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Increases and/or decreases in the genes expression regulate the progression of these immature cells toward their final forms as blood cells such as erythrocytess, certain types of lymphocytes, monocytes, and platelets) as well as certain types of tissue cells such as macrophages and mast cells. The GATA2 gene is also expressed in human endothelium, certain types of non-hematological stem cells, and, to lesser extents, prostate, endometrium, and some cancerous tissues.Monosomy of chromosome 7 (i.e. lose of one of the two chromosomes 7) or deletion of the "q" (i.e. short) in one of these two chromosomes often occurs in the various GATA2 deficiency manifestations including the Emberger syndrome. These genetic abnormalities are known causes of acute myeloid leukemia and, while not essential for, may contribute to the development of acute myeloid leukemia in the syndrome by, for example, lowering the age and/or increasing the chances of the disorder evolving into acute myeloid leukemia. GATA2 transcription factor
The GATA2 transcription factor contains two zinc finger (i.e. ZnF) structural motifs. C-ZnF is located toward the proteins C-terminus and is responsible for binding to specific DNA sites. N-ZnF is located toward the proteins N-terminus and is responsible for interacting with various other nuclear proteins that regulate its activity. | The transcription factor also contains two transactivation domains and one negative regulatory domain which interact with nuclear proteins to up-regulate and down-regulate, respectively, its activity.GATA2 binds to a specific nucleic acid sequence viz., (T/A(GATA)A/G) on the promoter and enhancer sites of its target genes and in doing so either stimulates or suppresses these genes expression. However, there are thousands of sites in human DNA with this nucleotide sequence but, for unknown reasons, GATA2 binds to <1% of these. Furthermore, all members of the GATA transcription factor family bind to this same nucleotide sequence and in doing so may interfere with GATA2 binding or even displace GATA2 already bound to these sites. For example, the displacement of GATA2 bond to this sequence by GATA1 appears important for the normal development of certain hematological stem cells. This phenomenon is termed the "GATA switch". Given these many variables, the GATA2 transcription factors actions in promoting or inhibiting its target genes is exceedingly complex and not completely understood. Pathophysiology
The GATA2 transcription factor is critical for the emergence of hematologic stem cells from the hemogenic endothelium during embryogenesis. Deletion of both Gata2 genes in mice is lethal by about day 10 of embryogenesis due to collapse in the formation of mature blood cells. Inactivation of one mouse Gata2 gene is neither lethal nor associated with most of the signs of human GATA2 deficiency except that these animals have ~50% reduction in their hematologic stem cells. | 11
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It is implied that individuals will drink more in a given setting, or on average because they are less receptive towards negative social attitudes surrounding excessive consumption. This however acts on another component, by where individualism protects from maladaptive consumption by lowering the need to drink socially. The final axis by which individualism protects from abusive consumption is that it promotes higher degrees of individualization and achievement values which promote personally suited rewards, this allow the individual to be more cognizant of potential alcohol abuse, and therefore protect from damaging mentalities in those who already identify as drinkers.Alcohol abuse also has a variety of biosocial implications, such as the physiologically effects of a detox, how the detox period interacts with ones social life and how these interactions can make beating alcoholism a complex, difficult process. Alcohol abuse can lead to a number of physical issues and may even create a mental health condition, leading to a double classification for the alcoholic. The stress, the social perceptions of these issues may reinforce abusive drinking habits. Alcoholism is the most severe form of alcohol dependence and abuse, it is often in fact a complicated hereditary disease. While it can run it families this is only a correlation and does not prove that the inherited factors play a role. Genetic influences on its genesis are suggested by numerous different sources of evidence. Alcoholism among adopted people has a stronger correlation with their biological parents than with their adopted parents, according to adoption research. | Anisomelia is a genus of moths in the family Geometridae described by Warren in 1895. == References == | 0-1
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In general the surgical treatment is done for improvement of the thumb function. However, an extra advantage of the surgery is the improvement in appearance of the thumb. In the past, surgical treatment of the triphalangeal thumb was not indicated, but now it is generally agreed that operative treatment improves function and appearance. Because an operation was not indicated in the past, there’s still a population with an untreated triphalangeal thumb. The majority of this population doesn’t want surgery, because the daily functioning of the hand is good. The main obstacle for the untreated patients might not be the diminished function, but the appearance of the triphalangeal thumb. The timing of surgery differs between Wood and Buck-Gramcko. Wood advises operation between the age of six months and two years, while Buck-Gramcko advises to operate for all indications before the age of six years. For TPT types I and II of the Buck-Gramcko classification, the surgical treatment typically consists of removing the extra phalanx and reconstructing the ulnar collateral ligament and the radial collateral ligament if necessary. For type III of Buck-Gramcko classification proposable surgical treatments:- smaller trapezoidal phalanx and under six years: removal of the extra phalanx and reconstruction of the ulnar collateral ligament. Lengthening of the radial collateral ligament is only indicated when the clinodactyly is still present after reconstruction of the ulnar collateral ligament. - trapezoidal phalanx and older than six years: partial removal of the extra phalanx with correction of the angle. | Because triphalangeal thumbs cannot easily oppose and do not possess many of the optimal qualities found in most opposable thumbs, they tend to cause the hand to be less effective in use and, therefore, prove to be more problematic in daily life. Cause
Malformations of the upper extremities can occur in the third to seventh embryonic week. In some cases the TPT is hereditary. In these cases, there is a mutation on chromosome 7q36. If the TPT is hereditary, it is mostly inherited as an autosomal dominant trait, non-opposable and bilateral. The sporadic cases are mostly opposable and unilateral. Syndromes
Triphalangeal thumb can occur in syndromes but it can also be isolated. The triphalangeal thumb can appear in combination with other malformations or syndromes.Syndromes include:
Holt-Oram syndrome
Aase syndrome
Diamond-Blackfan anemia
Townes-Brocks syndromeMalformations include:
Radial polydactyly
Syndactyly
Claw-like hand or foot
Diagnosis
Classifications
There are multiple classifications for the triphalangeal thumb. The reason for these different classifications is the heterogeneity in appearance of the TPT. The classification according to Wood describes the shape of the extra phalanx: delta (Fig. 4), rectangular or full phalanx (Table 1). With the classification made by Buck-Gramcko a surgical treatment can be chosen (Table 1). Buck-Gramcko differentiates between six different shapes of the extra phalanx and associated malformations.Table 1: Classifications of Wood and Buck-Gramcko
Treatment
The goals of surgical treatment are: reducing length of the thumb, creating a good functioning, a stable and non-deviated joint and improving the position of the thumb if necessary. Hereby improving function of the hand and thumb. | 11
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This changed after Arab slave traders entered central Africa from the east, following the Congo River, bringing parasites along. Gambian sleeping sickness travelled up the Congo River, and then further east.An Arab writer of the 14th century left the following description in the case of a sultan of the Mali Kingdom: "His end was to be overtaken by the sleeping sickness (illat an-nawm) which is a disease that frequently befalls the inhabitants of these countries especially their chieftains. Sleep overtakes one of them in such a manner that it is hardly possible to awake him. "The British naval surgeon John Atkins described the disease on his return from West Africa in 1734:
The Sleepy Distemper (common among the Negroes) gives no other previous Notice, than a want of Appetite 2 or 3 days before; their sleeps are sound, and Sense and Feeling very little; for pulling, drubbing or whipping will scarce stir up Sense and Power enough to move; and the Moment you cease beating the smart is forgot, and down they fall again into a state of Insensibility, drivling constantly from the Mouth as in deep salivation; breathe slowly, but not unequally nor snort. Young people are more subject to it than the old; and the Judgement generally pronounced is Death, the Prognostik seldom failing. | African trypanosomiasis, also known as African sleeping sickness or simply sleeping sickness, is an insect-borne parasitic infection of humans and other animals. It is caused by the species Trypanosoma brucei. Humans are infected by two types, Trypanosoma brucei gambiense (TbG) and Trypanosoma brucei rhodesiense (TbR). TbG causes over 98% of reported cases. Both are usually transmitted by the bite of an infected tsetse fly and are most common in rural areas.Initially, the first stage of the disease is characterized by fevers, headaches, itchiness, and joint pains, beginning one to three weeks after the bite. Weeks to months later, the second stage begins with confusion, poor coordination, numbness, and trouble sleeping. Diagnosis is by finding the parasite in a blood smear or in the fluid of a lymph node. A lumbar puncture is often needed to tell the difference between first- and second-stage disease. If the disease is not treated quickly it can lead to death. Prevention of severe disease involves screening the at-risk population with blood tests for TbG. Treatment is easier when the disease is detected early and before neurological symptoms occur. Treatment of the first stage has been with the medications pentamidine or suramin. Treatment of the second stage has involved eflornithine or a combination of nifurtimox and eflornithine for TbG. Fexinidazole is a more recent treatment that can be taken by mouth, for either stages of TbG. While melarsoprol works for both types, it is typically only used for TbR, due to serious side effects. | 11
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(The seventh category, not included in the table, is Undifferentiated and includes any patient with JIA who does not meet criteria for other subtypes, or who meets criteria for two or more subtypes). Oligoarticular arthritis
Oligoarticular (or pauciarticular) JIA is the most common JIA subtype, and occurs when there are up to four joints involved during the first six months of disease. Two subtypes of oligoarticular arthritis exist: persistent oligoarthritis, where no more than four joints are affected throughout the whole disease course; and extended oligoarthritis, where more than four joints are affected after the first six months of disease. Patients in this subtype are often young, typically aged two to three years and with a female preponderance. The most commonly involved joint is the knee, but other affected joints may include the ankles, wrists, elbows and others. The anti-nuclear antigen (ANA) is positive in up to 80% of patients with oligoarthritis and is associated with a higher risk of associated eye disease (uveitis), particularly in younger patients. The prefixes oligo- and pauci- mean few. Differential diagnosis
There are several other disorders and diseases that present with symptoms like JIA. These causes include, but are not limited to, infectious (for example septic arthritis or osteomyelitis) and post-infectious conditions (reactive arthritis, acute rheumatic fever, and in some geographic areas Lyme disease); hematologic and neoplastic diseases such as leukemia or bony tumors; and other connective tissue diseases (such as systemic lupus erythematosus). For the systemic-onset form of JIA, the differential diagnosis also includes Kawasaki disease and periodic fever syndromes. | Myopia has been increasing rapidly throughout the developed world, suggesting environmental factors are involved.A single-author literature review in 2021 proposed that myopia is the result of corrective lenses interfering with emmetropization. Genetics
A risk for myopia may be inherited from ones parents. Genetic linkage studies have identified 18 possible loci on 15 different chromosomes that are associated with myopia, but none of these loci is part of the candidate genes that cause myopia. Instead of a simple one-gene locus controlling the onset of myopia, a complex interaction of many mutated proteins acting in concert may be the cause. Instead of myopia being caused by a defect in a structural protein, defects in the control of these structural proteins might be the actual cause of myopia. A collaboration of all myopia studies worldwide identified 16 new loci for refractive error in individuals of European ancestry, of which 8 were shared with Asians. The new loci include candidate genes with functions in neurotransmission, ion transport, retinoic acid metabolism, extracellular matrix remodeling and eye development. The carriers of the high-risk genes have a tenfold increased risk of myopia. Aberrant genetic recombination and gene splicing in the OPNLW1 and OPNMW1 genes that code for two retinal cone photopigment proteins can produce high myopia by interfering with refractive development of the eye.Human population studies suggest that contribution of genetic factors accounts for 60–90% of variance in refraction. | 0-1
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However the trend of substituting sweetened drinks for milk has been found to lead to excess weight gain. Legal
