and transient cortical blindness, including:
Congenital abnormalities of the occipital lobe
Head trauma to the occipital lobe of the brain
Bilateral lesions of the primary visual cortex
Infection
Creutzfeldt–Jakob disease (CJD), in association with a rapid onset of dementia
rarely Dissociative identity disorder (DID)
Side effect of some anti-epilepsy drugs (AEDs)
Hyperammonemia
Eclampsia and, rarely, pre-eclampsiaThe most common causes of congenital cortical blindness are:
Traumatic brain injury (TBI) to the occipital lobe of the brain
Congenital abnormalities of the occipital lobe
Perinatal ischemia
Encephalitis
Meningitis
Diagnosis
A patient with cortical blindness has no vision but the response of his/her pupil to light is intact (as the reflex does not involve the cortex). Therefore, one diagnostic test for cortical blindness is to first objectively verify the optic nerves and the non-cortical functions of the eyes are functioning normally. This involves confirming that patient can distinguish light/dark, and that his/her pupils dilate and contract with light exposure. Then, the patient is asked to describe something he/she would be able to recognize with normal vision. | 0-1
|
A comparison with MC58 showed that four genes are uniquely present in H44/76 and nine genes are only present in MC58. Of all ORFs in H44/76, 2,317 (93%) show more than 99% sequence identity.The complete genome sequence of strain NMA510612 (serogroup A) consists of one circular chromosome with a size of 2,188,020 bp, and the average GC content is 51.5%. The chromosome is predicted to possess 4 rRNA operons, 163 insertion elements (IS), 59 tRNAs, and 2,462 ORFs.There is a public database available for N. meningitidis core genome multilocus sequence typing (cMILST). Available at: https://pubmlst.org/bigsdb?db=pubmlst_neisseria_seqdef
Genetic transformation
Genetic transformation is the process by which a recipient bacterial cell takes up DNA from a neighboring cell and integrates this DNA into the recipients genome by recombination. In N. meningitidis, DNA transformation requires the presence of short DNA sequences (9–10 mers residing in coding regions) of the donor DNA. These sequences are called DNA uptake sequences (DUSs). Specific recognition of these sequences is mediated by a type IV pilin. In N. meningitidis DUSs occur at a significantly higher density in genes involved in DNA repair and recombination (as well as in restriction-modification and replication) than in other annotated gene groups. The over-representation of DUS in DNA repair and recombination genes may reflect the benefit of maintaining the integrity of the DNA repair and recombination machinery by preferentially taking up genome maintenance genes, that could replace their damaged counterparts in the recipient cell.N. meningititis colonizes the nasopharyngeal mucosa, which is rich in macrophages. | Growth can and often does fail, either because antibiotics have been given preemptively, or because specimens have been inappropriately transported, as the organism is extremely susceptible to antibiotics and fastidious in its temperature, CO2 and growth medium requirements. Polymerase chain reaction (PCR) tests where available, mostly in industrialized countries, have been increasingly used; PCR can rapidly identify the organism, and works even after antibiotics have been given. Prevention
All recent contacts of the infected patient over the 7 days before onset should receive medication to prevent them from contracting the infection. This especially includes young children and their child caregivers or nursery-school contacts, as well as anyone who had direct exposure to the patient through kissing, sharing utensils, or medical interventions such as mouth-to-mouth resuscitation. Anyone who frequently ate, slept or stayed at the patients home during the 7 days before the onset of symptom, or those who sat beside the patient on an airplane flight or classroom for 8 hours or longer, should also receive chemoprophylaxis. The agent of choice is usually oral rifampicin for a few days.Receiving a dose of the Meningococcal vaccine before traveling to a country in the "meningitis belt" or having a booster meningitis vaccine, normally five years apart could prevent someone from getting an infection from the pathogen. Vaccination
United States
A number of vaccines are available in the U.S. to prevent meningococcal disease. Some of the vaccines cover serogroup B, while others cover A, C, W, and Y. | 11
|
The more invasive methicillin-resistant Staphylococcus aureus (MRSA) may also be a source of infection, though is much rarer. Among spinal subdural abscesses, methicillin-sensitive Staphylococcus aureus is the most common organism involved.Rarely parasites can cause abscesses and this is more common in the developing world. Specific parasites known to do this include dracunculiasis and myiasis. Perianal abscess
Surgery of the anal fistula to drain an abscess treats the fistula and reduces likelihood of its recurrence and the need for repeated surgery. There is no evidence that fecal incontinence is a consequence of this surgery for abscess drainage.Perianal abscesses can be seen in people with, for example, inflammatory bowel disease (such as Crohns disease) or diabetes. Often the abscess will start as an internal wound caused by ulceration, hard stool, or penetrative objects with insufficient lubrication. This wound typically becomes infected as a result of the normal presence of feces in the rectal area, and then develops into an abscess. This often presents itself as a lump of tissue near the anus which grows larger and more painful with time. Like other abscesses, perianal abscesses may require prompt medical treatment, such as an incision and debridement or lancing. Incisional abscess
An incisional abscess is one that develops as a complication secondary to a surgical incision. It presents as redness and warmth at the margins of the incision with purulent drainage from it. If the diagnosis is uncertain, the wound should be aspirated with a needle, with aspiration of pus confirming the diagnosis and availing for Gram stain and bacterial culture. | Packing
In North America, after drainage, an abscess cavity is usually packed, often with special iodoform-treated cloth. This is done to absorb and neutralize any remaining exudate as well as to promote draining and prevent premature closure. Prolonged draining is thought to promote healing. The hypothesis is that though the hearts pumping action can deliver immune and regenerative cells to the edge of an injury, an abscess is by definition a void in which no blood vessels are present. Packing is thought to provide a wicking action that continuously draws beneficial factors and cells from the body into the void that must be healed. Discharge is then absorbed by cutaneous bandages and further wicking promoted by changing these bandages regularly. However, evidence from emergency medicine literature reports that packing wounds after draining, especially smaller wounds, causes pain to the person and does not decrease the rate of recurrence, nor bring faster healing, or fewer physician visits. Loop drainage
More recently, several North American hospitals have opted for less-invasive loop drainage over standard drainage and wound packing. In one study of 143 pediatric outcomes, a failure rate of 1.4% was reported in the loop group versus 10.5% in the packing group (P<.030), while a separate study reported a 5.5% failure rate among the loop group. Primary closure
Closing an abscess immediately after draining it appears to speed healing without increasing the risk of recurrence. This may not apply to anorectal abscesses as while they may heal faster, there may be a higher rate of recurrence than those left open. | 11
|
The affected individual might feel that these actions will either prevent a dreaded event from occurring, or push the event from their thoughts. In any case, their reasoning is so idiosyncratic or distorted that it results in significant distress, either personally, or for those around the affected individual. Excessive skin picking, hair pulling, nail biting, and other body-focused repetitive behavior disorders are all on the obsessive–compulsive spectrum. Some individuals with OCD are aware that their behaviors are not rational, but they feel compelled to follow through with them to fend off feelings of panic or dread. Furthermore, compulsions often stem from memory distrust, a symptom of OCD characterized by insecurity in ones skills in perception, attention, and memory, even in cases where there is no clear evidence of a deficit.Common compulsions may include hand washing, cleaning, checking things (such as locks on doors), repeating actions (such as repeatedly turning on and off switches), ordering items in a certain way, and requesting reassurance. Although some individuals perform actions repeatedly, they do not necessarily perform these actions compulsively; for example, morning or nighttime routines and religious practices are not usually compulsions. Whether behaviors qualify as compulsions or mere habit depends on the context in which they are performed. For instance, arranging and ordering books for eight hours a day would be expected of someone who works in a library, but this routine would seem abnormal in other situations. In other words, habits tend to bring efficiency to ones life, while compulsions tend to disrupt it. | Randomized controlled trials have shown that DBT and MBT may be the most effective, and the two share many similarities. Researchers are interested in developing shorter versions of these therapies to increase accessibility, to relieve the financial burden on patients, and to relieve the resource burden on treatment providers.Some research indicates that mindfulness meditation may bring about favorable structural changes in the brain, including changes in brain structures that are associated with BPD. Mindfulness-based interventions also appear to bring about an improvement in symptoms characteristic of BPD, and some clients who underwent mindfulness-based treatment no longer met a minimum of five of the DSM-IV-TR diagnostic criteria for BPD. Medications
A 2010 review by the Cochrane collaboration found that no medications show promise for "the core BPD symptoms of chronic feelings of emptiness, identity disturbance, and abandonment". However, the authors found that some medications may impact isolated symptoms associated with BPD or the symptoms of comorbid conditions. A 2017 review examined evidence published since the 2010 Cochrane review and found that "evidence of effectiveness of medication for BPD remains very mixed and is still highly compromised by suboptimal study design". A 2020 review found that research into pharmacological treatments had declined, with more results confirming no benefits. The review found "moderate to large, statistically significant effects for both doses of quetiapine (150 mg/day and 300 mg/day) regarding BPD severity, psychosocial impairment and aggression, and an additional effect for the higher dose regarding manic symptoms." | 0-1
|
Likewise, a change in
x
j
{\displaystyle x_{j}}
is not necessary to change y, because a change in y could be caused by something implicit in the error term (or by some other causative explanatory variable included in the model). The above way of testing for causality requires belief that there is no reverse causation, in which y would cause
x
j
{\displaystyle x_{j}}
. This belief can be established in one of several ways. | Algorithms have been developed to systematically determine the skeleton of the underlying graph and, then, orient all arrows whose directionality is dictated by the conditional independencies observed.Alternative methods of structure learning search through the many possible causal structures among the variables, and remove ones which are strongly incompatible with the observed correlations. In general this leaves a set of possible causal relations, which should then be tested by analyzing time series data or, preferably, designing appropriately controlled experiments. In contrast with Bayesian Networks, path analysis (and its generalization, structural equation modeling), serve better to estimate a known causal effect or to test a causal model than to generate causal hypotheses. For nonexperimental data, causal direction can often be inferred if information about time is available. This is because (according to many, though not all, theories) causes must precede their effects temporally. This can be determined by statistical time series models, for instance, or with a statistical test based on the idea of Granger causality, or by direct experimental manipulation. The use of temporal data can permit statistical tests of a pre-existing theory of causal direction. For instance, our degree of confidence in the direction and nature of causality is much greater when supported by cross-correlations, ARIMA models, or cross-spectral analysis using vector time series data than by cross-sectional data. Derivation theories
Nobel laureate Herbert A. Simon and philosopher Nicholas Rescher claim that the asymmetry of the causal relation is unrelated to the asymmetry of any mode of implication that contraposes. | 11
|
However, there is no clear evidence that has shown live vaccinations to increase the risk for miscarriage or fetal abnormalities.Some live vaccinations include: MMR, varicella, certain types of the influenza vaccine, and rotavirus. Treatments for cancer
Ionizing radiation levels given to a woman during cancer treatment cause miscarriage. Exposure can also impact fertility. The use of chemotherapeutic drugs used to treat childhood cancer increases the risk of future miscarriage. Pre-existing diseases