Some jurisdictions use laws and regulations in an effort to steer children and parents towards making healthier food choices. Two examples are calorie count laws and banning soft drinks from sale at vending machines in schools. In the United Kingdom the Obesity Health Alliance has called on whichever party wins the general election to take measures to reduce childhood obesity, for example by banning advertisements for unhealthy foods before 9:00 pm and banning sports sponsorship by manufacturers of unhealthy foods. The failure of the present UK government to cut sugar, fat and salt content in foods has been criticised. Health experts, the health select committee and campaigners described Conservative plans over childhood obesity as, "weak" and "watered down". Physical activity
Physical inactivity of children has also shown to be a serious cause, and children who fail to engage in regular physical activity are at greater risk of obesity. Researchers studied the physical activity of 133 children over a three-week period using an accelerometer to measure each childs level of physical activity. They discovered the obese children were 35% less active on school days and 65% less active on weekends compared to non-obese children. Physical inactivity as a child could result in physical inactivity as an adult. | Researchers also did a cohort study on 19,397 babies, from their birth until age seven and discovered that high weight babies at four months were 1.38 times more likely to be overweight at seven years old compared to normal weight babies. High weight babies at the age of one were 1.17 times more likely to be overweight at age seven compared to normal weight babies. Medical illness
Cushings syndrome (a condition in which the body contains excess amounts of cortisol) may also influence childhood obesity. Researchers analyzed two isoforms (proteins that have the same purpose as other proteins, but are programmed by different genes) in the cells of 16 adults undergoing abdominal surgery. They discovered that one type of isoform created oxo-reductase activity (the alteration of cortisone to cortisol) and this activity increased 127.5 pmol mg sup when the other type of isoform was treated with cortisol and insulin. The activity of the cortisol and insulin can possibly activate Cushings syndrome.Hypothyroidism is a hormonal cause of obesity, but it does not significantly affect obese people who have it more than obese people who do not have it. In a comparison of 108 obese patients with hypothyroidism to 131 obese patients without hypothyroidism, researchers discovered that those with hypothyroidism had only 0.077 points more on the caloric intake scale than did those without hypothyroidism. Psychological factors
Researchers surveyed 1,520 children, ages 9–10, with a four-year follow up and discovered a positive correlation between obesity and low self-esteem in the four-year follow up. | 11
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Wernicke encephalopathy (WE), also Wernickes encephalopathy, or wet brain is the presence of neurological symptoms caused by biochemical lesions of the central nervous system after exhaustion of B-vitamin reserves, in particular thiamine (vitamin B1). The condition is part of a larger group of thiamine deficiency disorders that includes beriberi, in all its forms, and alcoholic Korsakoff syndrome. When it occurs simultaneously with alcoholic Korsakoff syndrome it is known as Wernicke–Korsakoff syndrome.Classically, Wernicke encephalopathy is characterised by a triad of symptoms: ophthalmoplegia, ataxia, and confusion. Around 10% of patients exhibit all three features, and other symptoms may also be present. While it is commonly regarded as a condition peculiar to malnourished people with alcohol misuse, it can be caused by a variety of diseases. It is treated with thiamine supplementation, which can lead to improvement of the symptoms and often complete resolution, particularly in those where alcohol misuse is not the underlying cause. Often other nutrients also need to be replaced, depending on the cause. Wernicke encephalopathy may be present in the general population with a prevalence of around 2%, and is considered underdiagnosed; probably, many cases are in patients who do not have commonly-associated symptoms. Signs and symptoms
The classic triad of symptoms found in Wernicke encephalopathy is:
ophthalmoplegia (later expanded to other eye movement disorders, most commonly affecting the lateral rectus muscle. Lateral nystagmus is most commonly seen although lateral rectus palsy, usually bilateral, may be seen). ataxia (later expanded to imbalance or any cerebellar signs)
confusion (later expanded to other mental changes. | sinus tachycardia
Orthostatic hypotension
Hypotension
Swollen tongue
Dysphagia (difficulty swallowing)
Glossodynia
Oral paraesthesiaRare (0.01-0.1% incidence) adverse effects include:
Neuroleptic malignant syndrome (Combination of fever, muscle stiffness, faster breathing, sweating, reduced consciousness, and sudden change in blood pressure and heart rate)
Tardive dyskinesia
Speech disturbance
Rhabdomyolysis
Angioedema
Blood dyscrasias such as agranulocytosis, leukopenia and neutropenia
Accommodation disorder
Pulmonary embolism
Gynaecomastia
GalactorrhoeaUnknown incidence adverse effects
Allergic reaction
Restless legs syndrome
Nausea
Oral mucosal lesions (ulcerations, blistering and inflammation)
Salivary hypersecretion
HyperprolactinaemiaAsenapine seems to have a relatively low weight gain liability for an atypical antipsychotic (which are notorious for their metabolic side effects) and a 2013 meta-analysis found significantly less weight gain (SMD [standard mean difference in weight gained in those on placebo vs. active drug]: 0.23; 95% CI: 0.07-0.39) than, paliperidone (SMD: 0.38; 95% CI: 0.27-0.48), risperidone (SMD: 0.42; 95% CI: 0.33-0.50), quetiapine (SMD: 0.43; 95% CI: 0.34-0.53), sertindole (SMD: 0.53; 95% CI: 0.38-0.68), chlorpromazine (SMD: 0.55; 95% CI: 0.34-0.76), iloperidone (SMD: 0.62; 95% CI: 0.49-0.74), clozapine (SMD: 0.65; 95% CI: 0.31-0.99), zotepine (SMD: 0.71; 95% CI: 0.47-0.96) and olanzapine (SMD: 0.74; 95% CI: 0.67-0.81) and approximately (that is, no statistically significant difference at the p=0.05 level) as much as weight gain as aripiprazole (SMD: 0.17; 95% CI: 0.05-0.28), lurasidone (SMD: 0.10; 95% CI: –0.02-0.21), amisulpride (SMD: 0.20; 95% CI: 0.05-0.35), haloperidol (SMD: 0.09; 95% CI: 0.00-0.17) and ziprasidone (SMD: 0.10; 95% CI: –0.02-0.22). Its potential for elevating plasma prolactin levels seems relatively limited too according to this meta-analysis. | 0-1
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Nervous system problems may occur in Pagets disease, resulting from increased pressure on the brain, spinal cord, or nerves, and reduced blood flow to the brain and spinal cord. When Pagets disease affects the facial bones, the teeth may become loose. Disturbance in chewing may occur. Chronic dental problems may lead to infection of the jaw bone. Angioid streaks may develop, possibly as a result of calcification of collagen or other pathological deposition.Pagets disease is not associated with osteoporosis. Although Pagets disease and osteoporosis can occur in the same patient, they are different disorders. Despite their marked differences, several treatments for Pagets disease are also used to treat osteoporosis. Causes
Viral
Pagets disease may be caused by a slow virus infection (i.e., paramyxoviridae) present for many years before symptoms appear. Associated viral infections include respiratory syncytial virus, canine distemper virus, and the measles virus. However, recent evidence has cast some doubt upon the measles association. Laboratory contamination may have played a role in past studies linking paramyxovirus (e.g. measles) to Pagets disease. Genetic
There is a hereditary factor in the development of Pagets disease of bone. Two genes, SQSTM1 and RANK, and specific regions of chromosome 5 and 6 are associated with Pagets disease of bone. Genetic causes may or may not involve a family history of Pagets disease.About 40–50% of people with the inherited version of Pagets disease have a mutation in the gene SQSTM1, which encodes a protein, called p62, that is involved in regulating the function of osteoclasts (bone cells). | First arch syndromes are congenital defects caused by a failure of neural crest cells to migrate into the first pharyngeal arch. They can produce facial anomalies. Examples of first arch syndromes include Treacher Collins syndrome and Pierre Robin syndrome. == References == | 0-1
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Part of the lining epithelium consists of chloragogen cells which are used for the storage of nutrients and in excretion. There are 10 to 17 pairs of metanephridia (excretory organs) in the mid-region of the leech. From these, ducts typically lead to a urinary bladder, which empties to the outside at a nephridiopore. Reproduction and development
Leeches are hermaphrodites, with the male reproductive organs, the testes, maturing first and the ovaries later. In hirudinids, a pair will line up with the clitellar regions in contact, with the anterior end of one leech pointing towards the posterior end of the other; this results in the male gonopore of one leech being in contact with the female gonopore of the other. The penis passes a spermatophore into the female gonopore and sperm is transferred to, and probably stored in, the vagina.Some jawless leeches (Rhynchobdellida) and proboscisless leeches (Arhynchobdellida) lack a penis, and in these, sperm is passed from one individual to another by hypodermic injection. The leeches intertwine and grasp each other with their suckers. A spermatophore is pushed by one through the integument of the other, usually into the clitellar region. The sperm is liberated and passes to the ovisacs, either through the coelomic channels or interstitially through specialist "target tissue" pathways.Some time after copulation, the small, relatively yolkless eggs are laid. In most species, an albumin-filled cocoon is secreted by the clitellum and receives one or more eggs as it passes over the female gonopore. | Almost seven hundred species of leech are currently recognised, of which some hundred are marine, ninety terrestrial and the remainder freshwater. Leeches have been used in medicine from ancient times until the 19th century to draw blood from patients. In modern times, leeches find medical use in treatment of joint diseases such as epicondylitis and osteoarthritis, extremity vein diseases, and in microsurgery, while hirudin is used as an anticoagulant drug to treat blood-clotting disorders. Diversity and phylogeny
Some 680 species of leech have been described, of which around 100 are marine, 480 freshwater and the remainder terrestrial. Among Euhirudinea, the true leeches, the smallest is about 1 cm (1⁄2 in) long, and the largest is the giant Amazonian leech, Haementeria ghilianii, which can reach 30 cm (12 in). Except for Antarctica, leeches are found throughout the world but are at their most abundant in temperate lakes and ponds in the northern hemisphere. The majority of freshwater leeches are found in the shallow, vegetated areas on the edges of ponds, lakes and slow-moving streams; very few species tolerate fast-flowing water. In their preferred habitats, they may occur in very high densities; in a favourable environment with water high in organic pollutants, over 10,000 individuals were recorded per square metre (over 930 per square foot) under rocks in Illinois. Some species aestivate during droughts, burying themselves in the sediment, and can lose up to 90% of their bodyweight and still survive. | 11
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Cardiotoxicity is a major problem with people treated with higher dose regimens.High-dose intravenous cyclophosphamide can cause the syndrome of inappropriate antidiuretic hormone secretion (SIADH) and a potentially fatal hyponatremia when compounded by intravenous fluids administered to prevent drug-induced cystitis. While SIADH has been described primarily with higher doses of cyclophosphamide, it can also occur with the lower doses used in the management of inflammatory disorders. Bladder bleeding
Acrolein is toxic to the bladder epithelium and can lead to hemorrhagic cystitis, which is associated with microscopic or gross hematuria and occasionally dysuria. Risks of hemorrhagic cystitis can be minimized with adequate fluid intake, avoidance of nighttime dosage and mesna (sodium 2-mercaptoethane sulfonate), a sulfhydryl donor which binds and detoxifies acrolein. Intermittent dosing of cyclophosphamide decreases cumulative drug dose, reduces bladder exposure to acrolein and has equal efficacy to daily treatment in the management of lupus nephritis. Infection
Neutropenia or lymphopenia arising secondary to cyclophosphamide usage can predispose people to a variety of bacterial, fungal and opportunistic infections. No published guidelines cover PCP prophylaxis for people with rheumatological diseases receiving immunosuppressive drugs, but some advocate its use when receiving high-dose medication. Infertility
Cyclophosphamide has been found to significantly increase the risk of premature menopause in females and of infertility in males and females, the likelihood of which increases with cumulative drug dose and increasing patient age. Such infertility is usually temporary, but can be permanent. The use of leuprorelin in women of reproductive age before administration of intermittently dosed cyclophosphamide may diminish the risks of premature menopause and infertility. | Toxic amblyopia, or nutritional optic neuropathy, is a condition where a toxic reaction in the optic nerve results in visual loss. Various poisonous substances may cause the condition as well as nutritional factors.Tobacco amblyopia is a form of toxic amblyopia caused by tobacco containing cyanide. Tobacco amblyopia is marked by a gradual impairment of vision characterised by visual field defects and hindered central vision. Methyl alcohol amblyopia occurs through acute poisoning by methyl alcohol and may lead to complete blindness. Since the term toxic amblyopia is a misnomer according to modern definition of amblyopia, it is now more accurately termed as toxic retinopathies or neuropathies. == References == | 0-1
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All three initial cases (belonging to one family – two brothers and one sister) had died by 3 November. The fourth case – a health care worker – developed symptoms on 4 November and was admitted to a hospital. The first confirmed case traveled to Kenya before the death. A close contact of the second confirmed case traveled to Kampala. It is reported that several hundred people may have been exposed to infection. 2021 Guinean cases