Several pre-existing diseases in pregnancy can potentially increase the risk of miscarriage, including diabetes, polycystic ovary syndrome (PCOS), hypothyroidism, certain infectious diseases, and autoimmune diseases. PCOS may increase the risk of miscarriage. Two studies suggested treatment with the drug metformin significantly lowers the rate of miscarriage in women with PCOS, but the quality of these studies has been questioned. Metformin treatment in pregnancy has not been shown to be safe. In 2007 the Royal College of Obstetricians and Gynaecologists also recommended against use of the drug to prevent miscarriage. Thrombophilias or defects in coagulation and bleeding were once thought to be a risk in miscarriage but have been subsequently questioned. Severe cases of hypothyroidism increase the risk of miscarriage. The effect of milder cases of hypothyroidism on miscarriage rates has not been established. A condition called luteal phase defect (LPD) is a failure of the uterine lining to be fully prepared for pregnancy. | This involves no radiation, no needles and no contrast agents that may cause allergic reactions. This test has good sensitivity and specificity.Typically duplex ultrasound scan is the only investigation required for decision making in carotid stenosis as it is widely available and rapidly performed. However, further imaging can be required if the stenosis is not near the bifurcation of the carotid artery.One of several different imaging modalities, such as a computed tomography angiogram (CTA) or magnetic resonance angiogram (MRA) may be useful. Each imaging modality has its advantages and disadvantages - Magnetic resonance angiography and CT angiography with contrast is contraindicated in patients with chronic kidney disease, catheter angiography has a 0.5% to 1.0% risk of stroke, MI, arterial injury or retroperitoneal bleeding. The investigation chosen will depend on the clinical question and the imaging expertise, experience and equipment available.For purpose of treatment, the degree of carotid stenosis is defined as:
percent stenosis = ( 1 − ( minimum diameter within stenosis) / ( poststenotic diameter ) ) × 100%. Screening
The U.S. Preventive Services Task Force (USPSTF) recommends against routine screening for carotid artery stenosis in those without symptoms as of 2021.While routine population screening is not advised, the American Heart Association and the Society for Vascular Surgery recommend screening in those diagnosed with related medical conditions or have risk factors for carotid artery disease. | 0-1
|
In the largest U.S. study, only 29% of patients received a liver graft, while 10% of the overall group (one fourth of patients listed for transplantation) died on the waiting list. Other series have reported death rates of those listed for transplant as high as 40%. In the ALFSG, the transplantation rate was higher in the groups with lower short-term spontaneous survival, making overall survival similar in all groups: acetaminophen, 73%; drug induced, 70%; indeterminate group, 64%; and other causes, 61%. Causes of death for the 101 patients who died within the 3-week period included cerebral edema, multiorgan failure, sepsis, cardiac arrhythmia or arrest and respiratory failure. The median time to death after admission was 5 days. Acetylcysteine
Intravenous N-acetylcysteine has been found to be beneficial in both acetaminophen toxicity and non-acetaminophen-related acute liver failure. Prognosis
Historically mortality has been high, being in excess of 80%. In recent years the advent of liver transplantation and multidisciplinary intensive care support have improved survival significantly. At present overall short-term survival with transplant is more than 65%.Several prognostic scoring systems have been devised to predict mortality and to identify who will require an early liver transplant. These include Kings College Hospital criteria, MELD score, APACHE II, and Clichy criteria. Terminology
To date, no universally accepted nomenclature has been adopted. | Definition
Acute liver failure is defined as "the rapid development of hepatocellular dysfunction, specifically coagulopathy and mental status changes (encephalopathy) in a patient without known prior liver disease".page 1557The diagnosis of acute liver failure is based on physical exam, laboratory findings, patient history, and past medical history to establish mental status changes, coagulopathy, rapidity of onset, and absence of known prior liver disease respectively.page 1557The exact definition of "rapid" is somewhat questionable, and different sub-divisions exist which are based on the time from onset of first hepatic symptoms to onset of encephalopathy. One scheme defines "acute hepatic failure" as the development of encephalopathy within 26 weeks of the onset of any hepatic symptoms. This is sub-divided into "fulminant hepatic failure", which requires onset of encephalopathy within 8 weeks, and "subfulminant", which describes onset of encephalopathy after 8 weeks but before 26 weeks. Another scheme defines "hyperacute" as onset within 7 days, "acute" as onset between 7 and 28 days, and "subacute" as onset between 28 days and 24 weeks.page 1557
Treatment
General concerns
Because ALF often involves the rapid deterioration of mental status and the potential for multiorgan failure, patients should be managed in the intensive care unit. For patients not at a transplant center, the possibility of rapid progression of ALF makes early consultation with a transplant facility critical. Accordingly, plans for transfer to a transplant center should begin in patients with any abnormal mentation. Early institution of antidotes or specific therapy may prevent the need for liver transplantation and reduce the likelihood of poor outcome. | 11
|
Normal tension glaucoma (NTG) is an eye disease, a neuropathy of the optic nerve, that shows all the characteristics of primary open angle glaucoma except one: the elevated intraocular pressure (IOP) - the classic hallmark of glaucoma - is missing. Normal tension glaucoma is in many cases closely associated with general issues of blood circulation and of organ perfusion like arterial hypotension, metabolic syndrome, and Flammer syndrome. Clinical relevance
Over many years, glaucoma has been defined by an intraocular pressure of more than 20 mm Hg. Incompatible with this (now obsolete) definition of glaucoma was the ever larger number of cases that have been reported in medical literature in the 1980s and 1990s who had the typical signs of glaucomatous damage, like optic nerve head excavation and thinning of the retinal nerve fiber layer, while these patients had an IOP that would generally have been regarded as "normal". It is now widely estimated that a larger percentage of patients with primary open-angle glaucoma (POAG) are suffering from normal tension glaucoma. Among Americans of Japanese descent, for instance, the prevalence of NTG is about four times as high as the prevalence of the "classical glaucoma" with an IOP of 22 mm Hg and higher. | In patients with systemic hypertension under therapy, the blood pressure should not be lowered too rigorously. NTG might be the only severe (= sight-threatening) disease caused in numerous cases by a too low blood pressure. Both magnesium and low dose calcium channel blockers have been employed in the treatment of some NTG patients. There are therapeutic approaches to underlying conditions like Flammer syndrome. A change in nutrition like the intake of sodium-rich foods has been tried as has the oral administration of low-dosed steroids. Lifestyle interventions are recommended in patients with Flammer syndrome like avoidance of fasting and certain stimuli like a cold environment and stress. == References == | 11
|
They can be triggered in certain individuals, by alcohol, sleep deprivation, physical activity, emotional stress, depression, medications, or a fevered illness. These disorders of arousal can range from confusional arousals, somnambulism, to night terrors. Other specific disorders include sleepeating, sleep sex, teeth grinding, rhythmic movement disorder, restless legs syndrome, and somniloquy. Differential diagnosis for NREM-related parasomnias
Sleep-disordered breathing
REM-related parasomnias
Nocturnal seizures
Psychogenic dissociative disorders
Confusional arousals
Confusional arousal is a condition when an individual awakens from sleep and remains in a confused state. It is characterized by the individuals partial awakening and sitting up to look around. They usually remain in bed and then return to sleep. These episodes last anywhere from seconds to minutes and may not be reactive to stimuli. Confusional arousal is more common in children than in adults. It has a lifetime prevalence of 18.5% in children and a lifetime prevalence of 2.9–4.2% in adults. Infants and toddlers usually experience confusional arousals beginning with large amounts of movement and moaning, which can later progress to occasional thrashings or inconsolable crying. In rare cases, confusional arousals can cause injuries and drowsy driving accidents, thus it can also be considered dangerous. Another sleeping disorder may be present triggering these incomplete arousals. Somniloquy (sleep talking)
A form of parasomnia where a person will speak during their dreams. Sleep-related abnormal sexual behavior
Sleep-related abnormal sexual behavior, Sleep sex, or sexsomnia, is a form of confusional arousal that may overlap with somnambulism. Thereby, a person will engage in sexual acts while still asleep. | Sleep enuresis
Parasomnias due to medical disorder
Parasomnias due to medication or substance
Parasomnia, unspecific
Sleep drunkenness, also known as confusional arousal, is the feeling of confusion or sudden action upon waking up from deep sleep. Severe sleep inertia, one cause of oversleeping, is considered to develop sleep drunkenness. Isolated symptom/normal variant
Sleep talking (somniloquy)
According to ICSD-3 it is not defined a disorder in particular. It is rather an isolated symptom or normal variant and ranges from isolated speech to full conversations without recall. With a lifetime prevalence of 69% it is considered fairly common. Sleep talking is associated with REM-related parasomnias as well as with disorders or arousal. It occurs in all sleep states. As yet, there is no specific treatment for sleeptalking available. Diagnosis
Parasomnias are most commonly diagnosed by means of questionnaires. These questionnaires include a detailed analyses of the clinical history and contain questions to:
Rule out sleep deprivation
Rule out effects of intoxication or withdrawal
Rule out sleep disorders causing sleep instability
Rule out medical disorders or treatments associated with sleep instability
Confirm presence of NREM parasomnias in other family members and during the patients childhood
Determine the timing of the events
Determine the morphology of the events.Furthermore, a sleep diary is helpful to exclude that sleep deprivation could be a precipitating factor. An additional tool could be the partners log of the events. The following questions should therefore be considered:
Do you or your bed partner believe that you move your arms, legs, or body too much, or have unusual behaviors during sleep? | 11
|
Additionally, some individuals may require ongoing medication therapy at home, which may include diuretics (such as furosemide or spironolactone), analgesics (such as paracetamol), cardiac glycosides (such as digoxin), anticoagulants (such as heparin or aspirin), or other medications. If the individual has undergone stenting, an anticoagulant will be a necessity to prevent build-up around the stent(s), as the body will perceive the foreign body as a wound and attempt to heal it. Some patients who had alternate corrective surgery, such as the Mustard or Senning procedure, may have issues with SA and VA nodal transmissions in later life. Typical symptoms include palpitations and problems with low heart rates. This is commonly solved with a Pacemaker unit, providing scar tissue from the original operation does not block its functionality. More recently, ACE inhibitors have been prescribed to patients in the hope of relieving stress on the heart. Prognosis
With simple d-TGA, if the foramen ovale and ductus arteriosus are allowed to close naturally, the newborn will likely not survive long enough to receive corrective surgery. With complex d-TGA, the infant will fail to thrive and is unlikely to survive longer than a year if corrective surgery is not performed. | dextro-Transposition of the great arteries (d-Transposition of the great arteries, dextro-TGA, or d-TGA) is a potentially life-threatening birth defect in the large arteries of the heart. The primary arteries (the aorta and the pulmonary artery) are transposed.It is called a cyanotic congenital heart defect (CHD) because the newborn infant turns blue from lack of oxygen. In segmental analysis, this condition is described as ventriculoarterial discordance with atrioventricular concordance, or just ventriculoarterial discordance. d-TGA is often referred to simply as transposition of the great arteries (TGA); however, TGA is a more general term which may also refer to levo-transposition of the great arteries (l-TGA).Another term commonly used to refer to both d-TGA and l-TGA is transposition of the great vessels (TGV), although this term might have an even broader meaning than TGA. Prenatally, a baby with d-TGA experiences no symptoms as the lungs will not be used until after birth, and oxygen is provided by the mother via the placenta and umbilical cord; in order for the red blood to bypass the lungs in utero, the fetal heart has two shunts that begin to close when the newborn starts breathing; these are the foramen ovale and the ductus arteriosus. The foramen ovale is a hole in the atrial septum which allows blood from the right atrium to flow into the left atrium; after birth, the left atrium will be filled with blood returning from the lungs and the foramen ovale will close. | 11
|
Ramelteon and fluvoxamine should not be coadministered.Ramelteon has significant drug–drug interaction with the following drugs: amiodarone, ciprofloxacin, fluvoxamine, ticlopidine. Ramelteon should be administered with caution in patients taking other CYP1A2 inhibitors, strong CYP3A4 inhibitors such as ketoconazole, and strong CYP2C9 inhibitors such as fluconazole.Efficacy may be reduced when ramelteon is used in combination with potent CYP enzyme inducers such as rifampin, since ramelteon concentrations may be decreased. Pharmacology
Pharmacodynamics