In August 2021, two months after the re-emergent Ebola epidemic in the Guéckédou prefecture was declared over, a case of the Marburg disease was confirmed by health authorities through laboratory analysis. Other potential case of the disease in a contact awaits official results. These are the first cases of the Marburg hemorrhagic fever confirmed to happen in West Africa. The cases of Marburg also have been identified in Guéckédou. On August 10, the WHO said health authorities in Guinea have confirmed one death from Marburg virus. 2022 Ghanaian cases
In July 2022, preliminary analysis of samples taken from two patients – both deceased – in Ghana indicated the cases were positive for Marburg. However, per standard procedure, the samples were sent to the Pasteur Institute of Dakar for confirmation. On 17 July 2022 the two cases were confirmed by Ghana, which caused the country to declare a Marburg virus disease outbreak. An additional case was identified, bringing the total to three. | Marburg virus disease (MVD; formerly Marburg hemorrhagic fever) is a viral hemorrhagic fever in humans and primates caused by either of the two Marburgviruses: Marburg virus (MARV) and Ravn virus (RAVV). Its clinical symptoms are very similar to those of Ebola virus disease (EVD).Egyptian fruit bats are believed to be the normal carrier in nature and Marburg virus RNA has been isolated from them. Signs and symptoms
The most detailed study on the frequency, onset, and duration of MVD clinical signs and symptoms was performed during the 1998–2000 mixed MARV/RAVV disease outbreak. A maculopapular rash, petechiae, purpura, ecchymoses, and hematomas (especially around needle injection sites) are typical hemorrhagic manifestations. However, contrary to popular belief, hemorrhage does not lead to hypovolemia and is not the cause of death (total blood loss is minimal except during labor). Instead, death occurs due to multiple organ dysfunction syndrome (MODS) due to fluid redistribution, hypotension, disseminated intravascular coagulation, and focal tissue necroses.Clinical phases of Marburg Hemorrhagic Fevers presentation are described below. Note that phases overlap due to variability between cases. Incubation: 2–21 days, averaging 5–9 days. Generalization Phase: Day 1 up to Day 5 from the onset of clinical symptoms. MHF presents with a high fever 104 °F (~40˚C) and a sudden, severe headache, with accompanying chills, fatigue, nausea, vomiting, diarrhea, pharyngitis, maculopapular rash, abdominal pain, conjunctivitis, and malaise. Early Organ Phase: Day 5 up to Day 13. Symptoms include prostration, dyspnea, edema, conjunctival injection, viral exanthema, and CNS symptoms, including encephalitis, confusion, delirium, apathy, and aggression. | 11
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Bacteriuria is the presence of bacteria in urine. Bacteriuria accompanied by symptoms is a urinary tract infection while that without is known as asymptomatic bacteriuria. Diagnosis is by urinalysis or urine culture. Escherichia coli is the most common bacterium found. People without symptoms should generally not be tested for the condition. Differential diagnosis include contamination.If symptoms are present treatment is generally with antibiotics. Bacteriuria without symptoms generally does not require treatment. Exceptions may include pregnant women, those who have had a recent kidney transplant, young children with significant vesicoureteral reflux, and those undergoing surgery of the urinary tract.Bacteriuria without symptoms is present in about 3% of otherwise healthy middle aged women. In nursing homes rates are as high as 50% among women and 40% in men. In those with a long term indwelling urinary catheter rates are 100%. Up to 10% of women have a urinary tract infection in a given year and half of all women have at least one infection at some point in their lives. There is an increased risk of asymptomatic or symptomatic bacteriuria in pregnancy due to physiological changes that occur in a pregnant women which promotes unwanted pathogen growth in the urinary tract. Signs and symptoms
Asymptomatic
Asymptomatic bacteriuria is bacteriuria without accompanying symptoms of a urinary tract infection and is commonly caused by the bacterium Escherichia coli. Other potential pathogens are Klebsiella spp., and group B streptococci. | Primary sclerosing cholangitis (PSC) is a long-term progressive disease of the liver and gallbladder characterized by inflammation and scarring of the bile ducts, which normally allow bile to drain from the gallbladder. Affected individuals may have no symptoms or may experience signs and symptoms of liver disease, such as yellow discoloration of the skin and eyes, itching, and abdominal pain. The bile duct scarring that occurs in PSC narrows the ducts of the biliary tree and impedes the flow of bile to the intestines. Eventually, it can lead to cirrhosis of the liver and liver failure. PSC increases the risk of various cancers, including liver cancer, gallbladder carcinoma, colorectal cancer, and cholangiocarcinoma. The underlying cause of PSC is unknown. Genetic susceptibility, immune system dysfunction, and abnormal composition of the gut flora may play a role. This is further suggested by the observation that around 75% of individuals with PSC also have inflammatory bowel disease (IBD), most often ulcerative colitis.No effective medical treatment for primary sclerosing cholangitis is known. Its most definitive treatment is a liver transplant, but it can recur after transplantation. Many people affected by PSC require a liver transplant. PSC is a rare disease and most commonly affects people with IBD. About 3.0 to 7.5% of people with ulcerative colitis have PSC, and 80% of people with PSC have some form of IBD. Diagnosis usually occurs in people in their 30s or 40s. Individuals of Northern European ancestry are affected more often than people of Southern European or Asian descent. | 0-1
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Typical onset is under the age of 30. Skin usually regrows over two to three weeks; however, complete recovery can take months. Overall, the risk of death with SJS is 5 to 10%. Signs and symptoms
SJS usually begins with fever, sore throat, and fatigue, which is commonly misdiagnosed and therefore treated with antibiotics. SJS, SJS/TEN, and TEN are often heralded by fever, sore throat, cough, and burning eyes for 1 to 3 days. Patients with these disorders frequently experience burning pain of their skin at the start of disease. Ulcers and other lesions begin to appear in the mucous membranes, almost always in the mouth and lips, but also in the genital and anal regions. Those in the mouth are usually extremely painful and reduce the patients ability to eat or drink. Conjunctivitis occurs in about 30% of children who develop SJS. A rash of round lesions about an inch across arises on the face, trunk, arms and legs, and soles of the feet, but usually not the scalp. Causes
SJS is thought to arise from a disorder of the immune system. The immune reaction can be triggered by drugs or infections. Genetic factors are associated with a predisposition to SJS. The cause of SJS is unknown in one-quarter to one-half of cases. SJS, SJS/TEN, and TEN are considered a single disease with common causes and mechanisms.Individuals expressing certain human leukocyte antigen (i.e. HLA) serotypes (i.e. | genetic alleles), genetical-based T cell receptors, or variations in their efficiency to absorb, distribute to tissues, metabolize, or excrete (this combination is termed ADME) a drug are predisposed to develop SJS. Medications
Although SJS can be caused by viral infections and malignancies, the main cause is medications. A leading cause appears to be the use of antibiotics, particularly sulfa drugs. Between 100 and 200 different drugs may be associated with SJS. No reliable test exists to establish a link between a particular drug and SJS for an individual case. Determining what drug is the cause is based on the time interval between first use of the drug and the beginning of the skin reaction. Drugs discontinued more than 1 month prior to onset of mucocutaneous physical findings are highly unlikely to cause SJS and TEN. SJS and TEN most often begin between 4 and 28 days after culprit drug administration. A published algorithm (ALDEN) to assess drug causality gives structured assistance in identifying the responsible medication.SJS may be caused by the medications rivaroxaban, vancomycin, allopurinol, valproate, levofloxacin, diclofenac, etravirine, isotretinoin, fluconazole, valdecoxib, sitagliptin, oseltamivir, penicillins, barbiturates, sulfonamides, phenytoin, azithromycin, oxcarbazepine, zonisamide, modafinil, lamotrigine, nevirapine, pyrimethamine, ibuprofen, ethosuximide, carbamazepine, bupropion, telaprevir, and nystatin.Medications that have traditionally been known to lead to SJS, erythema multiforme, and toxic epidermal necrolysis include sulfonamide antibiotics, penicillin antibiotics, cefixime (antibiotic), barbiturates (sedatives), lamotrigine, phenytoin (e.g., Dilantin) (anticonvulsants) and trimethoprim. | 11
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Mutation of the MDM2 SNP309 gene is associated with increased risks for younger women.Fair- and red-haired people, persons with multiple atypical nevi or dysplastic nevi and persons born with giant congenital melanocytic nevi are at increased risk.A family history of melanoma greatly increases a persons risk, because mutations in several genes have been found in melanoma-prone families. People with a history of one melanoma are at increased risk of developing a second primary tumor.Fair skin is the result of having less melanin in the skin, which means less protection from UV radiation exists. A family history could indicate a genetic predisposition to melanoma. Pathophysiology
The earliest stage of melanoma starts when melanocytes begin out-of-control growth. Melanocytes are found between the outer layer of the skin (the epidermis) and the next layer (the dermis). This early stage of the disease is called the radial growth phase, when the tumor is less than 1 mm thick, and spreads at the level of the basal epidermis. Because the cancer cells have not yet reached the blood vessels deeper in the skin, it is very unlikely that this early-stage melanoma will spread to other parts of the body. If the melanoma is detected at this stage, then it can usually be completely removed with surgery.When the tumor cells start to move in a different direction – vertically up into the epidermis and into the papillary dermis – cell behaviour changes dramatically.The next step in the evolution is the invasive radial growth phase, in which individual cells start to acquire invasive potential. | Neutropenic enterocolitis is inflammation of the cecum (part of the large intestine) that may be associated with infection. It is particularly associated with neutropenia, a low level of neutrophil granulocytes (the most common form of white blood cells) in the blood. Signs and symptoms
Signs and symptoms of typhlitis may include diarrhea, a distended abdomen, fever, chills, nausea, vomiting, and abdominal pain or tenderness. Cause
The condition is usually caused by Gram-positive enteric commensal bacteria of the gut (gut flora). Clostridium difficile is a species of Gram-positive bacteria that commonly causes severe diarrhea and other intestinal diseases when competing bacteria are wiped out by antibiotics, causing pseudomembranous colitis, whereas Clostridium septicum is responsible for most cases of neutropenic enterocolitis.Typhlitis most commonly occurs in immunocompromised patients, such as those undergoing chemotherapy, patients with AIDS, kidney transplant patients, or the elderly. Diagnosis
Typhlitis is diagnosed with a radiograph CT scan showing thickening of the cecum and "fat stranding". Treatment
Typhlitis is a medical emergency and requires prompt management. Untreated typhlitis has a poor prognosis, particularly if associated with pneumatosis intestinalis (air in the bowel wall) and/or bowel perforation, and has significant morbidity unless promptly recognized and aggressively treated.Successful treatment hinges on:
Early diagnosis provided by a high index of suspicion and the use of CT scanning
Nonoperative treatment for uncomplicated cases
Empiric antibiotics, particularly if the patient is neutropenic or at other risk of infection.In rare cases of prolonged neutropenia and complications such as bowel perforation, neutrophil transfusions can be considered but have not been studied in a randomized control trial. | 0-1
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Mutations in the PC gene reduce the amount of pyruvate carboxylase in cells or disrupt the enzymes activity. The missing or altered enzyme cannot carry out its essential role in generating glucose, which impairs the bodys ability to make energy in mitochondria. Additionally, a loss of pyruvate carboxylase allows potentially toxic compounds, such as lactic acid and ammonia, to build up and damage organs and tissues. Loss of pyruvate carboxylase function in the nervous system, particularly the role of the enzyme in myelin formation and neurotransmitter production, may contribute to the neurological features of pyruvate carboxylase deficiency. Diagnosis
Classification
There are at least three types of pyruvate carboxylase deficiency, which are distinguished by the severity of their signs and symptoms. Type A
Type A, which has been identified mostly in people from North America, has moderately severe symptoms that begin in infancy. Characteristic features include developmental delay and a buildup of lactic acid in the blood (lactic acidosis). Increased acidity in the blood can lead to vomiting, abdominal pain, extreme tiredness (fatigue), muscle weakness, and difficulty breathing. In some cases, episodes of lactic acidosis are triggered by an illness or periods without food. Children with pyruvate carboxylase deficiency type A typically survive only into early childhood. Type B
Pyruvate carboxylase deficiency type B has life-threatening signs and symptoms that become apparent shortly after birth. This form of the condition has been reported mostly in Europe, particularly France. Affected infants have severe lactic acidosis, a build-up of ammonia in the blood (hyperammonemia), and liver failure. | Ross syndrome consists of Adies syndrome (myotonic pupils and absent deep tendon reflexes) plus segmental anhidrosis (typically associated with compensatory hyperhidrosis).It was characterized in 1958 by A.T. Ross.By 1992, eighteen cases had been documented.Signs and Symptoms