Ramelteon is a melatonin receptor agonist with both high affinity for melatonin MT1 and MT2 receptors and selectivity over the non-human MT3 receptor. Ramelteon demonstrates full agonist activity in vitro in cells expressing human MT1 or MT2 receptors, and high selectivity for human MT1 and MT2 receptors compared to the non-human MT3 receptor. The affinity of ramelteon for the MT1 and MT2 receptors is 3 to 16 times higher than that of melatonin. Ramelteon has 8-fold higher affinity for the MT1 receptor over the MT2 receptor. The binding profile of ramelteon distinguishes it from melatonin, tasimelteon, and agomelatine.The major metabolite of ramelteon, M-II, is active and has approximately one-tenth and one-fifth the binding affinity of the parent molecule for the human MT1 and MT2 receptors, respectively, and is 17- to 25-fold less potent than ramelteon in in vitro functional assays. Although the potency of M-II at MT1 and MT2 receptors is lower than the parent drug, M-II circulates at higher concentrations than the parent producing 20- to 100-fold greater mean systemic exposure when compared to ramelteon. | Optic papillitis is a specific type of optic neuritis. Inflammation of the optic nerve head is called "papillitis" or "intraocular optic neuritis"; inflammation of the orbital portion of the nerve is called "retrobulbar optic neuritis" or "orbital optic neuritis". It is often associated with substantial losses in visual fields, pain on moving the globe, and sensitivity to light pressure on the globe. It is often an early sign of multiple sclerosis.Papillitis may have the same appearance as papilledema. However, papillitis may be unilateral, whereas papilledema is almost always bilateral. Papillitis can be differentiated from papilledema by an afferent pupillary defect (Marcus Gunn pupil), by its greater effect in decreasing visual acuity and color vision, and by the presence of a central scotoma. Papilledema that is not yet chronic will not have as dramatic an effect on vision. Because increased intracranial pressure can cause both papilledema and a sixth (abducens) nerve palsy, papilledema can be differentiated from papillitis if esotropia and loss of abduction are also present. However, esotropia may also develop secondarily in an eye that has lost vision from papillitis. Retrobulbar neuritis, an inflamed optic nerve, but with a normal-appearing nerve head, is associated with pain and the other findings of papillitis. Pseudopapilledema is a normal variant of the optic disk, in which the disk appears elevated, with indistinct margins and a normal vascular pattern. Pseudopapilledema sometimes occurs in hyperopic individuals. Workup of the patient with papillitis includes lumbar puncture and cerebrospinal fluid analysis. B henselae infection can be detected by serology. | 0-1
|
Contraindications
Vaccination with live-attenuated or live vaccines is not recommended during treatment and should be administered at least four weeks before starting satralizumab. Side effects
The most common side effects observed were the common cold (nasopharyngitis), headache, upper respiratory tract infection, inflammation of the lining of the stomach, rash, joint pain, extremity pain, fatigue and nausea.The FDA label for satralizumab includes a warning for increased risk of infection, including serious and potentially fatal infections – such as potential reactivation of hepatitis B and tuberculosis. Other warnings and precautions for satralizumab include elevated liver enzymes, decreased neutrophil counts and hypersensitivity reactions. Pharmacology
Mechanism of action
Satralizumab is a humanized IgG2 monoclonal antibody that binds to soluble and membrane-bound human interleukin-6 (IL-6) receptors and thereby prevents IL-6-mediated signal transmission through these receptors.IL-6 is a pleiotropic cytokine that is produced by a large number of cell types and is involved in a variety of inflammatory processes. Patients with NMO and NMOSD have elevated levels of IL-6 in cerebro-spinal fluid and serum during periods of active disease. Some of the pro-inflammatory processes involved in NMOSD are thought to involve IL-6, including the formation of pathological autoantibodies against aquaporin-4 (AQP4), and the permeability of the blood-brain barrier to mediators of inflammation. Efficacy
The effectiveness and safety of satralizumab for the treatment of neuromyelitis optica spectrum disorder (NMOSD) were demonstrated in two 96-week clinical studies.A study of satralizumab as monotherapy for NMOSD included 95 adult participants, 64 of whom had antibodies against AQP4 (i.e. were anti-AQP4 positive). | The amyloid plaque load and dense-core Congo red positive plaque load in the cortex were reduced by both drugs at all doses. The chronic inflammation response (microglial activation) was also reduced by all treatments
Cai HY et al. demonstrated in a study that lixisenatide could reduce amyloid plaques, neurofibrillary tangles and neuroinflammation in the hippocampi of 12-month-old APP/PS1/tau female mice; activation of PKA-CREB signaling pathway and inhibition of p38-MAPK might be the important mechanisms in the neuroprotective function of lixisenatide. So, lixisenatide might have the potential to be developed as a novel therapy for AD
Liu Wet al found an interesting results when comparing exendin-4 (10 nmol/kg), liraglutide (25 nmol/kg) and lixisenatide (10 nmol/kg), it was found that exendin-4 showed no protective effects at the dose chosen, while both liraglutide and lixisenatide showed effects in preventing the MPTP-induced motor impairment (Rotarod, open-field locomotion, catalepsy test), reduction in tyrosine hydroxylase (TH) levels (dopamine synthesis) in the substantia nigra and basal ganglia, a reduction of the pro-apoptotic signaling molecule BAX and an increase in the anti-apoptotic signaling molecule B-cell lymphoma-2. The previous results demonstrate that both liraglutide and lixisenatide are superior to exendin-4, and both drugs show promise as a novel treatment of Parkinson disease
Another study done by Kerry Hunter et al. profiled the GLP-1 receptor agonists liraglutide and lixisenatide. The kinetics of crossing the blood brain barrier (BBB), activation of the GLP-1R by measuring cAMP levels, and physiological effects in the brain on neuronal stem cell proliferation and neurogenesis were evaluated. Both drugs were able to cross the BBB. | 0-1
|
Nix or NIX may refer to:
Places
Nix, Alabama, an unincorporated community, United States
Nix, Texas, a ghost town in southwestern Lampasas County, Texas, United States
Nix (moon), a moon of Pluto
People
Nix (surname), listing people with the surname
In science and technology
Nix (gene), a pro-apoptotic gene
Norwegian Internet Exchange (NIX), an Internet exchange point in Oslo
Neutral Internet Exchange of the Czech Republic, the Internet exchange point in Prague
Nix package manager, a "purely functional" package and configuration manager for computer systems
Unix-like, abbreviated *nix or nix
Codes
Nioro Airport, Mali (IATA airport code: NIX)
Hema language, (ISO 639-3 code: nix)
Other uses
Nix Federal Building, a historic building in Philadelphia, Pennsylvania
Nix Professional Building, a hospital in San Antonio, Texas
Permethrin, branded as Nix in North America, an anti-lice drug
"Nix", a song by Golden Boy with Miss Kittin from Or
The Nix, a 2017 novel by Nathan Hill
Neck (water spirit) or nix, an aquatic being in Germanic folklore
See also
All pages with titles beginning with Nix
Nyx (disambiguation)
Nixe (disambiguation)
Nick (disambiguation)
NIC (disambiguation)
New York Knicks, basketball team | Epidermal nevus syndrome (also known as "Feuerstein and Mims syndrome", and "Solomons syndrome": 775 ) is a rare disease that was first described in 1968 and consists of extensive epidermal nevi with abnormalities of the central nervous system (CNS), skeleton, skin, cardiovascular system, genitourinary system and eyes. : 634 However, since the syndromes first description, a broader concept for the "epidermal nevus" syndrome has been proposed, with at least six types being described:: 776
Schimmelpenning syndrome
Nevus comedonicus syndrome
Pigmented hairy epidermal nevus syndrome
Proteus syndrome
CHILD syndrome
Phakomatosis pigmentokeratotica
See also
Epidermis
List of cutaneous conditions
References
== External links == | 0-1
|
The Salk vaccine had been 60–70% effective against PV1 (poliovirus type 1), over 90% effective against PV2 and PV3, and 94% effective against the development of bulbar polio. Soon after Salks vaccine was licensed in 1955, childrens vaccination campaigns were launched. In the U.S, following a mass immunization campaign promoted by the March of Dimes, the annual number of polio cases fell from 35,000 in 1953 to 5,600 by 1957. By 1961 only 161 cases were recorded in the United States.A week before the announcement of the Francis Field Trial results in April 1955, Pierre Lépine at the Pasteur Institute in Paris had also announced an effective polio vaccine. Safety incidents
In April 1955, soon after mass polio vaccination began in the US, the Surgeon General began to receive reports of patients who contracted paralytic polio about a week after being vaccinated with Salk polio vaccine from the Cutter pharmaceutical company, with the paralysis limited to the limb the vaccine was injected into. The Cutter vaccine had been used in vaccinating 200,000 children in the western and midwestern United States. Later investigations showed that the Cutter vaccine had caused 40,000 cases of polio, killing 10. In response the Surgeon General pulled all polio vaccines made by Cutter Laboratories from the market, but not before 250 cases of paralytic illness had occurred. Wyeth polio vaccine was also reported to have paralyzed and killed several children. | Function of the Golgi apparatus in protein maturation
The most important subcellular structure in the context of wrinkly skin syndrome (WSS), is the Golgi apparatus. The Golgi apparatus is an important part of the endomembrane system because it processes proteins and lipids prior to their delivery to the plasma membrane and/or secretion into the extracellular environment. The Golgi is organized into a polarized series of membrane-bound stacks, called cisternae, through which proteins are trafficked in sequence once they leave the endoplasmic reticulum (ER), where the proteins and lipids are synthesized. Proteins destined for secretion or delivery to the plasma membrane arrive first at the cis-Golgi, before being trafficked through the medial and trans-Golgi. In the Golgi, proteins undergo extensive post-translational modifications (PTMs). In the context of WSS, the most significant PTM events are the glycosylation of proteins comprising the extracellular matrix (ECM) of epidermal cells. The two types of glycosylation events in the Golgi are N-linked glycosylation and O-linked glycosylation. Glycosylation of proteins destined for secretion occurs through the forward movement of proteins throughout the Golgi apparatus. The proteins destined for secretion are then trafficked to the plasma membrane in secretory vesicles. Retrograde (backward) transport in the Golgi apparatus is also important. In order to retain the enzymes responsible for protein glycosylation in the correct regions of the Golgi, there must be retrograde transport of these enzymes back into the Golgi apparatus. | 0-1
|
Then, 10 to 20% of those develop scarring will have increased risk of hypertension in later life. Epidemiology
Urinary tract infections are the most frequent bacterial infection in women. They occur most frequently between the ages of 16 and 35 years, with 10% of women getting an infection yearly and more than 40–60% having an infection at some point in their lives. Recurrences are common, with nearly half of people getting a second infection within a year. Urinary tract infections occur four times more frequently in females than males. Pyelonephritis occurs between 20 and 30 times less frequently. They are the most common cause of hospital-acquired infections accounting for approximately 40%. Rates of asymptomatic bacteria in the urine increase with age from two to seven percent in women of child-bearing age to as high as 50% in elderly women in care homes. Rates of asymptomatic bacteria in the urine among men over 75 are between 7–10%. Asymptomatic bacteria in the urine occurs in 2% to 10% of pregnancies.Urinary tract infections may affect 10% of people during childhood. Among children, urinary tract infections are most common in uncircumcised males less than three months of age, followed by females less than one year. Estimates of frequency among children, however, vary widely. In a group of children with a fever, ranging in age between birth and two years, two to 20% were diagnosed with a UTI. History
Urinary tract infections have been described since ancient times with the first documented description in the Ebers Papyrus dated to c. 1550 BC. | Autosomal recessive axonal neuropathy with neuromyotonia, also known as Gamstorp-Wohlfart syndrome, is a rare hereditary disorder which is characterized by progressive poly-neuropathy, neuromyotonia, myokymia, pseudo-myotonia, hand-foot contractures, and abnormal neuro-myotonic/myokimic activity visible on needle EMG. According to OMIM, around 52 cases have been reported in medical literature However; new cases (mostly from Europe and North America) have been reported since 2014. Description
People with this disorder usually show the following symptoms: axonal neuropathy, atrophy (wasting/degeneration) of the muscles in the hands, feet and legs, chronic muscular weakness which is very apparent when exercise is being done, abnormal gait, high chance of accidental falls, and joint contractures, neuromyotonia, and myokymia. In some people, the axonal neuropathy ends up reducing their sensitivity to cold and hot temperatures and touch in the distal parts of the arms and legs. Some of the symptoms worsen temporarily when a person with this disorder is exposed to cold temperatures. Causes
This disorder is caused by a homozygous mutation in the HINT1 gene, in chromosome 5 (c.334 C > A, p.H112 N). Etimology
This condition was discovered in 1991 by Hahn et al., when they described two Chinese-Canadian siblings of the opposite sex. | 0-1