Initial manifestations often include an abnormal segmental sweating response (described as hyperhidrosis or anhidrosis in some patients) and a tonic pupil. Other commonly reported symptoms included fatigue, chronic cough, and increased urinary frequency.Prognosis
Ross syndrome is a non life-threatening benign condition but delay in diagnosis can result in slow progression of autonomic symptoms. Epidemiology
Ross Syndrome is a progressive autonomic dysfunction that can occur in any age, ethnicity, or gender. The average age of diagnosis for Ross syndrome is 36 years and affects more females than males. See also
Hypohidrosis
List of cutaneous conditions
References
== External links == | 0-1
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Diagnosis
Diagnosis is made through a combination of patient history, neurological examination, and medical imaging. Magnetic resonance imaging (MRI) is considered the preferred imaging modality for Chiari malformation. The MRI visualizes neural tissue such as the cerebellar tonsils and spinal cord as well as bone and other soft tissues. CT and CT myelography are other options and were used prior to the advent of MRI, unfortunately the resolution of CT based modalities do not characterize syringomyelia and other neural abnormalities as well.By convention, the cerebellar tonsil position is measured relative to the basion-opisthion line, using sagittal T1 MRI images or sagittal CT images. The selected cutoff distance for abnormal tonsil position is somewhat arbitrary, as not every person will be symptomatic at a certain amount of tonsil displacement, and the probability of symptoms and syrinx increases with greater displacement; however, greater than 5 mm is the most frequently cited cutoff number, though some consider 3–5 mm to be "borderline,"; pathological signs and syrinx may occur beyond that distance. One study showed little difference in cerebellar tonsil position between standard recumbent MRI and upright MRI for patients without a history of whiplash injury. Neuroradiological investigation is used to first rule out any intracranial condition that could be responsible for tonsillar herniation. Neuroradiological diagnostics evaluate the severity of crowding of the neural structures within the posterior cranial fossa and their pressure against the foramen magnum. | Hyperglycinemia may refer to one of two related inborn amino acid disorders that are characterized by elevated levels of glycine in the blood. Propionic acidemia, also known as "ketotic glycinemia"
Glycine encephalopathy, also known as "non-ketotic hyperglycinemia" | 0-1
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A pivotal clinical trial carried out in the early 1970s showed that among 303 GBM patients randomized to radiation or nonradiation therapy, those who received radiation had a median survival more than double those who did not. Subsequent clinical research has attempted to build on the backbone of surgery followed by radiation. On average, radiotherapy after surgery can reduce the tumor size to 107 cells. Whole-brain radiotherapy does not improve when compared to the more precise and targeted three-dimensional conformal radiotherapy. A total radiation dose of 60–65 Gy has been found to be optimal for treatment.GBM tumors are well known to contain zones of tissue exhibiting hypoxia, which are highly resistant to radiotherapy. Various approaches to chemotherapy radiosensitizers have been pursued, with limited success as of 2016. As of 2010, newer research approaches included preclinical and clinical investigations into the use of an oxygen diffusion-enhancing compound such as trans sodium crocetinate as radiosensitizers, and as of 2015 a clinical trial was underway. Boron neutron capture therapy has been tested as an alternative treatment for glioblastoma, but is not in common use. Chemotherapy
Most studies show no benefit from the addition of chemotherapy. However, a large clinical trial of 575 participants randomized to standard radiation versus radiation plus temozolomide chemotherapy showed that the group receiving temozolomide survived a median of 14.6 months as opposed to 12.1 months for the group receiving radiation alone. This treatment regimen is now standard for most cases of glioblastoma where the person is not enrolled in a clinical trial. | The neural subtype was typified by the expression of neuron markers such as NEFL, GABRA1, SYT1, and SLC12A5, while often presenting themselves as normal cells upon pathological assessment.Many other genetic alterations have been described in glioblastoma, and the majority of them are clustered in two pathways, the RB and the PI3K/AKT. Glioblastomas have alterations in 68–78% and 88% of these pathways, respectively.Another important alteration is methylation of MGMT, a "suicide" DNA repair enzyme. Methylation impairs DNA transcription and expression of the MGMT gene. Since the MGMT enzyme can repair only one DNA alkylation due to its suicide repair mechanism, reserve capacity is low and methylation of the MGMT gene promoter greatly affects DNA-repair capacity. MGMT methylation is associated with an improved response to treatment with DNA-damaging chemotherapeutics, such as temozolomide. Cancer stem cells
Glioblastoma cells with properties similar to progenitor cells (glioblastoma cancer stem cells) have been found in glioblastomas. Their presence, coupled with the glioblastomas diffuse nature results in difficulty in removing them completely by surgery, and is therefore believed to be the possible cause behind resistance to conventional treatments, and the high recurrence rate. Glioblastoma cancer stem cells share some resemblance with neural progenitor cells, both expressing the surface receptor CD133. CD44 can also be used as a cancer stem cell marker in a subset of glioblastoma tumour cells. Glioblastoma cancer stem cells appear to exhibit enhanced resistance to radiotherapy and chemotherapy mediated, at least in part, by up-regulation of the DNA damage response. | 11
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Of late there has been renewed interest in liver transplantation from deceased donors along with add on therapy. Prognosis remains poor. Epidemiology
Approximately 15,000 new cases of liver and biliary tract carcinoma are diagnosed annually in the United States, with roughly 10% of these cases being Klatskin tumors. Cholangiocarcinoma accounts for approximately 2% of all cancer diagnoses, with an overall incidence of 1.2/100,000 individuals. Two-thirds of cases occur in patients over the age of 65, with a nearly ten-fold increase in patients over 80 years of age. The incidence is similar in both men and women. References
== External links == | This type of softening occurs in areas that continue to be poorly perfused, with little to no blood flow. These are known as "pale" or "anemic infarcts" and are areas that contain dead neuronal tissue, which result in a softening of the cerebrum. Yellow softening
Yellow softening is the third type of cerebral softening. As its name implies, the affected softened areas of the brain have a yellow appearance. This yellow appearance is due to atherosclerotic plaque build-up in interior brain arteries coupled with yellow lymph around the choroid plexus, which occurs in specific instances of brain trauma. Stages
Early life
Newborn cerebral softening has traditionally been attributed to trauma at birth and its effect on brain tissue into adulthood. However, more recent research shows that cerebral softening in newborns and the degeneration of white matter is caused by asphyxia and/or later infection. There is no causal evidence to support the hypothesis that problems in labor contribute to the development of softening in infant white matter. Also, further evidence shows a possible connection between low sugar and high protein levels in cerebral spinal fluid that can contribute to disease or virus susceptibility leading to cerebral softening. Later life
Cases of cerebral softening in infancy versus in adulthood are much more severe due to an infants inability to sufficiently recover brain tissue loss or compensate the loss with other parts of the brain. | 0-1
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Environmental fate
Abiotic degradation
EDTA is in such widespread use that questions have been raised whether it is a persistent organic pollutant. While EDTA serves many positive functions in different industrial, pharmaceutical and other avenues, the longevity of EDTA can pose serious issues in the environment. The degradation of EDTA is slow. It mainly occurs abiotically in the presence of sunlight.The most important process for the elimination of EDTA from surface waters is direct photolysis at wavelengths below 400 nm. Depending on the light conditions, the photolysis half-lives of iron(III) EDTA in surface waters can range as low as 11.3 minutes up to more than 100 hours. Degradation of FeEDTA, but not EDTA itself, produces iron complexes of the triacetate (ED3A), diacetate (EDDA), and monoacetate (EDMA) – 92% of EDDA and EDMA biodegrades in 20 hours while ED3A displays significantly higher resistance. Many environmentally-abundant EDTA species (such as Mg2+ and Ca2+) are more persistent. Biodegradation
In many industrial wastewater treatment plants, EDTA elimination can be achieved at about 80% using microorganisms. Resulting byproducts are ED3A and iminodiacetic acid (IDA) – suggesting that both the backbone and acetyl groups were attacked. Some microorganisms have even been discovered to form nitrates out of EDTA, but they function optimally at moderately alkaline conditions of pH 9.0–9.5.Several bacterial strains isolated from sewage treatment plants efficiently degrade EDTA. Specific strains include Agrobacterium radiobacter ATCC 55002 and the sub-branches of Pseudomonadota like BNC1, BNC2, and strain DSM 9103. The three strains share similar properties of aerobic respiration and are classified as gram-negative bacteria. | Hyoscyamine (also known as daturine or duboisine) is a naturally occurring tropane alkaloid and plant toxin. It is a secondary metabolite found in certain plants of the family Solanaceae, including henbane, mandrake, angels trumpets, jimsonweed, tomato, the sorcerers tree, and deadly nightshade. It is the levorotary isomer of atropine (third of the three major nightshade alkaloids) and thus sometimes known as levo-atropine.Brand names for hyoscyamine include Symax, HyoMax, Anaspaz, Egazil, Buwecon, Cystospaz, Levsin, Levbid, Levsinex, Donnamar, NuLev, Spacol T/S, and Neoquess. Uses
Hyoscyamine is used to provide symptomatic relief of spasms caused by various lower abdominal and bladder disorders including peptic ulcers, irritable bowel syndrome, diverticulitis, pancreatitis, colic, and interstitial cystitis. It has also been used to relieve some heart problems, control some of the symptoms of Parkinsons disease, as well as for control of abnormal respiratory symptoms and "hyper-mucus secretions" in patients with lung disease.It is also useful in pain control for neuropathic pain, chronic pain and palliative care – "comfort care" – for those with intractable pain from treatment resistant, untreatable, and incurable diseases. When combined with opioids it increases the level of analgesia (pain relief) obtained. Several mechanisms are thought to contribute to this effect. The closely related drugs atropine and hyoscine and other members of the anticholinergic drug group like cyclobenzaprine, trihexyphenidyl, and orphenadrine are also used for this purpose. When hyoscyamine is used along with opioids or other anti-peristaltic agents, measures to prevent constipation are especially important given the risk of paralytic ileus. | 0-1
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Mold
Some studies have found that Sick building syndrome may be associated with indoor mould or mycotoxin contamination. However, the attribution of sick building syndrome to mould is controversial and supported by little evidence. Reducing the dampness in all indoor environment will prevent all the respiratory issues caused by dampness and mold in indoor environment. Indoor temperature
Indoor temperature under 18 °C (64 °F) has been shown to be associated with increased respiratory and cardiovascular diseases, increased blood levels, and increased hospitalization. Diagnosis
While sick building syndrome (SBS) encompasses a multitude of non-specific symptoms, building-related illness (BRI) comprises specific, diagnosable symptoms caused by certain agents (chemicals, bacteria, fungi, etc.). These can typically be identified, measured, and quantified. There are usually 4 causal agents in BRI; 1.) Immunologic, 2.) Infectious, 3.) toxic, and 4.) irritant. For instance, Legionnaires disease, usually caused by Legionella pneumophila, involves a specific organism which could be ascertained through clinical findings as the source of contamination within a building. Prevention
Regular inspections to indicate for presence of mold or other toxins
Adequate maintenance of all building mechanical systems
Toxin-absorbing plants, such as sansevieria
Roof shingle non-pressure cleaning for removal of algae, mold, and Gloeocapsa magma
Using ozone to eliminate the many sources, such as VOCs, molds, mildews, bacteria, viruses, and even odors. However, numerous studies identify high-ozone shock treatment as ineffective despite commercial popularity and popular belief. | Immune factors
A common feature of immune factors in causing recurrent pregnancy loss appears to be a decreased maternal immune tolerance towards the fetus. Antiphospholipid syndrome
The antiphospholipid syndrome is an autoimmune disease that is a common cause of recurrent pregnancy loss. Around 15% of the women who have recurrent miscarriages have high levels of antiphospholipid antibodies. Women who have had more than one miscarriage in the first trimester, or a miscarriage in the second trimester, may have their blood tested for antibodies, to determine if they have antiphospholipid syndrome. Women diagnosed with antiphospholipid syndrome generally take aspirin or heparin in subsequent pregnancies, but questions remain due to the lack of high quality trials. Thyroid antibodies
Anti-thyroid autoantibodies are associated with an increased risk of recurrent miscarriage with an odds ratio of 2.3 with a 95% confidence interval of 1.5–3.5. Increased uterine NK cells