|
However, testicular torsion may cause abnormal sperm function on semen analysis, although these abnormalities are more likely to be found in adolescents and in adults. Torsion does not seem to affect long-term sperm function in neonates. The cause of abnormal sperm function is thought to be due to the following mechanisms:
Immunological theory, also known as "sympathetic orchidopathia": It is thought that following injury to the testicle, the bodys immune system is activated to clean up damaged cells. In the process, it creates anti-testicular cell antibodies, or proteins that cross the injured blood-testis barrier and damage both the affected and contralateral testicles. Abnormalities in microcirculation within the testicle
Reperfusion injury: This type of injury is seen in tissues that have been deprived of blood supply for a prolonged period. Gangrene, or a type of tissue damage caused by lack of blood supply, of the testis. Sepsis, in extremely rare cases (0,03%), if not treated for a long period of time, it could lead to sepsis and cause severe life-threatening infections and injuries through the blood and organs, which could lead to death. Recurrence of torsion may occur even after surgical fixation, although this is very unlikely. Psychological impact of losing a testicle. Risk factors
Most of those affected with testicular torsion have no prior underlying health problems or predisposing conditions. However, there are certain factors that may increase risk of testicular torsion. A larger testicle either due to normal variation or testicular tumor increases the risk of torsion. | Clinical exam
The absence of the cremasteric reflex in an acutely painful testicle is most indicative of testicular torsion (the twisting of the spermatic cord of the testicle makes reflexive responses all but impossible). The cremasteric reflex normally causes elevation of the testicle by stroking the inner thigh. Absence is especially common in children, but its presence does not exclude a diagnosis of testicular torsion.On physical examination, the testis can be swollen, tender, high-riding, and with an abnormal transverse lie.Prehns sign, a classic physical exam finding, has not been reliable in distinguishing torsion from other causes of testicular pain such as epididymitis. The individual will not usually have a fever, though nausea is common. Imaging
A doppler ultrasound scan of the scrotum can identify the absence of blood flow in the twisted testicle and is nearly 90% accurate in diagnosis. It can also help distinguish torsion from epididymitis.Radionuclide scanning (scintigraphy) of the scrotum is the most accurate imaging technique, but it is not routinely available, particularly with the urgency that might be required. The agent of choice for this purpose is technetium-99m pertechnetate. Initially it provides a radionuclide angiogram, followed by a static image after the radionuclide has perfused the tissue. In the healthy patient, initial images show symmetric flow to the testes, and delayed images show uniformly symmetric activity. In testicular torsion, the images may show heterogenous activity within the affected testicle. Treatment
Testicular torsion is a surgical emergency that requires immediate intervention to restore the flow of blood to the testicle. | 11
|
Withdrawal of methamphetamine in dependent persons may lead to post-acute withdrawal which persists months beyond the typical withdrawal period.Magnetic resonance imaging studies on human methamphetamine users have also found evidence of neurodegeneration, or adverse neuroplastic changes in brain structure and function. In particular, methamphetamine appears to cause hyperintensity and hypertrophy of white matter, marked shrinkage of hippocampi, and reduced gray matter in the cingulate cortex, limbic cortex, and paralimbic cortex in recreational methamphetamine users. Moreover, evidence suggests that adverse changes in the level of biomarkers of metabolic integrity and synthesis occur in recreational users, such as a reduction in N-acetylaspartate and creatine levels and elevated levels of choline and myoinositol.Methamphetamine has been shown to activate TAAR1 in human astrocytes and generate cAMP as a result. Activation of astrocyte-localized TAAR1 appears to function as a mechanism by which methamphetamine attenuates membrane-bound EAAT2 (SLC1A2) levels and function in these cells.Methamphetamine binds to and activates both sigma receptor subtypes, σ1 and σ2, with micromolar affinity. Sigma receptor activation may promote methamphetamine-induced neurotoxicity by facilitating hyperthermia, increasing dopamine synthesis and release, influencing microglial activation, and modulating apoptotic signaling cascades and the formation of reactive oxygen species. Addictive
Current models of addiction from chronic drug use involve alterations in gene expression in certain parts of the brain, particularly the nucleus accumbens. The most important transcription factors that produce these alterations are ΔFosB, cAMP response element binding protein (CREB), and nuclear factor kappa B (NFκB). | Due to the high lipophilicity of methamphetamine, it can readily move through the blood–brain barrier faster than other stimulants, where it is more resistant to degradation by monoamine oxidase. The amphetamine metabolite peaks at 10–24 hours. Methamphetamine is excreted by the kidneys, with the rate of excretion into the urine heavily influenced by urinary pH. When taken orally, 30–54% of the dose is excreted in urine as methamphetamine and 10–23% as amphetamine. Following IV doses, about 45% is excreted as methamphetamine and 7% as amphetamine. The elimination half-life of methamphetamine varies with a range of 5–30 hours, however it is on average 9 to 12 hours in most studies. The elimination half-life of methamphetamine does not vary by route of administration, but is subject to substantial interindividual variability.CYP2D6, dopamine β-hydroxylase, flavin-containing monooxygenase 3, butyrate-CoA ligase, and glycine N-acyltransferase are the enzymes known to metabolize methamphetamine or its metabolites in humans. The primary metabolites are amphetamine and 4-hydroxymethamphetamine; other minor metabolites include: 4-hydroxyamphetamine, 4-hydroxynorephedrine, 4-hydroxyphenylacetone, benzoic acid, hippuric acid, norephedrine, and phenylacetone, the metabolites of amphetamine. Among these metabolites, the active sympathomimetics are amphetamine, 4‑hydroxyamphetamine, 4‑hydroxynorephedrine, 4-hydroxymethamphetamine, and norephedrine. Methamphetamine is a CYP2D6 inhibitor.The main metabolic pathways involve aromatic para-hydroxylation, aliphatic alpha- and beta-hydroxylation, N-oxidation, N-dealkylation, and deamination. The known metabolic pathways include:
Detection in biological fluids
Methamphetamine and amphetamine are often measured in urine or blood as part of a drug test for sports, employment, poisoning diagnostics, and forensics. | 11
|
q) arm of chromosome 11; inversion of chromosome 16 occurring between p13.3 and q24.3 denoted as inv(16)(p13.3q24.3) that results in the production of a CBFA2T3-GLIS2 fusion protein; and increases in chromosome numbers from a normal of 46 to anywhere from 47 to >50. The relationships of these and the many other genetic abnormalities detected in non-Down-AMKL to the diseases development require further investigations. Presentation
Non-DS-AMKL occurs in neonates, infants, and children of all ages. Except for the lack of Down syndrome, no history of TMD, and occurrences in children that can be >4 years of age, individuals with non-DS-AMKL present with many of the symptoms, signs, and hematological findings seen in DS-AMKL. However, non-DS-AMKL is a more aggressive and rapidly progressing disorder than DS-AMKL. Nonetheless, the presentation of non-DS-AMKL is also like DS-AMKL in that it is not often accompanied by one or more extramedullary signs or symptoms of the disease such as liver enlargement, spleen enlargement, leukemia cutis, and leukostasis. Diagnosis
The diagnosis of non-DS-AMKL is made in children who do not have Down syndrome but exhibit the same clinical symptoms, signs, hematological abnormalities, and specialized laboratory findings seen in DS-AMKL. These children should bear one or more of the genetic aberrations associated with the disease but not the inactivating GATA1 mutations, extra copies of chromosome 21 genes, or other genetic abnormalities associated with DS-AMKL. | Immunochemical analyses, often conducted by flow cytometry, of the surface antigens on leukemic blast cells are positive for CD41, CD42b, CD51, and Von Willebrand factor in AMKL but not leukemia involving non-platelet malignant cells.Where indicated and available, the diagnosis of DS-AMKL is further supported by; immunophenotyping analysis using monoclonal antibody directed against megakaryocyte restricted antigen (CD41 and CD61) and DNA sequencing to detect GATA1 mutations that are projected to cause the gene to make GATA1-S but not GATA1 transcription factors. Treatment
The chemotherapy regimens used for all types of AMKL are similar to those used for AML. A final confirmation of safety and efficacy phase 3 study consisted of 4 cycles of induction therapy with cytarabine and daunorubicin followed by a single course of intensification therapy consisting of cytarabine and L-asparaginase, and concluded with a central nervous system consolidation course of 3 additional doses of intrathecal cytarabine. The dosages of cytarabine in this study were kept low because DS-AMKL patients proved highly susceptible to the toxic effects of the regimen which used a higher cytarabine dosage to treat AML. The low-dose cytarabine regimen achieved excellent results in DS-AMKL with relatively reduced overall toxicity and is currently recommended as a preferred treatment regimen for the disease.Autologous hematopoietic stem cell transplantation (i.e. transplantation of stem cells derived from the individual being transplanted) did not improve relapse-free survival in one large study of DS-AMKL. Allogenic hematopoietic stem cell transplantation (i.e. | 11
|
They include pathology of the right hemisphere of the brain, substance abuse, arteriovenous malformations in the brain, and temporal lobe epilepsy.Social anxiety disorder (SAD) and ORS have some demographic and clinical similarities. Where the social anxiety and avoidance behavior is primarily focussed on concern about body odors, ORS is a more appropriate diagnosis than avoidant personality disorder or SAD. Body dismorphic disorder (BDD) has been described as the closest diagnosis in DSM-IV to ORS as both primarily focus on bodily symptoms. The defining difference between the two is that in BDD the preoccupation is with physical appearance, not body odors. Similarly, where obsessive behaviors are directly and consistently related to body odors rather than anything else, ORS is a more appropriate diagnosis than obsessive–compulsive disorder, in which obsessions are different and multiple over time.ORS may be misdiagnosed as schizophrenia. About 13% of people with schizophrenia have olfactory hallucinations. Generally, schizophrenic hallucinations are perceived as having an imposed, external origin, while in ORS they are recognized as originating from the individual. The suggested diagnostic criteria mean that the possibility of ORS is negated by a diagnosis of schizophrenia in which persistent delusions of an offensive body odor and olfactory hallucinations are contributing features for criterion A. However, some reported ORS cases were presented as co-morbid. Indeed, some have suggested that ORS may in time transform into schizophrenia, but others state there is little evidence for this. | It is also suggested to be a type of body dysmorphic disorder or, as it involves a single delusional belief, some suggest that ORS is a monosymptomatic hypochondriacal psychosis (hypochondriacal type of delusional disorder, see monothematic delusion).The World Health Organizations 10th revision of the International Statistical Classification of Diseases and Related Health Problems (ICD-10) does not have a specific entry for ORS, or use the term, but in the "persistent delusional disorders" section, states delusions can "express a conviction that others think that they smell. "ORS has also never been allocated a dedicated entry in any edition of the American Psychiatric Associations Diagnostic and Statistical Manual of Mental Disorders. In the third edition (DSM-III), ORS was mentioned under "atypical somatoform disorders". The revised third edition (DSM-III-R) mentions ORS in the text, stating that "convictions that the person emits a foul odor are one of the most common types of delusion disorder, somatic type." The fourth edition (DSM-IV), does not use the term ORS but again mentions such a condition under "delusional disorder, somatic type", stating "somatic delusions can occur in several forms. Most common are the persons conviction that he or she emits a foul odor from the skin, mouth, rectum or vagina." In the fifth edition (DSM-5), ORS again does not appear as a distinct diagnosis, but it is mentioned in relation to taijin kyōfushō (対人恐怖症, "disorder of fear of personal interaction"). | 11
|
The study is ongoing and preliminary results have been published in the New England Journal of Medicine.The full analysis of the COMET-ICE trial was published in JAMA and it showed that sotrovimab reduced risk of hospitalization for more than 24 hours or death by 79% compared to placebo (1% for sotrovoimab group and 6% for the placebo group). The trial involved 1057 participants and took place before the omicron variant was prevalent. Manufacturing
Sotrovimab is a biologic product which takes six months to manufacture in living cells. It is produced in Chinese hamster ovary cells. At product launch in May 2021, sotrovimabs active pharmaceutical ingredient was produced by WuXi Biologics in China and sent to a GlaxoSmithKline plant in Parma, Italy for further processing into the finished product. In January 2022, the spread of the SARS-CoV-2 Omicron variant began to render other monoclonal antibodies obsolete and caused global demand for sotrovimab to skyrocket. In response, Vir and GlaxoSmithKline announced they were working with Samsung Biologics on manufacturing sotrovimab at an additional site in South Korea. Society and culture