Natural killer cells, a type of white blood cell, are present in uterine tissue. High levels of these cells may be linked to RPL but high numbers or the presence of these cells is not a predictor of pregnancy loss in women who have not have had a miscarriage. Parental HLA sharing
Earlier studies that perhaps paternal sharing of HLA genes would be associated with increased pregnancy loss have not been confirmed. Male-specific minor histocompatibility
Immunization of mothers against male-specific minor histocompatibility (H-Y) antigens has a pathogenic role in many cases of secondary recurrent miscarriage, that is, recurrent miscarriage in pregnancies succeeding a previous live birth. | 0-1
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Dentin dysplasia (DD) is a rare genetic developmental disorder affecting dentine production of the teeth, commonly exhibiting an autosomal dominant inheritance that causes malformation of the root. It affects both primary and permanent dentitions in approximately 1 in every 100,000 patients. It is characterized by presence of normal enamel but atypical dentin with abnormal pulpal morphology. Witkop in 1972 classified DD into two types which are Type I (DD-1) is the radicular type, and type II (DD-2) is the coronal type. DD-1 has been further divided into 4 different subtypes (DD-1a,1b,1c,1d) based on the radiographic features. Signs and symptoms
Clinically the teeth look normal in colour and morphologic appearance; however, they are commonly very mobile and exfoliated prematurely.Both primary and permanent dentitions can be affected by either type I or type II dentin dysplasia. However, deciduous teeth affected by type II dentin dysplasia have a characteristic blue-amber discolouration, whilst the other dentition appears normal. Causes
The mutation in collagen type 1 (COL1 A1, COL1 A2) causes DI-1. It is similar to the systemic condition dental features known as osteogenesis imperfect. DI-2, DI-3 and DD-2 share the same genetic mutation of dentin sialophosphoprotein, that is located on chromosome 4. They are autosomal-dominant diseases with complete penetrance and variable expressivity. Due to the same genetic mutation, these diseases would often result in overlapping clinical and radiographic features. Therefore, prevailing theories suggests that DI-2, DI-3 and DD-2 are categorized as a single disease entity with variable severity of expression. However, the causes of DD-1 have yet to be theorized. | Diagnosis
Diagnosis is mostly based on general examination and radiographs, and it should be taken when abnormality of the teeth is suspected as most of the affected teeth have normal clinical appearance.Differential diagnosis is very important to have a definitive diagnosis as some radiographic or histologic features of dentine dysplasia may bear a resemblance to different disorders:
Dentinogenesis imperfecta
Odontodysplasia
Calcinosis
Osteogenesis imperfecta
Ehlers–Danlos syndromes
Goldblatt syndrome
Schimke immuno-osseous dysplasia
Brachio-skeleto-genital syndrome. Type I: Radicular type
Type I has been known as radicular dentine dysplasia because the teeth have undeveloped root(s) with abnormal pulp tissue. Morphology and colour of the crown mostly appear normal, but occasionally teeth appear slightly amber coloured or bluish-brown shine in primary teeth with no or only immature root development. The teeth are mostly maligned and have higher risk of fracture. Radiographic feature
In other words, affected primary teeth usually have abnormal shaped or shorter than normal roots. “Crescent/half-moon shaped” pulp chamber remnant in permanent teeth can be seen on x-rays. The roots may appear to be darker or radiolucent/pointy and short with apical constriction. Dentine is laid down abnormally and causes excessive growth within the pulp chamber. This will reduce the pulp space and eventually cause incomplete and total pulp chamber obliteration in permanent teeth. Sometimes periapical pathology or cysts can be seen around the root apex. Most cases of DD associated with peri-apical radiolucency/ pathology have been diagnosed as radicular cysts, but some of them have been as diagnosed peri-apical grauloma instead. Type II: Coronal type
Type II would mostly cause discolouration to the primary teeth. | 11
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Studies of infested lakes and outbreaks in Europe and North America have found cases where infection risk appears to be evenly distributed around the margins of water bodies as well as instances where risk increases in endemic swimmers itch "hotspots". Children may become infected more frequently and more intensely than adults but this probably reflects their tendency to swim for longer periods inshore, where cercariae also concentrate. Stimuli for cercarial penetration into host skin include unsaturated fatty acids, such as linoleic and linolenic acids. These essential fatty acids are found in many edible crops, derived plant oils, sun lotions and creams based on plant oils. Control
Various strategies targeting the mollusc and avian hosts of schistosomes have been used by lakeside residents in recreational areas of North America to deal with outbreaks of swimmers itch. In Michigan, for decades, authorities used copper sulfate as a molluscicide to reduce snail host populations and thereby the incidence of swimmers itch. The results with this agent have been inconclusive, possibly because:
Snails become tolerant
Local water chemistry reduces the molluscicides efficacy
Local currents diffuse it
Adjacent snail populations repopulate a treated areaMore importantly, perhaps, copper sulfate is toxic to more than just molluscs, and the effects of its use on aquatic ecosystems are not well understood.Another method targeting the snail host, mechanical disturbance of snail habitat, has been also tried in some areas of North America and Lake Annecy in France, with promising results. Some work in Michigan suggests that administering praziquantel to hatchling waterfowl can reduce local swimmers itch rates in humans. | Each papule corresponds to the penetration site of a single parasite. After locating a bird, the parasite penetrates through the skin (usually the feet), dropping the forked tail in the process. Inside the circulatory system, the immature worms (schistosomula) develop into mature male and female worms, mate and migrate through the hosts circulatory system (or nervous system in case of T. regenti) to the final location (veins feeding the gastrointestinal tract) within the host body. There they lay eggs in the small veins in the intestinal mucosa from which they make their way into the lumen of the gut, and are dumped into the water when the bird defecates. One European species, Trichobilharzia regenti, instead infects the bird hosts nasal tissues and larvae hatch from the eggs directly in the tissue during drinking/feeding of the infected birds. Risk factors
Humans usually become infected after swimming in slow-moving rivers, lakes or ponds. Some laboratory evidence indicates snails shed cercariae most intensely in the morning and on sunny days, and exposure to water in these conditions may therefore increase risk. Duration of swimming is correlated with increased risk of infection in Europe and North America, and shallow inshore waters may harbour higher densities of cercariae than open waters offshore. Onshore winds are thought to cause cercariae to accumulate along shorelines. | 11
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interphalangeal joints of the fingers) and there can be associated tenosynovitis, but there is no erosion or deformation in the long term. Diarrhea may also occur. Mechanism
Immune complexes are thought to cause blood vessel damage, attracting neutrophils into the skin and synovium in BADAS. These antigen-antibody complexes are thought to be caused by excessive exposure to bacterial antigens (especially peptidoglycans). Bacterial overgrowth appears to be a frequent underlying condition. These antibodies possibly stimulate migration of neutrophils into the affected joints and skin. The effect of antibacterial therapy supports a role for bacteria in the disease mechanism (pathogenesis). Diagnosis
A diagnosis of this syndrome is made when an individual has the constellation of the characteristic recurrent neutrophilic dermatosis, flu-like symptoms, arthralgias or arthritis, and myalgias in the setting of a pathology of the bowels that is not best explained by another diagnosis. See also
Sweets syndrome-like dermatosis
List of cutaneous conditions
== References == | An overlap syndrome is a medical condition which shares features of at least two more widely recognised disorders. Examples of overlap syndromes can be found in many medical specialties such as overlapping connective tissue disorders in rheumatology, and overlapping genetic disorders in cardiology. Rheumatology
Examples of overlap syndromes in rheumatology include mixed connective tissue disease and scleromyositis. Diagnosis depends on which diseases the patient shows symptoms and has positive antibodies for in their lab serology. In overlap syndrome, features of the following diseases are found (most common listed):
Systemic lupus erythematosus (SLE)
Systemic sclerosis
Polymyositis
Dermatomyositis
Rheumatoid arthritis (RA)
Sjögrens syndrome
Eosinophilic granulomatosis with polyangiitis (EGPA)
Autoimmune thyroiditis
Antiphospholipid antibody syndromeThe treatment of overlapping connective tissue disorders is mainly based on the use of corticosteroids and immunosuppressants. Biologic drugs, i.e. anti-TNFα or anti-CD20 monoclonal antibodies, have been recently introduced as alternative treatments in refractory cases. There are some concerns with the use of anti-TNF agents in patients with systemic autoimmune diseases due to the risk of triggering disease exacerbations.The term polyangiitis overlap syndrome refers to a systemic vasculitis that shares features with two or more distinct vasculitis syndromes. The most common type of polyangiitis overlap syndrome is microscopic polyangiitis (MPA), which shares features with EGPA, granulomatosis with polyangiitis and panarteritis nodosa. Sometimes polyangiitis overlap syndrome is used as a synonym for MPA. Gastroenterology
In gastroenterology, the term overlap syndrome may be used to describe autoimmune liver diseases that combine characteristic features of autoimmune hepatitis, primary biliary cirrhosis and primary sclerosing cholangitis. | 0-1
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Therefore, fracture of the bone may not only lead to lesion of the radial nerve, but also haematoma of the internal structures of the arm. The artery then continues on to anastamose with the recurrent radial branch of the brachial artery, providing a diffuse blood supply for the elbow joint. Veins
The veins of the arm carry blood from the extremities of the limb, as well as drain the arm itself. The two main veins are the basilic and the cephalic veins. There is a connecting vein between the two, the median cubital vein, which passes through the cubital fossa and is clinically important for venepuncture (withdrawing blood). The basilic vein travels on the medial side of the arm and terminates at the level of the seventh rib. The cephalic vein travels on the lateral side of the arm and terminates as the axillary vein. It passes through the deltopectoral triangle, a space between the deltoid and the pectoralis major muscles. Society and culture
In Hindu, Buddhist and Egyptian iconography the symbol of the arm is used to illustrate the power of the sovereign. In Hindu tradition gods are depicted with several arms which carry specific symbols of their powers. It is believed that several arms depict omnipotence of gods. In popular culture Thakur did not have arms in the movie Sholay. In West Africa, the Bambara use forearm to symbolize the spirit, which is a link between God and man. Symbolic gestures of raising both hands signal surrender, appeals for mercy, and justice. | Self-defeating personality disorder (also known as masochistic personality disorder) was a proposed personality disorder. As a descriptor for Other personality disorder it was mentioned in the DSM-III in 1980. It was discussed in an appendix of the revised third edition of the Diagnostic and Statistical Manual of Mental Disorders (DSM-III-R) in 1987, but was never formally admitted into the manual. Because of its significant overlap with other personality disorders (borderline, avoidant and dependent) the distinction was not seen as clinically valuable. It was entirely excluded from the DSM-IV. Since the DSM-5, the diagnoses other specified personality disorder and unspecified personality disorder have mostly replaced its use. Diagnosis
Definition proposed in DSM III-R for further review
Self-defeating personality disorder is:
A) A pervasive pattern of self-defeating behavior, beginning by early adulthood and present in a variety of contexts. | 0-1
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Alternatively, a drug or its metabolite may stimulate these T cells by inserting into the groove on a HLA protein to serve as a non-self epitope or bind outside of this groove to alter a HLA protein so that it forms a non-self epitope. In all these cases, however, a non-self epitope must bind to a specific HLA serotype (i.e. variation) in order to stimulate T cells. Since the human population expresses some 13,000 different HLA serotypes while an individual expresses only a fraction of them and since a SJS-inducing drug or metabolite interacts with only one or a few HLA serotypes, a drugs ability to induce SCARs is limited to those individuals who express HLA serotypes targeted by the drug or its metabolite. Accordingly, only rare individuals are predisposed to develop a SCARs in response to a particular drug on the bases of their expression of HLA serotypes: Studies have identified several HLA serotypes associated with development of SJS, SJS/TEN, or TEN in response to certain drugs. In general, these associations are restricted to the cited populations.In some East Asian populations studied (Han Chinese and Thai), carbamazepine- and phenytoin-induced SJS is strongly associated with HLA-B*1502 (HLA-B75), an HLA-B serotype of the broader serotype HLA-B15. A study in Europe suggested the gene marker is only relevant for East Asians. | CD4+ T cells) to initiate autoimmune reactions that attack self tissues. In particular, it is a type IV, subtype IVc, delayed hypersensitivity reaction dependent in part on the tissue-injuring actions of natural killer cells. This contrasts with the other types of SCARs disorders, i.e., the DRESS syndrome which is a Type IV, Subtype IVb, hypersensitivity drug reaction dependent in part on the tissue-injuring actions of eosinophils and acute generalized exanthematous pustulosis which is a Type IV, subtype IVd, hypersensitivity reaction dependent in part on the tissue-injuring actions of neutrophils.Like other SCARs-inducing drugs, SJS-inducing drugs or their metabolites stimulate CD8+ T cells or CD4+ T cells to initiate autoimmune responses. Studies indicate that the mechanism by which a drug or its metabolites accomplishes this involves subverting the antigen presentation pathways of the innate immune system. The drug or metabolite covalently binds with a host protein to form a non-self, drug-related epitope. An antigen presenting cell (APC) takes up these alter proteins; digests them into small peptides; places the peptides in a groove on the human leukocyte antigen (i.e. HLA) component of their major histocompatibility complex (i.e. MHC); and presents the MHC-associated peptides to T-cell receptors on CD8+ T cells or CD4+ T cells. Those peptides expressing a drug-related, non-self epitope on one of their various HLA protein forms (HLA-A, HLA-B, HLA-C, HLA-DM, HLA-DO, HLA-DP, HLA-DQ, or HLA-DR) can bind to a T-cell receptor and thereby stimulate the receptor-bearing parent T cell to initiate attacks on self tissues. | 11
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In a few cases a sore mouth can develop, and if so pain is sometimes made worse by toothpastes, or hot or spicy food. The lesions can extend to involve the palate.Plasma cell cheilitis appears as well defined, infiltrated, dark red plaque with a superficial lacquer-like glazing. Plasma cell cheilitis usually involves the lower lip. The lips appear dry, atrophic and fissured. Angular cheilitis is sometimes present.Where the condition involves the tongue, there is an erythematous enlargement with furrows, crenation and loss of the normal dorsal tongue coating. Causes
Plasma cell gingivitis and plasma cell cheilitis are thought to be hypersensitivity reactions to some antigen. Possible sources of antigens include ingredients in toothpastes, chewing gum, mints, pepper, or foods. Specifically, cinnamonaldehyde and cinnamon flavoring are often to blame. However, the exact cause in most is unknown. Diagnosis
Histologically plasma cell gingivitis shows mainly plasma cells. The differential diagnosis is with acute leukemia and multiple myeloma. Hence, blood tests are often involved in ruling out other conditions. A biopsy is usually taken, and allergy testing may also be used. The histopathologic appearance is characterized by diffuse, sub-epithelial plasma cell inflammatory infiltration into the connective tissue. The epithelium shows spongiosis. Some consider that plasmoacanthoma (solitary plasma cell tumor) is part of the same spectrum of disease as plasma cell cheilitis. Classification
Depending upon the site of involvement, this condition could be considered a type of gingivitis (or gingival enlargement); a type of cheilitis; glossitis; or stomatitis. | Epidural hematoma is when bleeding occurs between the tough outer membrane covering the brain (dura mater) and the skull. Often there is loss of consciousness following a head injury, a brief regaining of consciousness, and then loss of consciousness again. Other symptoms may include headache, confusion, vomiting, and an inability to move parts of the body. Complications may include seizures.The cause is typically head injury that results in a break of the temporal bone and bleeding from the middle meningeal artery. Occasionally it can occur as a result of a bleeding disorder or blood vessel malformation. Diagnosis is typically by a CT scan or MRI. When this condition occurs in the spine it is known as a spinal epidural hematoma.Treatment is generally by urgent surgery in the form of a craniotomy or burr hole. Without treatment, death typically results. The condition occurs in one to four percent of head injuries. Typically it occurs in young adults. Males are more often affected than females. Signs and symptoms
Many people with epidural hematomas experience a lucid period immediately following the injury, with a delay before symptoms become evident. Because of this initial period of lucidity, it has been called "Talk and Die" syndrome. As blood accumulates, it starts to compress intracranial structures, which may impinge on the third cranial nerve, causing a fixed and dilated pupil on the side of the injury. | 0-1
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Oligodendrocyte precursor cells and neural stem cells have been transplanted successfully and have shown to be healthy a year later. Fractional anisotropy and radial diffusivity maps showed possible myelination in the region of the transplant. Induced pluripotent stem cells, oligodendrocyte precursor cells, gene correction, and transplantation to promote the maturation, survival, and myelination of oligodendrocytes seem to be the primary routes for possible treatments.For three types of leukodystrophies (X-linked adrenoleukodystrophy (X-ALD), metachromatic leukodystrophy (MLD) and Krabbe Disease (globoid cell leukodystrophy - GLD), gene therapy using autologous hematopoietic stem cells to transfer the disease gene with lentiviral vectors have shown to be successful and are currently being used in clinical trials for X-ALD and MLD. The progression of X-ALD has shown to be disrupted with hematopoietic stem cell gene therapy but the exact reason why demyelination stops and the amount of stem cells needed is unclear. While there is an accumulation of very long chain fatty acids in the brain, it does not seem to be the reason behind the disease as gene therapy does not correct it.For those leukodystrophies that result from a deficiency of lysozyme enzymes, such as Krabbe disease, enzyme replacement therapy seems hopeful. However, enzyme delivery proves difficult, because the blood-brain barrier severely limits what can pass into the central nervous system. Current gene therapy research for metachromatic leukodystrophy has been reviewed with an emphasis on ex vivo transplantation of genetically modified hematopoietic stem cells. | Nasal vestibulitis is the diffuse dermatitis of nasal vestibule. It is often caused by Staphylococcus aureus. It may be secondary to chronic rhinorrhea, nose picking or viral infections. In acute vestibulitis, the skin is red, swollen and tender. In chronic vestibulitis, induration of vestibular skin and crusting is seen. Antibiotic steroid ointment is sometimes helpful. Chronic fissures can be cauterized with Silver Nitrate. == References == | 0-1
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However, an exercise stress test may provide more information about exercise tolerance than a pharmacologic stress test.Commonly used agents include:
Vasodilators acting as adenosine receptor agonists, such as adenosine itself, and dipyridamole (brand name "Persantine"), which acts indirectly at the receptor. Regadenoson (brand name "Lexiscan"), which acts specifically at the adenosine A2A receptor, thus affecting the heart more than the lung. Dobutamine. The effects of beta-agonists such as dobutamine can be reversed by administering beta-blockers such as propranolol.Lexiscan (Regadenoson) or Dobutamine is often used in patients with severe reactive airway disease (asthma or COPD) as adenosine and dipyridamole can cause acute exacerbation of these conditions. If the patients Asthma is treated with an inhaler then it should be used as a pre-treatment prior to the injection of the pharmacologic stress agent. In addition, if the patient is actively wheezing then the physician should determine the benefits versus the risk to the patient of performing a stress test especially outside of a hospital setting. Caffeine is usually held 24 hours prior to an adenosine stress test, as it is a competitive antagonist of the A2A adenosine receptor and can attenuate the vasodilatory effects of adenosine.Aminophylline may be used to attenuate severe and/or persistent adverse reactions to Adenosine and Lexiscan. Limitations
The stress test does not detect:
Atheroma
Vulnerable plaquesThe test has relatively high rates of false positives and false negatives compared with other clinical tests. Females in particular have a higher rate of false positives, which is theorized to be because on average they have smaller hearts. | Freshwater snails: Pila spp., Pomacea canaliculata, Cipangopaludina chinensis, Bellamya aeruginosa and Bellamya quadrata
Slugs: Limax maximus, Limax flavus Deroceras laeve, Deroceras reticulatum, Laevicaulis alte, Sarasinula plebeia, Vaginulus yuxjsjs, Lehmannia valentiana, Phiolomycus bilineatus, Macrochlamys loana, Meghimatium bilineatum and probably other species of slugs.Definitive hosts of A. cantonensis include wild rodents, especially the brown rat (Rattus norvegicus) and the black rat (Rattus rattus).Paratenic hosts of A. cantonensis include the predatory land flatworm Platydemus manokwari and the amphibians, Bufo asiaticus, Rana catesbeiana, Rhacophorus leucomystax and Rana limnocharis.In 2004, a captive yellow-tailed black cockatoo (Calyptorhynchus funereus) and two free-living tawny frogmouths (Podargus strigoides) suffering neurological symptoms were shown to have the parasite. They were the first avian hosts discovered for the organism. In 2018, in Mallorca two North African hedgehogs with signs of acute neurological disease were found to have A. cantonensis in their brains, one of them with a gravid female. It was the first report of hedgehogs as hosts of Angiostrongylus.The Hawaiʻi Dept. of Health states that fresh water opihi can carry the parasite, as well as other aquatic organisms such as prawns, frogs, and water monitor lizards. House pets may interact with A. cantonensis-carrying animals yet not well studied. Cats are known to carry and spread feline lungworm in rat and snail interactions. Pathogenesis of human angiostrongylosis
The presence of parasitic worms burrowed in the neural tissue of the human central nervous system (CNS) causes complications. | 0-1
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It is only since the late 1800s that narcissism has been defined in psychological terms:
Havelock Ellis (1898) was the first psychologist to use the term when he linked the myth to the condition in one of his patients. Sigmund Freud (1905-1953) used the terms "narcissistic libido" in his Three Essays on the Theory of Sexuality. Ernest Jones (1913/1951) was the first to construe extreme narcissism as a character flaw. Robert Waelder (1925) published the first case study of narcissism. His patient was a successful scientist with an attitude of superiority, an obsession with fostering self-respect, and a lack of normal feelings of guilt. The patient was aloof and independent from others and had an inability to empathize with others situations, and was selfish sexuality. Waelders patient was also overly logical and analytical and valued abstract intellectual thought (thinking for thinkings sake) over the practical application of scientific knowledge.Narcissistic personality was first described by the psychoanalyst Robert Waelder in 1925. The term narcissistic personality disorder (NPD) was coined by Heinz Kohut in 1968. Waelders initial study has been influential in the way narcissism and the clinical disorder Narcissistic personality disorder are defined today
Freudianism and psychoanalysis
Much early history of narcissism and NPD originates from psychoanalysis. Regarding the adult neurotics sense of omnipotence, Sigmund Freud said that "this belief is a frank acknowledgement of a relic of the old megalomania of infancy"; and concluded that: "we can detect an element of megalomania in most other forms of paranoic disorder. | Extravasation of urine refers to the condition where an interruption of the urethra leads to a collection of urine in other cavities, such as the scrotum or the penis in males. It can be associated with a calculus. Mechanism
An injury to the urethra leaving Bucks fascia intact results in a collection of urine (extravasation) limited to the penis, deep to Bucks fascia. However, if the injury to the bulb of the penis results in urethral injury accompanying a tear of the Bucks fascia, then extravasated blood and urine would accumulate in the superficial perineal space, passing into the penis (outer to Bucks fascia) as well as the scrotum and lower anterior abdominal wall. Extravasation of urine involving a compromised Bucks fascia can be appreciated clinically by blood collecting in the superficial pouch, resulting in a butterfly-shaped region around the penis. Urinoma
Long term complications of renal trauma, ureteral obstruction, or kidney transplant can lead to the formation of an urinoma encapsulating extravasated urine. References
== External links == | 0-1
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In addition, mitotane has direct and selective cytotoxic effects on the adrenal cortex, via an unknown mechanism, and thereby induces permanent adrenal atrophy similarly to DDD. Chemistry
Analogues of mitotane include aminoglutethimide, amphenone B, and metyrapone. History
Mitotane was introduced in 1960 for the treatment of adrenocortical carcinoma. Society and culture
Generic names
Mitotane is the generic name of the drug and its INN, USAN, BAN, and JAN. Brand names
Mitotane has been sold under the brand name Lysodren. Veterinary use
Mitotane is also used to treat Cushings disease (pituitary-dependent Cushings syndrome) in dogs. The medication is used in the controlled destruction of adrenal tissue, leading to a decrease in cortisol production. References
External links
V. P. Komissarenko; I. S. Chelnakova; A. S. Mikosha (1978). "Effect of o,p-dichlorodiphenyldichloroethane and perthane in vitro on glutathione reductase activity in the adrenals of dogs and guinea pigs". Bulletin of Experimental Biology and Medicine. 85 (2): 152–154. doi:10.1007/BF00800110. S2CID 23181221. Government of Canada: Benzene, 1-chloro-2-[2,2-dichloro-1-(4-chlorophenyl)ethyl]- (Mitotane)