Economics
In 2021, the United States government agreed to purchase 1.5 million doses of the drug at $2,100 per dose. Legal status
In May 2021, the Committee for Medicinal Products for Human Use (CHMP) of the European Medicines Agency (EMA) completed its review on the use of sotrovimab for the treatment of COVID-19. | Sotrovimab, sold under the brand name Xevudy, is a human neutralizing monoclonal antibody with activity against severe acute respiratory syndrome coronavirus 2, known as SARS-CoV-2. It is under development by GlaxoSmithKline and Vir Biotechnology, Inc. Sotrovimab is designed to attach to the spike protein of SARS-CoV-2.The most common side effects include hypersensitivity (allergic) reactions and infusion-related reactions. Medical uses
In the European Union, sotrovimab is indicated for the treatment of COVID-19 in people aged twelve years of age and older and weighing at least 40 kilograms (88 lb) who do not require supplemental oxygen and who are at increased risk of the disease becoming severe.Sotrovimab is given by intravenous infusion, preferably within 5 days of onset of COVID-19 symptoms. Development and mechanism of action
Sotrovimabs development began in December 2019, at Vir Biotechnology when Vir scientists first learned of the initial COVID-19 outbreak in China. Vir subsidiary Humabs BioMed had already compiled a library of frozen blood samples from patients infected with viral diseases, including two samples from patients infected with SARS-CoV-1. Vir scientists obtained samples of the novel SARS-CoV-2 virus and mixed them with various antibodies recovered from the old SARS-CoV-1 blood samples. The objective was to identify antibodies effective against both SARS-CoV-1 and SARS-CoV-2. This would imply that the antibodies were targeting highly conserved sequences and in turn would be more likely to remain effective against future variants of SARS-CoV-2. | 11
|
Chronically fatigued, sufferers can become fatigued quickly with minimal exertion and some might experience neck and back pain. Other symptoms include the feeling of pressure in the brain, mostly around the frontal lobe area, headaches and/or migraine headaches, ophthalmodynia periodica (ice pick stabbing headaches in the early stages of MdDs), ear pain, ear fullness and possibly tinnitus.Fluctuations in weather also affect sufferers, in particularly hot weather and barometric pressure changes. Many have photo-sensitivity and find it more difficult to walk in the dark as well as other sensitivities to strong smells including chemical smells. Cognitive impairment ("brain fog") includes an inability to recall words, short term memory loss, an inability to multi-task, misspelling and mispronunciation of words, difficulty in concentrating. Many MdDS sufferers report they are unable to use a computer for any length of time due to the visual over-stimulation, and some are even unable to watch television.Symptoms can be increased by stress, lack of sleep, crowds, flickering lights, loud sounds, fast or sudden movements, enclosed areas and visual intolerance of busy patterns and scrolling movement.MdDS sufferers may have hypersomnia and can sleep up to 12 or more hours a day, depending on their symptom levels. Research reveals MdDS is not migraine-related and many sufferers have never had migraine symptoms prior to the onset of the disorder. | Discontinuation
The British National Formulary recommends a gradual withdrawal when discontinuing antipsychotics to avoid acute withdrawal syndrome or rapid relapse. Symptoms of withdrawal commonly include nausea, vomiting, and loss of appetite. Other symptoms may include restlessness, increased sweating, and trouble sleeping. Less commonly there may be a feeling of the world spinning, numbness, or muscle pains. Symptoms generally resolve after a short period of time.There is tentative evidence that discontinuation of antipsychotics can result in psychosis. It may also result in reoccurrence of the condition that is being treated. Rarely tardive dyskinesia can occur when the medication is stopped. Pharmacology
Pharmacodynamics
Perphenazine has the following binding profile towards cloned human receptors unless otherwise specified:
Acronyms:
RS — Rat striatum receptor. RB — Rat brain receptor. Pharmacokinetics
Perphenazine has an oral bioavailability of approximately 40% and a half-life of 8 to 12 hours (up to 20 hours), and is usually given in 2 or 3 divided doses each day. It is possible to give two-thirds of the daily dose at bedtime and one-third during breakfast to maximize hypnotic activity during the night and to minimize daytime sedation and hypotension without loss of therapeutic activity. Formulations
It is sold under the brand names Trilafon (single drug) and Etrafon/Triavil/Triptafen (contains fixed dosages of amitriptyline). A brand name in Europe is Decentan pointing to the fact that perphenazine is approximately 10-times more potent than chlorpromazine. Usual oral forms are tablets (2, 4, 8, 16 mg) and liquid concentrate (4 mg/ml). The Perphenazine injectable USP solution is intended for deep intramuscular (i.m.) | 0-1
|
Some of the treatments focus on attempting to change the sexual preference of pedophiles, while others focus on keeping pedophiles from committing child sexual abuse, or on keeping child sexual abusers from committing child sexual abuse again. Cognitive behavioral therapy (CBT), for example, aims to reduce attitudes, beliefs, and behaviors that may increase the likelihood of sexual offenses against children. Its content varies widely between therapists, but a typical program might involve training in self-control, social competence and empathy, and use cognitive restructuring to change views on sex with children. The most common form of this therapy is relapse prevention, where the patient is taught to identify and respond to potentially risky situations based on principles used for treating addictions.The evidence for cognitive behavioral therapy is mixed. A 2012 Cochrane Review of randomized trials found that CBT had no effect on risk of reoffending for contact sex offenders. Meta-analyses in 2002 and 2005, which included both randomized and non-randomized studies, concluded that CBT reduced recidivism. There is debate over whether non-randomized studies should be considered informative. More research is needed.Sexual abuse is associated with many sub-clinical behavioral issues as well, including re-victimization in the teenage years, a bipolar-like switching between sexual compulsion and shut-down, and distorted thinking on the subject of sexual abuse (for instance, that it is common and happens to everyone). | 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
|
Diagnosis
This disease can be diagnosed by a doctor with and without the use of lab testing. Lab tests are not always relied upon because treatment may be necessary before the results are returned.Abnormal laboratory findings seen in patients with Rocky Mountain spotted fever may include a low platelet count, low blood sodium concentration, or elevated liver enzyme levels. Serology testing and skin biopsy are considered to be the best methods of diagnosis. Although immunofluorescent antibody assays are considered some of the best serology tests available, most antibodies that fight against R. rickettsii are undetectable on serology tests during the first seven days after infection.Differential diagnosis includes dengue, leptospirosis, chikungunya, and Zika fever. Treatment
Appropriate antibiotic treatment should be started immediately when there is a suspicion of Rocky Mountain spotted fever. Treatment should not be delayed for laboratory confirmation of disease as early initiation of treatment of Rocky Mountain spotted fever is associated with lower mortality. Failure to respond to a tetracycline argues against a diagnosis of Rocky Mountain spotted fever. Severely ill people may require longer periods before their fever resolves, especially if they have experienced damage to multiple organ systems. Preventive therapy in healthy people who have had recent tick bites is not recommended and may only delay the onset of disease.Doxycycline (a tetracycline) is the drug of choice for patients with Rocky Mountain spotted fever. According to the CDC, "Doctors often avoid prescribing doxycycline to young children because of a warning that tooth staining may occur when used in children less than 8 years old. | In clinical trial 1, 44% of patients succeeded successful treatment of Desonate versus 14% treated with the placebo. In clinical trial 2, 28% of patients succeeded successful treatment of Desonate versus 6% treated with the placebo. The FDA approved Tridesilon for the following treatments: Contact Dermatitis, Rhus Dermatitis, Eczema, Dermatitis, Discoid Lupus Erythematosus, Granuloma Annulare, Seborrheic Dermatitis, Polymorphous Light Eruption, Pruritus, Psoriasis, Lichen Simplex, Atopic Dermatitis, Lichen Planus, Xerosis, Exfoliative Dermatitis.Recently, in late 2014, phase 3 clinical trials were completed to evaluate treatments using Desonide cream versus amino acid moisturizing cream. Patients with eczema or atopic dermatitis could be enrolled in this study, both male and female, over the age of 18. The randomized, double-blind, active control, 5-week study compared the effects of treatment of both creams through twice-a-day application. The clinical trial is sponsored by NeoStrata Company, Inc.No research has been completed to evaluate the carcinogenic or photoco-carcinogenic potential of desonide. Additionally, no research has been completed to determine the effect on fertility. These are current opportunities for improvement to learn more about the risks and benefits associated with desonide. Regulatory
The first US. patent for desonide was US4185100A was granted on January 22, 1980. The patent was for topical anti-inflammatory treatment using desonide. The patent defends the suitability of the anti-inflammatory for the treatment of cutaneous disorders or disruptions characterized by skin inflammation and/or hyperproliferative activity in the epidermis. Side effects and the mechanism of action are both disclosed. This patent is currently expired. == References == | 0-1
|
The majority of these variants are in regions that have not been previously implicated in coronary artery disease. The following genes have an association with MI: PCSK9, SORT1, MIA3, WDR12, MRAS, PHACTR1, LPA, TCF21, MTHFDSL, ZC3HC1, CDKN2A, 2B, ABO, PDGF0, APOA5, MNF1ASM283, COL4A1, HHIPC1, SMAD3, ADAMTS7, RAS1, SMG6, SNF8, LDLR, SLC5A3, MRPS6, KCNE2. Other
The risk of having a myocardial infarction increases with older age, low physical activity, and low socioeconomic status. Heart attacks appear to occur more commonly in the morning hours, especially between 6AM and noon. Evidence suggests that heart attacks are at least three times more likely to occur in the morning than in the late evening. Shift work is also associated with a higher risk of MI. And one analysis has found an increase in heart attacks immediately following the start of daylight saving time.Women who use combined oral contraceptive pills have a modestly increased risk of myocardial infarction, especially in the presence of other risk factors. The use of non-steroidal anti inflammatory drugs (NSAIDs), even for as short as a week, increases risk.Endometriosis in women under the age of 40 is an identified risk factor.Air pollution is also an important modifiable risk. Short-term exposure to air pollution such as carbon monoxide, nitrogen dioxide, and sulfur dioxide (but not ozone) have been associated with MI and other acute cardiovascular events. For sudden cardiac deaths, every increment of 30 units in Pollutant Standards Index correlated with an 8% increased risk of out-of-hospital cardiac arrest on the day of exposure. | The foot (PL: feet) is an anatomical structure found in many vertebrates. It is the terminal portion of a limb which bears weight and allows locomotion. In many animals with feet, the foot is a separate organ at the terminal part of the leg made up of one or more segments or bones, generally including claws or nails. Etymology
The word "foot", in the sense of meaning the "terminal part of the leg of a vertebrate animal" comes from "Old English fot "foot," from Proto-Germanic *fot (source also of Old Frisian fot, Old Saxon fot, Old Norse fotr, Danish fod, Swedish fot, Dutch voet, Old High German fuoz, German Fuß, Gothic fotus "foot"), from PIE root *ped- "foot". The "plural form feet is an instance of i-mutation." Structure
The human foot is a strong and complex mechanical structure containing 26 bones, 33 joints (20 of which are actively articulated), and more than a hundred muscles, tendons, and ligaments. The joints of the foot are the ankle and subtalar joint and the interphalangeal joints of the foot. An anthropometric study of 1197 North American adult Caucasian males (mean age 35.5 years) found that a mans foot length was 26.3 cm with a standard deviation of 1.2 cm.The foot can be subdivided into the hindfoot, the midfoot, and the forefoot:
The hindfoot is composed of the talus (or ankle bone) and the calcaneus (or heel bone). The two long bones of the lower leg, the tibia and fibula, are connected to the top of the talus to form the ankle. | 0-1
|
Sulfide has the highest affinity to either the pulsed or partially reduced states of the enzyme, and is capable of partially reducing the enzyme at the heme a3 center. It is unclear whether endogenous H2S levels are sufficient to inhibit the enzyme. There is no interaction between hydrogen sulfide and the fully reduced conformation of COX.Methanol in methylated spirits is converted into formic acid, which also inhibits the same oxidase system. High levels of ATP can allosterically inhibit cytochrome c oxidase, binding from within the mitochondrial matrix. Extramitochondrial and subcellular localizations