Environment Canada & Health Canada: RISK MANAGEMENT SCOPE for Benzene, 1-chloro-2-[2,2-dichloro-1-(4-chlorophenyl)ethyl]- (Mitotane), 2013 | Endoscopic means can be used to secure a diagnosis although this may cause a perforation of the inflamed diverticular area. CT scanning is the more common method to diagnose diverticulitis. The scan will show mesenteric stranding in the involved segment of edematous colon which is usually in the sigmoid region. Micro perforations with free air may be seen.Ulcerative colitis or Crohns disease may cause colonic obstruction. The obstruction may be acute or chronic after years of uncontrolled disease leads to the formation of strictures and fistulas. The medical history is helpful in that most cases of inflammatory bowel disease are well known to both patient and doctor. Other rare syndromes, including Ogilvies syndrome, chronic constipation and impaction may cause a pseudo obstruction. Abdominal x-ray – tire-like shadow arising from right iliac fossa and passing to left
Upper GI series
Treatment
Sigmoid
Treatment for sigmoid volvulus may include sigmoidoscopy. If the mucosa of the sigmoid looks normal and pink, a rectal tube for decompression may be placed, and any fluid, electrolyte, cardiac, kidney or pulmonary abnormalities should be corrected. The affected person should then be taken to the operating room for surgical repair. If surgery is not performed, there is a high rate of recurrence.For people with signs of sepsis or an abdominal catastrophe, immediate surgery and resection are advised. Cecal
In a cecal volvulus, the cecum may be returned to a normal position and sutured in place, a procedure known as cecopexy. | 0-1
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This was later resolved with the revision of the DSM-III, which aided many by providing appropriate treatment for patients. BDD was initially considered non-delusional in European research, and was grouped with ‘monosymptomatic hypochondriacal psychoses’ – delusional paranoia disorders, before being introduced in the DSM-III. In 1991, the demographics of individuals who experience BDD were primarily single women aged 19 or older. This statistic has not changed over the decades, women are still considered the predominant gender to experience BDD. With the rise of social media platforms, individuals are easily able to seek validation and openly compare their physical appearance to online influences, finding more flaws and defects in their own appearance. This leads to attempts to conceal the defect such as seeking out surgeons to resolve the issue of ugliness.Universally, it is evident that different cultures place much emphasis on correcting the human body aesthetic, and that this preoccupation with body image is not exclusive to just one society; one example is the binding of women’s feet in Chinese culture.Whilst physically editing the body is not unique to any one culture, research suggests that it is more common throughout Western society and is on the rise. On close observation of contemporary Western societies, there has been an increase in disorders such as Body dysmorphic disorder, arising from ideals around the aesthetic of the human body. Scholars such as Nancy Scheper-Hughes have suggested such demand placed upon Western bodies has been around since the beginning of the 19th century, and that it has been driven by sexuality. | The rash involves the palms or soles in as many as 80% of people. However, this distribution may not occur until later on in the course of the disease. As many as 15% of patients may never develop a rash. Complications
People can develop permanent disabilities including "cognitive deficits, ataxia, hemiparesis, blindness, deafness, or amputation following gangrene". Cause
Ticks are the natural hosts of the disease, serving as both reservoirs and vectors of R. rickettsii. Ticks transmit the bacteria primarily by their bites. Less commonly, infections may occur following exposure to crushed tick tissues, fluids, or tick feces. A female tick can transmit R. rickettsii to her eggs in a process called transovarial transmission. Ticks can also become infected with R. rickettsii while feeding on blood from the host in either the larval or nymphal stage. After the tick develops into the next stage, the R. rickettsii may be transmitted to the second host during the feeding process. Furthermore, male ticks may transfer R. rickettsii to female ticks through body fluids or spermatozoa during the mating process. These types of transmission represent how generations or life stages of infected ticks are maintained. Once infected, the tick can carry the pathogen for life.Rickettsiae are transmitted through saliva injected while a tick is feeding. Unlike Lyme disease and other tick-borne pathogens that require a prolonged attachment period to establish infection, a person can become infected with R. rickettsii in a feeding time as short as 2 hours. | 0-1
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Epidemiology
This disease is endemic in Portuguese locations Póvoa de Varzim and Vila do Conde (Caxinas), with more than 1000 affected people, coming from about 500 families, where 70% of the people develop the illness. ll the analysed Portuguese families presented the same haplotype (haplotype I) associated with the Met 30 mutation. In northern Sweden, more specifically Skellefteå (it is locally called "Skelleftesjukan", the Skellefteå disease), 1.5% of the population has the mutated gene. There are many other populations in the world who exhibit the illness after having developed it independently. References
External links
GeneReviews/NIH/NCBI/UW entry on Familial Transthyretin Amyloidosis | Crohns disease typically affects fairly well demarcated segments of bowel in the colon and often affects the end of the small bowel. Irritable bowel syndrome
Another possible cause of diarrhea is irritable bowel syndrome (IBS), which usually presents with abdominal discomfort relieved by defecation and unusual stool (diarrhea or constipation) for at least three days a week over the previous three months. Symptoms of diarrhea-predominant IBS can be managed through a combination of dietary changes, soluble fiber supplements and medications such as loperamide or codeine. About 30% of patients with diarrhea-predominant IBS have bile acid malabsorption diagnosed with an abnormal SeHCAT test. Other diseases
Diarrhea can be caused by other diseases and conditions, namely:
Chronic ethanol ingestion
Hyperthyroidism
Certain medications
Bile acid malabsorption
Ischemic bowel disease: This usually affects older people and can be due to blocked arteries. Microscopic colitis, a type of inflammatory bowel disease where changes are seen only on histological examination of colonic biopsies. Bile salt malabsorption (primary bile acid diarrhea) where excessive bile acids in the colon produce a secretory diarrhea. Hormone-secreting tumors: some hormones, e.g. serotonin, can cause diarrhea if secreted in excess (usually from a tumor). Chronic mild diarrhea in infants and toddlers may occur with no obvious cause and with no other ill effects; this condition is called toddlers diarrhea. Environmental enteropathy
Radiation enteropathy following treatment for pelvic and abdominal cancers. Medications
Some medications, such as the penicillin can cause diarrhea. Over 700 medications are known to cause diarrhea. | 0-1
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Treatment methodology is informed by the presence of comorbid conditions. Medication
In the case of pathological gambling, along with fluvoxamine, clomipramine has been shown effective in the treatment, with reducing the problems of pathological gambling in a subject by up to 90%. Whereas in trichotillomania, the use of clomipramine has again been found to be effective, fluoxetine has not produced consistent positive results. Fluoxetine, however, has produced positive results in the treatment of pathological skin picking disorder, although more research is needed to conclude this information. Fluoxetine has also been evaluated in treating IED and demonstrated significant improvement in reducing frequency and severity of impulsive aggression and irritability in a sample of 100 subjects who were randomized into a 14-week, double-blind study. Despite a large decrease in impulsive aggression behavior from baseline, only 44% of fluoxetine responders and 29% of all fluoxetine subjects were considered to be in full remission at the end of the study. Paroxetine has shown to be somewhat effective although the results are inconsistent. Another medication, escitalopram, has shown to improve the condition of the subjects of pathological gambling with anxiety symptoms. The results suggest that although SSRIs have shown positive results in the treatment of pathological gambling, inconsistent results with the use of SSRIs have been obtained which might suggest a neurological heterogeneity in the impulse-control disorder spectrum. Psychosocial
The psychosocial approach to the treatment of ICDs includes cognitive behavioral therapy (CBT) which has been reported to have positive results in the case of treatment of pathological gambling and sexual addiction. | However, research conducted in the early 1990s in the United States gave prevalence estimates between 5–6% in the U.S. population, with male cases being higher than female. Internet addiction
The disorder of Internet addiction has only recently been taken into consideration and has been added as a form of ICD. It is characterized by excessive and damaging usage of Internet with increased amount of time spent chatting, web surfing, gambling, shopping or consuming pornography. Excessive and problematic Internet use has been reported across all age, social, economic, and educational ranges. Although initially thought to occur mostly in males, increasing rates have been also observed in females. However, no epidemiological study has been conducted yet to understand its prevalence. Compulsive shopping
Compulsive shopping or buying is characterized by a frequent irresistible urge to shop even if the purchases are not needed or cannot be afforded. The prevalence of compulsive buying in the U.S. has been estimated to be 2–8% of the general adult population, with 80–95% of these cases being females. The onset is believed to occur in late teens or early twenties and the disorder is considered to be generally chronic. Pyromania
Pyromania is characterized by impulsive and repetitive urges to deliberately start fires. Because of its nature, the number of studies performed for fire-setting are understandably limited. However, studies done on children and adolescents with pyromania have reported its prevalence to be between 2.4 and 3.5% in the United States. | 11
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Richters syndrome (RS), also known as Richters transformation, is a transformation of B cell chronic lymphocytic leukemia (CLL) or hairy cell leukemia into a fast-growing diffuse large B cell lymphoma, a variety of non-Hodgkin lymphoma which is refractory to treatment and carries a bad prognosis. There is also a less common variant in which the CLL changes into a Hodgkins lymphoma. Rarely, transformations to a form of myeloid leukemia have been observed. These extraordinarily rare transformations carry a very poor prognosis. Richters transformation affects about 5% of CLL patients at some point during their lives. Signs and symptoms
Symptoms of Richter’s transformation in a CLL patient include fever (without infection), elevated serum levels of lactate dehydrogenase, and rapidly enlarging lymph nodes. While about 8% of all CLL patients will have elevated levels of serum lactate dehydrogenase (LDH), more than 50% of CLL patients with Richters transformation will have elevated LDH levels.Richters can appear suddenly, even in patients who were in remission. Cause
A case of RS may have arisen by one of two different routes: a transformation of the CLL cells into lymphoma, or the appearance of an unrelated lymphoma.It is thought that genetic defects may introduce the additional abnormalities necessary to transform CLL cells into Richters syndrome cells. Treatment
Treatment with conventional immunochemotherapy is usually indicated; in younger patients, allogeneic bone marrow transplantation may be curative. Prognosis
The prognosis is generally poor. | Article 25 of the Universal Declaration of Human Rights, adopted 10 December 1948 by the UN General Assembly, contains this text regarding housing and quality of living:
Everyone has the right to a standard of living adequate for the health and well-being of himself and of his family, including food, clothing, housing, and medical care and necessary social services, and the right to security in the event of unemployment, sickness, disability, widowhood, old age or other lack of livelihood in circumstances beyond his control. The ETHOS Typology of Homelessness and Housing Exclusion was developed as a means of improving understanding and measurement of homelessness in Europe, and to provide a common "language" for transnational exchanges on homelessness. The ETHOS approach confirms that homelessness is a process (rather than a static phenomenon) that affects many vulnerable households at different points in their lives.The typology was launched in 2005 and is used for different purposes: as a framework for debate, for data collection purposes, for policy purposes, monitoring purposes, and in the media. This typology is an open exercise that makes abstraction of existing legal definitions in the EU member states. It exists in 25 language versions, the translations being provided mainly by volunteer translators. Despite all the international agreements and efforts, many countries and individuals in power do not consider housing as a human right. Former US President, Jimmy Carter, addressed this issue in a 2017 interview. | 0-1
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Bruns nystagmus is an unusual type of bilateral nystagmus most commonly occurring in patients with cerebellopontine angle tumours. It is caused by the combination of slow, large amplitude nystagmus (gaze paretic nystagmus) when looking towards the side of the lesion, and rapid, small amplitude nystagmus (vestibular nystagmus) when looking away from the side of the lesion. It occurs in 11% of patients with vestibular schwannoma, and occurs mainly in patients with larger tumours (67% of patients with tumours over 3.5 cm diameter). Bruns nystagmus is also associated with an increased incidence of balance disturbance in patients with vestibular schwannoma. It may be caused by the compression of both flocculi, the vestibular part of the cerebellum, and improvement in both the nystagmus and balance problems occur commonly after removal of the tumour.Bruns nystagmus is named for Ludwig Bruns (1858 – 1915). == References == | Cambia can mean:
Cambia, Haute-Corse, a commune in the Haute-Corse department of France
Cambia (non-profit organization), an open science and biology non-profit institute based in Australia