Cytochrome c oxidase has 3 subunits which are encoded by mitochondrial DNA (cytochrome c oxidase subunit I, subunit II, and subunit III). Of these 3 subunits encoded by mitochondrial DNA, two have been identified in extramitochondrial locations. In pancreatic acinar tissue, these subunits were found in zymogen granules. Additionally, in the anterior pituitary, relatively high amounts of these subunits were found in growth hormone secretory granules. The extramitochondrial function of these cytochrome c oxidase subunits has not yet been characterized. Besides cytochrome c oxidase subunits, extramitochondrial localization has also been observed for large numbers of other mitochondrial proteins. This raises the possibility about existence of yet unidentified specific mechanisms for protein translocation from mitochondria to other cellular destinations. Genetic defects and disorders
Defects involving genetic mutations altering cytochrome c oxidase (COX) functionality or structure can result in severe, often fatal metabolic disorders. Such disorders usually manifest in early childhood and affect predominantly tissues with high energy demands (brain, heart, muscle). | Mutation in this gene causing disorder of phototransduction pathway through brain which detect light fail to function. Diagnosis
Paediatric nurses, medical officers and paediatricians trained in eye screening could detect small or large eyeballs, nystagmus, strabismus, “white pupils” and birth defects like coloboma and aniridia. For pregnant women from family with history of congenital blindness will be closely monitor and need to carry out genetic test in order to identify is there mutation occur or not. Gene therapy treatment
Gene therapy treatment is done as an outpatient. Patients come to the hospital for the treatment, then return home. Patients do not need to be monitored strictly or stay in the hospital. The gene therapy treatment is in vivo which involves the use of a delivery vector to transmit the therapeutic gene into the targeted cells. The delivery vector uses a recombinant adeno-associated virus (AAV) carrying the RPE65 gene (AAV2-hRPE65v2). The procedure is a single injection of the AAV2-hRPE65v2 therapeutic gene into the unilateral subretinal of the eye. Gene therapy can only improve eye vision, but cannot cure the condition. The therapeutic gene, Voretigene neparvovec (Luxturna), was the first gene therapy approved by FDA for inherited diseases. == References == | 0-1
|
Azelastine, sold under the brand name Optivar among others, is a medication primarily used as a nasal spray to treat allergic rhinitis (hay fever) and as eye drops for allergic conjunctivitis. Other uses may include asthma and skin rashes for which it is taken by mouth. Onset of effects is within minutes when used in the eyes and within an hour when used in the nose. Effects last for up to 12 hours.Common side effects include headache, sleepiness, change in taste, and sore throat. It is unclear if use is safe during pregnancy or breastfeeding. It is a second-generation antihistamine and works by blocking the release of a number of inflammatory mediators including histamine.Azelastine was patented in 1971 and came into medical use in 1986. It is available as a generic medication. In 2019, it was the 167th most commonly prescribed medication in the United States, with more than 3 million prescriptions. Medical uses
Azelastine nasal spray is indicated for the local treatment of the symptoms of seasonal allergic rhinitis and perennial allergic rhinitis, such as rhinorrhea, sneezing and nasal pruritus in people five years of age and older. In some countries, it is also indicated for the treatment of vasomotor rhinitis in adults and children ≥ 12 years old. Azelastine eye drops are indicated for the local treatment of seasonal and perennial allergic conjunctivitis. Side effects
Azelastine is safe and well tolerated in both adults and children with allergic rhinitis. Bitter taste, headache, nasal burning and somnolence are the most frequently reported adverse events. | Drug Information Portal. U.S. National Library of Medicine. "Azelastine hydrochloride". Drug Information Portal. U.S. National Library of Medicine. | 11
|
Synthetic and wool fabrics are superior to cotton as they provide better insulation when wet and dry. Some synthetic fabrics, such as polypropylene and polyester, are used in clothing designed to wick perspiration away from the body, such as liner socks and moisture-wicking undergarments. Clothing should be loose fitting, as tight clothing reduces the circulation of warm blood. In planning outdoor activity, prepare appropriately for possible cold weather. Those who drink alcohol before or during outdoor activity should ensure at least one sober person is present responsible for safety.Covering the head is effective, but no more effective than covering any other part of the body. While common folklore says that people lose most of their heat through their heads, heat loss from the head is no more significant than that from other uncovered parts of the body. However, heat loss from the head is significant in infants, whose head is larger relative to the rest of the body than in adults. Several studies have shown that for uncovered infants, lined hats significantly reduce heat loss and thermal stress. Children have a larger surface area per unit mass, and other things being equal should have one more layer of clothing than adults in similar conditions, and the time they spend in cold environments should be limited. However children are often more active than adults, and may generate more heat. In both adults and children, overexertion causes sweating and thus increases heat loss.Building a shelter can aid survival where there is danger of death from exposure. | Myhre syndrome is a rare genetic disorder inherited in an autosomal dominant fashion. It is caused by mutation in SMAD4 gene. Signs and symptoms
The clinical presentation is variable but includes
developmental and growth delay
athletic muscular built
skeletal anomalies
joint stiffness
characteristic facial appearance
deafness
variable cognitive deficits
tracheal stenosis
aortic stenosis
pyloric stenosisThe facial abnormalities include:
blepharophimosis (an abnormally narrow gap between the upper and lower eyelids)
maxillary hypoplasia (underdevelopment of the upper jaw)
prognathism (prominent lower jaw)The skeletal abnormalities include:
short stature
square body shape
broad ribs
iliac hypoplasia
brachydactyly
flattened vertebrae
thickened calvariaCongenital heart disease and undescended testes have also been reported in association with this syndrome. Genetics
Myhre syndrome is due to mutations in the SMAD4 gene. This gene encodes a protein - transducer mediating transforming growth factor beta. Some researchers believe that the SMAD4 gene mutations that cause Myhre syndrome impair the ability of the SMAD4 protein to attach (bind) properly with the other proteins involved in the signaling pathway. Other studies have suggested that these mutations result in an abnormally stable SMAD4 protein that remains active in the cell longer. Changes in SMAD4 binding or availability may result in abnormal signaling in many cell types, which affects development of several body systems and leads to the signs and symptoms of Myhre syndrome.The patients of this disease exhibit hypertrophic phenotype in their muscle tissues. Myostatin target genes are found to be downregulated while bone morphogenetic protein (BMP) target genes display both upregulated and downregulated genotypes. Diagnosis
Treatment
History
This disorder was first reported in 1981. It has many similarities to LAPS Syndrome and they both arise from the same mutations in the SMAD4 gene. | 0-1
|
Plica syndrome is a condition that occurs when a plica (a vestigial extension of the protective synovial capsule of usually the knee) becomes irritated, enlarged, or inflamed. Cause
This inflammation is typically caused by the plica being caught on the femur, or pinched between the femur and the patella. The most common location of plica tissue is along the medial (inside) side of the knee. The plica can tether the patella to the femur, be located between the femur and patella, or be located along the femoral condyle. If the plica tethers the patella to the femoral condyle, the symptoms may cause it to be mistaken for chondromalacia. The plica themselves are remnants of the fetal stage of development where the knee is divided into three compartments. The plica normally diminish in size during the second trimester of fetal development, as the three compartments develop into the synovial capsule. In adults, they normally exist as sleeves of tissue called synovial folds. The plica are usually harmless and unobtrusive; plica syndrome only occurs when the synovial capsule becomes irritated, which thickens the plica themselves (making them prone to irritation/inflammation, or being caught on the femur). Diagnosis
If the plica tethers the patella to the femoral condyle, the symptoms may cause it to be mistaken for chondromalacia patellae. Diagnosis is often complicated by the thin structures of plicae, fenestrated septum or unfenestrated septum all being too fine to resolve well even in MRI. Treatment
Plica syndrome treatment focuses on decreasing inflammation of the synovial capsule. | A nonsteroidal anti-inflammatory drug (NSAID) is often used in conjunction with therapeutic exercise and modalities. Iontophoresis and phonophoresis have been utilized successfully against inflammation of the plica and synovial capsule. Failing these, surgical removal of the plica of the affected knee may be necessary. See also
Knee pain
Patellofemoral pain syndrome
Iliotibial band syndrome
References
== External links == | 11
|
Pernicious anemia is a type of vitamin B12 deficiency anemia, a disease in which not enough red blood cells are produced due to the malabsorption of vitamin B12. Malabsorption in pernicious anemia results from the lack or loss of intrinsic factor needed for the absorption of vitamin B12. Anemia is defined as a condition in which the blood has a lower than normal amount of red blood cells or hemoglobin. The disease may come on slowly and insidiously.The most common initial symptoms are tiredness, and weakness. Other signs and symptoms of anemia include breathlessness, dizziness, a sore red tongue, lightheadedness, headaches, poor ability to exercise, cold hands and feet, low blood pressure, pale or yellow skin, chest pain, and an irregular heartbeat. The digestive tract may also be disturbed giving symptoms that can include nausea and vomiting, heartburn, upset stomach and loss of appetite. Pernicious anemia can cause osteoporosis and may lead to bone fractures. Symptoms of severe vitamin B12 deficiency can include tingling or numbness in the hands and feet, memory problems, blurred vision, unsteady walking, poor balance, muscle weakness, impaired sense of taste and smell, poor reflexes, clumsiness, depression, and confusion. Without treatment, some of these problems may become permanent.Pernicious anemia occurs due to an autoimmune response that produces antibodies that attack the parietal cells in the stomach lining and prevents them from creating intrinsic factor. | A further important advance was made in the early 1960s by Doniach with the recognition that pernicious anemia is an autoimmune disease. Pernicious anemia is eventually treated with either injections or large oral doses of B12, typically between 1 and 4 mg daily. A medical author takes the view that Mary Todd Lincoln, the wife of American President Abraham Lincoln, had pernicious anemia for decades and died from it in 1882. Research
Permeation enhancers
Treatment using oral drugs is an easier option in management but the bioavailabity of B12 is low. This is due to low absorption in the intestine, and breakdown by enzyme activity. Research continues to focus on the use of permeation enhancers or permeation absorbers in combination with the treatment. One of the better performing enhancers studied is salcoprozate sodium (SNAC). SNAC is able to form a noncovalent complex with cobalamin while preserving its chemical integrity and protect B12 from gastric acidity. This complex is much more lipophilic than the water-soluble vitamin B12, so is able to pass through cellular membranes with greater ease. Molecular dynamics are used in experiments to gain an understanding of the molecular interactions involved in the different molecules used and the degree of ease achieved in absorption across the gastric epithelium. References
External links
Pernicious anemia at Curlie | 11
|
Orofaciodigital syndrome or oral-facial-digital syndrome is a group of at least 13 related conditions that affect the development of the mouth, facial features, and digits in between 1 in 50,000 to 250,000 newborns with the majority of cases being type I (Papillon-League-Psaume syndrome). Type
The different types are:s
Type I, Papillon-League-Psaume syndrome
Type II, Mohr syndrome
Type III, Sugarman syndrome
Type IV, Baraitser-Burn syndrome
Type V, Thurston syndrome
Type VI, Varadi-Papp syndrome
Type VII, Whelan syndrome
Type VIII, Oral-facial-digital syndrome, Edwards type (not to be confused with Edwards syndrome)
Type IX, OFD syndrome with retinal abnormalities
Type X, OFD with fibular aplasia
Type XI, Gabrielli syndrome
References
== External links == | Further it states the need for population based studies including both non-atypical and atypical hyperplasia to accurately estimate the risk of progression to cancer.If untreated with hysterectomy, endometrial hyperplasia progresses to adenocarcinoma within 20 years in:
28% of cases with atypia (95% CI, 8.6% to 42.5%), and
about 5% of cases without atypia.The rates are more favorable in cases with simple rather than complex hyperplasia, but as mentioned above this terminology was phased out of the WHO classification in 2014. In patients with samples showing atypia, carcinoma is already present in over 40% of cases. Given this, the aforementioned 28% atypia progression rate may be an underestimate, and the true number may closer to the 42.5% part of the studys remarkably wide confidence interval. Treatment
Treatment of endometrial hyperplasia is individualized, and may include hormonal therapy, such as cyclic or continuous progestin therapy, or hysterectomy. See also
Endometrial intraepithelial neoplasia
Endometrial carcinoma
Hyperplasia
References
== External links == | 0-1
|
There is a small amount of evidence that EMDR may improve re-experiencing symptoms in children and adolescents, but EMDR has not been shown to improve other PTSD symptoms, anxiety, or depression.The eye movement component of the therapy may not be critical for benefit. As there has been no major, high quality randomized trial of EMDR with eye movements versus EMDR without eye movements, the controversy over effectiveness is likely to continue. Authors of a meta-analysis published in 2013 stated, "We found that people treated with eye movement therapy had greater improvement in their symptoms of post-traumatic stress disorder than people given therapy without eye movements.... Secondly we found that in laboratory studies the evidence concludes that thinking of upsetting memories and simultaneously doing a task that facilitates eye movements reduces the vividness and distress associated with the upsetting memories." Interpersonal psychotherapy
Other approaches, in particular involving social supports, may also be important. An open trial of interpersonal psychotherapy reported high rates of remission from PTSD symptoms without using exposure. A current, NIMH-funded trial in New York City is now (and into 2013) comparing interpersonal psychotherapy, prolonged exposure therapy, and relaxation therapy. Medication
While many medications do not have enough evidence to support their use, four (sertraline, fluoxetine, paroxetine, and venlafaxine) have been shown to have a small to modest benefit over placebo. With many medications, residual PTSD symptoms following treatment is the rule rather than the exception. Antidepressants
Selective serotonin reuptake inhibitors (SSRIs) and serotonin-norepinephrine reuptake inhibitors (SNRIs) may have some benefit for PTSD symptoms. | Experiencing an enemy firefight was associated with an 18.3 percentage point increase in the probability of PTSD, while being wounded or injured in combat was associated with a 23.9 percentage point increase in the likelihood of a PTSD diagnosis. For the 2.16 million U.S. troops deployed in combat zones between 2001 and 2010, the total estimated two-year costs of treatment for combat-related PTSD are between $1.54 billion and $2.69 billion.As of 2013, rates of PTSD have been estimated at up to 20% for veterans returning from Iraq and Afghanistan. As of 2013 13% of veterans returning from Iraq were unemployed. Man-made disasters
The September 11 attacks took the lives of nearly 3,000 people, leaving 6,000 injured. First responders (police, firefighters, and emergency medical technicians), sanitation workers, and volunteers were all involved in the recovery efforts. The prevalence of probable PTSD in these highly exposed populations was estimated across several studies using in-person, telephone, and online interviews and questionnaires. Overall prevalence of PTSD was highest immediately following the attacks and decreased over time. However, disparities were found among the different types of recovery workers. The rate of probable PTSD for first responders was lowest directly after the attacks and increased from ranges of 4.8-7.8% to 7.4-16.5% between the 5-6 year follow-up and a later assessment. When comparing traditional responders to non-traditional responders (volunteers), the probable PTSD prevalence 2.5 years after the initial visit was greater in volunteers with estimates of 11.7% and 17.2% respectively. | 11
|
Such subsidies are subject to conditions, however. In Belgium, a fixed payment of €1,073 is made for each full cycle of the IVF process. The woman must be aged under 43 and may not carry out more than six cycles of ART. There is also a limit on the number of transferable embryos, which varies according to age and the number of cycles completed. In France, ART is subsidized in full by national health insurance for women up to age 43, with limits of 4 attempts at IVF and 6 at artificial insemination. Germany tightened its conditions for public funding in 2004, which caused a sharp drop in the number of ART cycles carried out, from more than 102,000 in 2003 to fewer than 57,000 the following year. Since then the figure has remained stable. 17 countries limit access to ART according to the age of the woman. 10 countries have established an upper age limit, varying from 40 (Finland, Netherlands) to 50 (including Spain, Greece and Estonia). Since 1994, France is one of a number of countries (including Germany, Spain, and the UK) which use the somewhat vague notion of "natural age of procreation". In 2017, the steering council of Frances Agency of Biomedicine established an age limit of 43 for women using ART. 10 countries have no age limit for ART. These include Austria, Hungary, Italy and Poland. Most European countries allow donations of gametes by third parties. But the situations vary depending on whether sperm or eggs are concerned. | This has the potential to give ART providers a difficult decision of whether to continue or refuse treatment.Some assisted reproductive technologies have the potential to be harmful to both the mother and child, posing a psychological and/or physical health risk, which may impact the ongoing use of these treatments. In Israel, there is research supporting using art, including recycled lab materials from the IVF process, to help women work through some of these mixed emotions. Fictional representation
Films and other fiction depicting emotional struggles of assisted reproductive technology have had an upswing in the latter part of the 2000s decade, although the techniques have been available for decades. As ART becomes more utilized, the number of people that can relate to it by personal experience in one way or another is growing.For specific examples, refer to the fiction sections in individual subarticles, e.g. surrogacy, sperm donation and fertility clinic. In addition, reproduction and pregnancy in speculative fiction has been present for many decades. Historical facts
25 July 1978, Louise Brown was born; this was the first successful birth of a child after IVF treatment. The procedure took place at Dr Kershaws Cottage Hospital (now Dr Kershaws Hospice) in Royton, Oldham, England. Patrick Steptoe (gynaecologist) and Robert Edwards (physiologist) worked together to develop the IVF technique. Steptoe described a new method of egg extraction and Edwards were carrying out a way to fertilise eggs in the lab. | 11
|
Players may play many hours per day, neglect personal hygiene, gain or lose significant weight, disrupt sleep patterns resulting in sleep deprivation, play at work, avoid phone calls from friends, or lie about how much time they spend playing video games.The APA has developed nine criteria for characterising the proposed Internet Gaming Disorder:
Pre-occupation. Do you spend a lot of time thinking about games even when you are not playing, or planning when you can play next? Withdrawal. Do you feel restless, irritable, moody, angry, anxious or sad when attempting to cut down or stop gaming, or when you are unable to play? Tolerance. Do you feel the need to play for increasing amounts of time, play more exciting games, or use more powerful equipment to get the same amount of excitement you used to get? Reduce/stop. Do you feel that you should play less, but are unable to cut back on the amount of time you spend playing games? Give up other activities. Do you lose interest in or reduce participation in other recreational activities due to gaming? Continue despite problems. Do you continue to play games even though you are aware of negative consequences, such as not getting enough sleep, being late to school/work, spending too much money, having arguments with others, or neglecting important duties? Deceive/cover up. Do you lie to family, friends or others about how much you game, or try to keep your family or friends from knowing how much you game? Escape adverse moods. | However, the clinical trials of potential treatments remain of low quality, except for cognitive-behavioral therapies, which shows efficacy to reduce gaming disorder and depressive symptoms but not total time spent. Although there is a scientific consensus that cognitive-behavioral therapy is preferable to pharmacological treatment, it remains difficult to make definitive statements about its benefits and efficiency due to methodological inconsistencies and lack of follow-up. Since efficacious treatments have not been well established, prevention of video gaming disorder is crucial. Some evidence suggest that up to 50% of people affected by the internet gaming disorder may recover naturally.Some countries, such as South Korea, China, the Netherlands, Canada, and the United States, have responded to the perceived threat of video game addiction by opening treatment centres. China
China was the first country to treat "internet addiction" clinically in 2008. The Chinese government operates several clinics to treat those who overuse online games, chatting and web surfing. Treatment for the patients, most of whom have been forced to attend by parents or government officials, includes various forms of pain including shock therapy. In August 2009, Deng Sanshan was reportedly beaten to death in a correctional facility for video game and Web addiction. Most of the addiction "boot camps" in China are actually extralegal militaristically managed centers, but have remained popular despite growing controversy over their practices.In 2019, China set up a curfew, banning minors from playing between certain hours. | 11
|
A follow-up study ten years later discovered that many had died from "unknown causes". Congenital Minamata disease
Local doctors and medical officials had noticed for a long time an abnormally high frequency of cerebral palsy and other infantile disorders in the Minamata area. In 1961, a number of medical professionals, including Masazumi Harada (later to be honored by the United Nations for his body of work on Minamata disease), set about re-examining children diagnosed with cerebral palsy. The symptoms of the children closely mirrored those of adult Minamata disease patients, but many of their mothers did not exhibit symptoms. The fact that these children had been born after the initial outbreak and had never been fed contaminated fish also led their mothers to believe they were not victims. At the time the medical establishment believed the placenta would protect the foetus from toxins in the bloodstream, which is indeed the case with most chemicals. What was not known at the time was that exactly the opposite is the case with methylmercury: the placenta removes it from the mothers bloodstream and concentrates the chemical in the foetus. After several years of study and the autopsies of two children, the doctors announced that these children had an as-yet unrecognised congenital form of Minamata disease. The certification committee convened on 29 November 1962 and agreed that the two dead children and the sixteen children still alive should be certified as patients, and therefore liable for "sympathy" payments from Chisso, in line with the 1959 agreement. | 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. | 11
|
Individuals with advanced HIV-1 disease, a history of peripheral neuropathy, or individuals on other drugs that have association with neuropathy develop this side effect more often.Stavudine has been shown in laboratory test to be genotoxic, but with clinical doses its carcinogenic effects are non-existent. Hyperlactatemia, bone mineral density (BMD) loss, reduction in limb fat and an increase in triglycerides were found when administered in high dosages. It is also one of the most likely antiviral drugs to cause lipodystrophy, and for this reason it is no longer considered an appropriate treatment for most patients in developed countries. HLA-B*4001 may be used as a genetic marker to predict which patients will develop stavudine-associated lipodystrophy, to avoid or shorten the duration of stavudine according to a study in Thailand.It is still used as first choice in first line therapy in resource poor settings such as in India. Only in case of development of peripheral neuropathy or pregnancy is it changed to the next choice, zidovudine. Safety and effectiveness of dosage titration was not reported in treatment naive patients. It was only reported in those patients with sustained virologic suppression. These findings are not generalized to Stavudine used in ART naive patients who have high viral loads. On Monday 30 November 2009, the World Health Organization stated that "[The WHO] recommends that countries phase out the use of Stavudine, or d4T, because of its long-term, irreversible side-effects. Stavudine is still widely used in first-line therapy in developing countries due to its low cost and widespread availability. | Infantile acropustulosis is an intensely itchy vesicopustular eruption of the hands and feet. : 205 Involvement of scabies has been suggested.infantile acropustulosis is characterized by itchy papules and vesicles that are similar to those found in scabies "mosquito like bites" but there is absence of the typical burrowing with S like burrows on the skin and can occur in small babies as opposed to scabies mostly found on children and young adults. See also
Acropustulosis
List of cutaneous conditions
References
== External links == | 0-1
|
Fibrothorax is a medical condition characterised by severe scarring (fibrosis) and fusion of the layers of the pleural space surrounding the lungs resulting in decreased movement of the lung and ribcage. The main symptom of fibrothorax is shortness of breath. There also may be recurrent fluid collections surrounding the lungs. Fibrothorax may occur as a complication of many diseases, including infection of the pleural space known as an empyema or bleeding into the pleural space known as a haemothorax.Fibrosis in the pleura may be produced intentionally using a technique called pleurodesis to prevent recurrent punctured lung (pneumothorax), and the usually limited fibrosis that this produces can rarely be extensive enough to lead to fibrothorax. The condition is most often diagnosed using an X-ray or CT scan, the latter more readily detecting mild cases. Fibrothorax is often treated conservatively with watchful waiting but may require surgery. The outlook is usually good as long as there is no underlying pulmonary fibrosis or complications following surgery. The disease is highly uncommon. Signs and symptoms
Signs
Reduced movement of the ribcage during breathing, reduced breath sounds on the affected side(s), and a dull feeling when the chest is pressed are common signs observed during examination for fibrothorax. Sharp chest pain with deep breaths or coughing may be seen in some cases. Severe cases of fibrothorax can lead to respiratory failure due to inadequate ventilation and cause abnormally high levels of carbon dioxide in the bloodstream. Symptoms
The condition only causes symptoms if the visceral pleura is affected. | Tobacco smoking cessation is strongly recommended since tobacco smoke exposure can worsen fibrosis. Severe cases of fibrothorax may require supportive mechanical ventilation if the affected person is unable to breathe adequately on their own.In cases of fibrothorax caused by medication, it is recommended that the offending medications be stopped. Ergot alkaloid medications, which can worsen pleural fibrosis, are typically avoided. Cases of fibrothorax attributable to medication typically stop worsening if the provoking medication is stopped. In some situations, medication-induced fibrothorax improves after stopping the causative medication but fibrothorax usually does not completely resolve.Watchful waiting is appropriate for milder cases of fibrothorax in certain situations. Fibrothorax caused by tuberculosis, empyema, or haemothorax often improves spontaneously 3–6 months after the precipitating illness. Corticosteroids are commonly used to treat fibrothorax but are not well-supported by available evidence. Surgical
In severe cases of fibrothorax that are compromising a persons ability to breathe, the scar tissue (fibrous peel) causing fibrothorax can be surgically removed using a technique called decortication. However, surgical decortication is an invasive procedure which carries the risk of complications including a small risk of death, and is therefore generally only considered if severe symptoms are present and have been for many months. Surgical decortication is generally considered for people with fibrothoraces that are severe, causing significant shortness of breath, and have otherwise relatively healthy lungs since this enhances the likelihood of a better outcome. Surgical removal of the pleura (pleurectomy) may be performed in refractory cases, as often happens when asbestosis is the cause. | 11
|
Ethambutol (EMB, E) is a medication primarily used to treat tuberculosis. It is usually given in combination with other tuberculosis medications, such as isoniazid, rifampicin and pyrazinamide. It may also be used to treat Mycobacterium avium complex, and Mycobacterium kansasii. It is taken by mouth.Common side effects include problems with vision, joint pain, nausea, headaches, and feeling tired. Other side effects include liver problems and allergic reactions. It is not recommended in people with optic neuritis, significant kidney problems, or under the age of five. Use during pregnancy or breastfeeding has not been found to cause harm. In the United States the FDA has raised concerns about eye issues in the baby if used during pregnancy. Ethambutol is believed to work by interfering with the bacterias metabolism.Ethambutol was discovered in 1961. It is on the World Health Organizations List of Essential Medicines and is available as a generic medication. Chirality and biological activity
(S,S)-(+)-Ethambutol is powerful and selective antitubercular drug. It is a typical example of an old drug that was introduced for clinical use in its unichiral form. Ethambutol contains two constitutionally symmetrical chiral centers in its structure and exists in three stereoisomeric forms. An enantiomeric pair (S,S)- and (R,R)-ethmabutol, along with the achiral stereoisomer called meso-form. The (S,S)-(+)-enantiomer harbors the antitubercular activity. This enantiomer is 500 and 12 fold more potent than the (R,R)-ethmabutol and the meso-form respectively. On the other hand, all the three isomers are equipotent in terms of the major side-effect of the drug, optic neuritis. | Toxicity is associated to both dose and duration of treatment. Hence the use of (S,S)-enantiomer greatly improved the risk/benefit balance. Medical uses
Ethambutol is used along with other medications to treat a number of infections including: tuberculosis, Mycobacterium avium complex, and Mycobacterium kansasii. Adverse effects
Optic neuritis (hence contraindicated in children below six years of age)
Red-green color blindness People taking ethambutol should be monitored for changes in visual acuity and color discrimination. Arthralgia
Hyperuricaemia
Vertical nystagmus
Milk skin reaction
Mechanism of action
Ethambutol is bacteriostatic against actively growing TB bacilli. It works by obstructing the formation of cell wall. Mycolic acids attach to the 5-hydroxyl groups of D-arabinose residues of arabinogalactan and form mycolyl-arabinogalactan-peptidoglycan complex in the cell wall. It disrupts arabinogalactan synthesis by inhibiting the enzyme arabinosyl transferase. Disruption of the arabinogalactan synthesis inhibits the formation of this complex and leads to increased permeability of the cell wall. Pharmacokinetics
It is well absorbed from the gastrointestinal tract and well distributed in body tissues and fluids. 50% is excreted unchanged in urine. See also
chiral drugs
chirality
enantiopure drug
stereochemistry
References
External links
"Ethambutol". Medicine Plus. "Ethambutol". Drug Information Portal. U.S. National Library of Medicine. | 11
|
Pseudoexfoliation syndrome, often abbreviated as PEX and sometimes as PES or PXS, is an aging-related systemic disease manifesting itself primarily in the eyes which is characterized by the accumulation of microscopic granular amyloid-like protein fibers. Its cause is unknown, although there is speculation that there may be a genetic basis. It is more prevalent in women than men, and in persons past the age of seventy. Its prevalence in different human populations varies; for example, it is prevalent in Scandinavia. The buildup of protein clumps can block normal drainage of the eye fluid called the aqueous humor and can cause, in turn, a buildup of pressure leading to glaucoma and loss of vision (pseudoexfoliation glaucoma, exfoliation glaucoma). As worldwide populations become older because of shifts in demography, PEX may become a matter of greater concern. Signs and symptoms
Patients may have no specific symptoms. In some cases, patients may complain of lessened visual acuity or changes in their perceived visual field, and such changes may be secondary to or different from symptoms normally associated with cataracts or glaucoma.PEX is characterized by tiny microscopic white or grey granular flakes which are clumps of proteins within the eye which look somewhat like dandruff when seen through a microscope and which are released by cells. The abnormal flakes, sometimes compared to amyloid-like material, are visible during an examination of the lens of an eye by an ophthalmologist or optometrist, which is the usual diagnosis. | These entities must be clinically excluded. Clinical condition characterized by ovarian mass, ascites, and right-sided pleural effusion. Ovarian malignancy and the other causes of pelvic mass, ascites, and pleural effusion to be considered, History of early satiety, weight loss with increased abdominal girth, bloating, intermittent abdominal pain, dyspnea, nonproductive cough may help in differentiating potential local factor causing such symptoms. Treatment
Treatment of Meigs syndrome consists of thoracentesis and paracentesis to drain off the excess fluid (exudate), and unilateral salpingo-oophorectomy or wedge resection to correct the underlying cause. Eponym
Meigs syndrome is named for Joe Vincent Meigs. References
== External links == | 0-1
|
Mechanism of action
Recently, it has been shown that trabectedin blocks DNA binding of the oncogenic transcription factor FUS-CHOP and reverses the transcriptional program in myxoid liposarcoma. By reversing the genetic program created by this transcription factor, trabectedin promotes differentiation and reverses the oncogenic phenotype in these cells.Other than transcriptional interference, the mechanism of action of trabectedin is complex and not completely understood. The compound is known to bind and alkylate DNA at the N2 position of guanine. It is known from in vitro work that this binding occurs in the minor groove, spans approximately three to five base pairs and is most efficient with CGG sequences. Additional favorable binding sequences are TGG, AGC, or GGC. Once bound, this reversible covalent adduct bends DNA toward the major groove, interferes directly with activated transcription, poisons the transcription-coupled nucleotide excision repair complex, promotes degradation of RNA polymerase II, and generates DNA double-strand breaks. Society and culture
Legal status
In September 2020, the European Medicines Agency recommended that the use of trabectedin in treating ovarian cancer remain unchanged. == References == | Progesterone helps the embryo implant by assisting passage through the fallopian tubes. It also affects the fallopian tubes and the uterus by stimulating an increase in secretions necessary for fetal nutrition. Progesterone, like hCG, is necessary to prevent spontaneous abortion because it prevents contractions of the uterus and is necessary for implantation. Estrogen is a crucial hormone in the process of proliferation. This involves the enlargement of the breasts and uterus, allowing for growth of the fetus and production of milk. Estrogen is also responsible for increased blood supply towards the end of pregnancy through vasodilation. The levels of estrogen during pregnancy can increase so that they are thirty times what a non-pregnant woman mid-cycles estrogen level would be. Human placental lactogen (hPL) is a hormone used in pregnancy to develop fetal metabolism and general growth and development. Human placental lactogen works with growth hormone to stimulate Insulin-like growth factor production and regulating intermediary metabolism. In the fetus, hPL acts on lactogenic receptors to modulate embryonic development, metabolism and stimulate production of IGF, insulin, surfactant and adrenocortical hormones. hPL values increase with multiple pregnancies, intact molar pregnancy, diabetes and Rh incompatibility. They are decreased with toxemia, choriocarcinoma, and Placental insufficiency. Immunological barrier
The placenta and fetus may be regarded as a foreign body inside the mother and must be protected from the normal immune response of the mother that would cause it to be rejected. The placenta and fetus are thus treated as sites of immune privilege, with immune tolerance. | 0-1
|
Nutcracker esophagus, Jackhammer esophagus, or hypercontractile peristalsis, is a disorder of the movement of the esophagus characterized by contractions in the smooth muscle of the esophagus in a normal sequence but at an excessive amplitude or duration. Nutcracker esophagus is one of several motility disorders of the esophagus, including achalasia and diffuse esophageal spasm. It causes difficulty swallowing, or dysphagia, to both solid and liquid foods, and can cause significant chest pain; it may also be asymptomatic. Nutcracker esophagus can affect people of any age but is more common in the sixth and seventh decades of life. The diagnosis is made by an esophageal motility study (esophageal manometry), which evaluates the pressure of the esophagus at various points along its length. The term "nutcracker esophagus" comes from the finding of increased pressures during peristalsis, with a diagnosis made when pressures exceed 180 mmHg; this has been likened to the pressure of a mechanical nutcracker. The disorder does not progress, and is not associated with any complications; as a result, treatment of nutcracker esophagus targets control of symptoms only. Signs and symptoms
Nutcracker esophagus is characterized as a motility disorder of the esophagus, meaning that it is caused by abnormal movement, or peristalsis of the esophagus. People with motility disorders present with two main symptoms: chest pain or difficulty with swallowing. Chest pain is the more common. The chest pain is very severe and intense, and mimics cardiac chest pain. It may spread into the arm and back. | The Gothenburg criterion consists of the presence of peristaltic contractions, with an amplitude of 180 mm Hg at any place in the esophagus. The Richter criterion involves the presence of peristaltic contractions with an amplitude of greater than 180 mm Hg from an average of measurements taken 3 and 8 cm above the lower esophageal sphincter. It has been incorporated into a number of clinical guidelines for the evaluation of dysphagia. The Achem criteria are more stringent, and are an extension of the study of 93 patients used by Richter and Castell in the development of their criteria, and require amplitudes of greater than 199 mm Hg at 3 cm above the lower esophageal sphincter (LES), greater than 172 mm Hg at 8 cm above the LES, or greater than 102 mm Hg at 13 cm above the LES. Treatment
People are usually reassured that the disease is unlikely to worsen. However, the symptoms of chest pain and trouble swallowing may be severe enough to require treatment with medications, and rarely, surgery. The initial step of treatment focuses on reducing risk factors. While weight reduction may be useful in reducing symptoms, the role of acid suppression therapy to reduce esophageal reflux is still uncertain. Very cold and very hot beverages may trigger esophageal spasms. Medications
Medications for nutcracker esophagus includes the use of calcium-channel blockers, which relax the lower esophageal sphincter (LES) and palliate the dysphagia symptoms. Diltiazem, a calcium-channel blocker, has been used in randomized control studies with good effect. | 11
|