Cambia Health Solutions, an American health insurance company
Cambia or cambiums, the four humours in medicine
Cambia, a trade name for the anti-inflammatory drug diclofenac
the plural of cambium (botany), a type of tissue found in plants
See also
Cambio (disambiguation)
Cambria (disambiguation)
Kambia (disambiguation)
Cumbia, a style of music and dance
Cumbria, a county of England | 0-1
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In particular, cases of bilateral Wilms tumor, as well as cases of Wilms tumor derived from certain genetic syndromes such as Denys-Drash syndrome, are strongly associated with nephrogenic rests. Most nephroblastomas are on one side of the body only and are found on both sides in less than 5% of cases, although people with Denys-Drash syndrome mostly have bilateral or multiple tumors. They tend to be encapsulated and vascularized tumors that do not cross the midline of the abdomen. In cases of metastasis it is usually to the lung. A rupture of Wilms tumor puts the patient at risk of bleeding and peritoneal dissemination of the tumor. In such cases, surgical intervention by a surgeon who is experienced in the removal of such a fragile tumor is imperative.Pathologically, a triphasic nephroblastoma comprises three elements:
blastema
mesenchyme (stroma)
epitheliumWilms tumor is a malignant tumor containing metanephric blastema, stromal and epithelial derivatives. Characteristic is the presence of abortive tubules and glomeruli surrounded by a spindled cell stroma. The stroma may include striated muscle, cartilage, bone, fat tissue, and fibrous tissue. | CGPD is more common in boys than girls. History
Gianotti et al. first described CGPD in five Italian children in 1970. In 1990, Williams et al. described a similar skin eruption in five children of Afro-Caribbean descent and coined the proposed term "facial Afro-Caribbean childhood eruption (FACE)". Subsequently, another article by Katz and Lesher first introduced the term CGPD since some reported cases were not found in children of Afro-Caribbean descent and to avoid confusion with perioral dermatitis. See also
Perioral dermatitis
== References == | 0-1
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Instead, the left heart fails to pump blood efficiently, leading to pooling of blood in the lungs and back pressure within the pulmonary system. This causes pulmonary edema and pleural effusions. In the absence of pulmonary blood vessel narrowing, the increased back pressure is described as isolated post-capillary pulmonary hypertension (older terms include passive or proportionate pulmonary hypertension or pulmonary venous hypertension). However, in some patients, the raised pressure in the pulmonary vessels triggers a superimposed component of vessel narrowing, which further increases the workload of the right side of the heart. This is referred to as post-capillary pulmonary hypertension with a pre-capillary component or combined post-capillary and pre-capillary pulmonary hypertension (older terms include reactive or out-of-proportion pulmonary hypertension).In pulmonary hypertension due to lung diseases and/or hypoxia (WHO Group III), low levels of oxygen in the alveoli (due to respiratory disease or living at high altitude) cause constriction of the pulmonary arteries. This phenomenon is called hypoxic pulmonary vasoconstriction and it is initially a protective response designed to stop too much blood flowing to areas of the lung that are damaged and do not contain oxygen. When the alveolar hypoxia is widespread and prolonged, this hypoxia-mediated vasoconstriction occurs across a large portion of the pulmonary vascular bed and leads to an increase in pulmonary arterial pressure, with thickening of the pulmonary vessel walls contributing to the development of sustained pulmonary hypertension. | Prolonged hypoxia also induces the transcription factor HIF1A, which directly activates downstream growth factor signaling that causes irreversible proliferation and remodeling of pulmonary arterial endothelial cells, leading to chronic pulmonary arterial hypertension.In CTEPH (WHO Group IV), the initiating event is thought to be blockage or narrowing of the pulmonary blood vessels with unresolved blood clots; these clots can lead to increased pressure and shear stress in the rest of the pulmonary circulation, precipitating structural changes in the vessel walls (remodeling) similar to those observed in other types of severe pulmonary hypertension. This combination of vessel occlusion and vascular remodeling once again increases the resistance to blood flow and so the pressure within the system rises. Molecular pathology
The molecular mechanism of pulmonary arterial hypertension (PAH) is not known yet, but it is believed that the endothelial dysfunction results in a decrease in the synthesis of endothelium-derived vasodilators such as nitric oxide and prostacyclin. Moreover, there is a stimulation of the synthesis of vasoconstrictors such as thromboxane and vascular endothelial growth factor (VEGF). These result in a severe vasoconstriction and vascular smooth muscle and adventitial hypertrophy characteristic of patients with PAH. Nitric oxide-soluble guanylate cyclase pathway
In normal conditions, the vascular endothelial nitric oxide synthase produces nitric oxide from L-arginine in the presence of oxygen.This nitric oxide diffuses into neighboring cells (including vascular smooth muscle cells and platelets), where it increases the activity of the enzyme soluble guanylate cyclase, leading to increased formation of cyclic guanosine monophosphate (cGMP) from guanosine triphosphate (GTP). | 11
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It should not be used in people with sick sinus syndrome, long QT syndrome, cardiogenic shock, uncontrolled heart failure, asthma or a related bronchospastic condition, or people with serum potassium below 4 meq/L. It should only be used in people with a second and third degree AV block if a functioning pacemaker is present.Since sotalol is removed from the body through the kidneys, it should not be used in people with a creatinine clearance rate below 40 mL/min. It is also excreted in breast milk, so mothers should not breastfeed while taking sotalol.Since sotalol prolongs the QT interval, the FDA recommends against using it in conjunction with other medications that prolong the QT interval. Studies have found serious side effects to be more common in individuals also taking digoxin, possibly because of pre-existing heart failure in those people. As with other beta blockers, it may interact with calcium channel blockers, catecholamine-depleting drugs, insulin or antidiabetic drugs, beta2-adrenergic agonists, and clonidine.Some evidence suggests that sotalol should be avoided in the setting of heart failure with a reduced ejection fraction (resulting in the heart squeezing little blood out into the circulation with each pump) due to an increased risk of death. Adverse effects
Over 10% of oral sotalol users experience fatigue, dizziness, lightheadedness, headache, weakness, nausea, shortness of breath, bradycardia (slow heart rate), a sensation of the heart beating too hard, fast, or irregularly, or chest pain. | In heart cells, calcium is important in generating electrical signals for heart muscle contraction, as well as generating force for this contraction. In consideration of these important properties of calcium, two conclusions can be drawn. First, with less calcium in the cell, there is a decrease in electrical signals for contraction, thus allowing time for the hearts natural pacemaker to rectify arrhythmic contractions. Secondly, lower calcium means a decrease in strength and rate of the contractions, which can be helpful in treatment of abnormally fast heart rates. Type III antiarrhythmic action
Sotalol also acts on potassium channels and causes a delay in relaxation of the ventricles. By blocking these potassium channels, sotalol inhibits efflux of K+ ions, which results in an increase in the time before another electrical signal can be generated in ventricular myocytes. This increase in the period before a new signal for contraction is generated, helps to correct arrhythmias by reducing the potential for premature or abnormal contraction of the ventricles but also prolongs the frequency of ventricular contraction to help treat tachycardia. History
Sotalol was first synthesized in 1960 by A. A. Larsen of Mead-Johnson Pharmaceutical. It was originally recognized for its blood pressure lowering effects and its ability to reduce the symptoms of angina. It was made available in the United Kingdom and France in 1974, Germany in 1975, and Sweden in 1979. It became widely used in the 1980s. In the 1980s, its antiarrhythmic properties were discovered. The United States approved the drug in 1992. | 11
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It is usually not effective in second-degree heart block Mobitz type 2, and in third-degree heart block with a low Purkinje or ventricular escape rhythm.Atropine has also been used in an effort to prevent a low heart rate during intubation of children; however, evidence does not support this use. Secretions
Atropines actions on the parasympathetic nervous system inhibit salivary and mucus glands. The drug may also inhibit sweating via the sympathetic nervous system. This can be useful in treating hyperhidrosis, and can prevent the death rattle of dying patients. Even though atropine has not been officially indicated for either of these purposes by the FDA, it has been used by physicians for these purposes. Poisonings
Atropine is not an actual antidote for organophosphate poisoning. However, by blocking the action of acetylcholine at muscarinic receptors, atropine also serves as a treatment for poisoning by organophosphate insecticides and nerve agents, such as tabun (GA), sarin (GB), soman (GD), and VX. Troops who are likely to be attacked with chemical weapons often carry autoinjectors with atropine and an oxime, for rapid injection into the muscles of the thigh. In a developed case of nerve-gas poisoning, maximum atropinization is desirable. Atropine is often used in conjunction with the oxime pralidoxime chloride. Some of the nerve agents attack and destroy acetylcholinesterase by phosphorylation, so the action of acetylcholine becomes excessive and prolonged. Pralidoxime (2-PAM) can be effective against organophosphate poisoning because it can re-cleave this phosphorylation. | Atropine degrades slowly, typically wearing off in 7 to 14 days, so it is generally used as a therapeutic mydriatic, whereas tropicamide (a shorter-acting cholinergic antagonist) or phenylephrine (an α-adrenergic agonist) is preferred as an aid to ophthalmic examination.In refractive and accommodative amblyopia, when occlusion is not appropriate sometimes atropine is given to induce blur in the good eye. Evidence suggests that atropine penalization is just as effective as occlusion in improving visual acuity. Antimuscarinic topical medication is effective in slowing myopia progression in children; accommodation difficulties and papillae and follicles are possible side-effects. All doses of atropine appear similarly effective, while higher doses have greater side effects. The lower dose of 0.01% is thus generally recommended due to fewer side effects and potential less rebound worsening when the atropine is stopped. Heart
Injections of atropine are used in the treatment of symptomatic or unstable bradycardia. Atropine was previously included in international resuscitation guidelines for use in cardiac arrest associated with asystole and PEA, but was removed from these guidelines in 2010 due to a lack of evidence for its effectiveness. For symptomatic bradycardia, the usual dosage is 0.5 to 1 mg IV push, may repeat every 3 to 5 minutes up to a total dose of 3 mg (maximum 0.04 mg/kg).Atropine is also useful in treating second-degree heart block Mobitz type 1 (Wenckebach block), and also third-degree heart block with a high Purkinje or AV-nodal escape rhythm. | 11
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However, they also showed that the compound was likely to cause birth defects, so in light of the events around thalidomide, Roche abandoned the product. In 1979, the New England Journal of Medicine published an article demonstrating the drugs effectiveness as a treatment of cystic and conglobate acne on fourteen patients, thirteen of which experienced complete clearing of their disease. Roche resumed work on the drug. In clinical trials, subjects were carefully screened to avoid including women who were or might become pregnant. Roches New Drug Application for isotretinoin for the treatment of acne included data showing that the drug caused birth defects in rabbits. The FDA approved the application in 1982. Scientists involved in the clinical trials published articles warning of birth defects at the same time the drug was launched in the US, but nonetheless, isotretinoin was taken up quickly and widely, both among dermatologists and general practitioners. Cases of birth defects showed up in the first year, leading the FDA to begin publishing case reports and to Roche sending warning letters to doctors and placing warning stickers on drug bottles, and including stronger warnings on the label. Lawsuits against Roche started to be filed. In 1983 the FDAs advisory committee was convened and recommended stronger measures, which the FDA took and were that time unprecedented: warning blood banks not to accept blood from people taking the drug and adding a warning to the label advising women to start taking contraceptives a month before starting the drug. | Isotretinoin, also known as 13-cis-retinoic acid and sold under the brand name Accutane among others, is a medication primarily used to treat severe acne. It is also used to prevent certain skin cancers (squamous-cell carcinoma), and in the treatment of other cancers. It is used to treat harlequin-type ichthyosis, a usually lethal skin disease, and lamellar ichthyosis. It is a retinoid, meaning it is related to vitamin A, and is found in small quantities naturally in the body. Its isomer, tretinoin, is also an acne drug. The most common adverse effects are dry lips (cheilitis), dry and fragile skin, and an increased susceptibility to sunburn. Uncommon and rare side effects include muscle aches and pains (myalgias), and headaches. Isotretinoin is known to cause birth defects due to in-utero exposure because of the molecules close resemblance to retinoic acid, a natural vitamin A derivative that controls normal embryonic development. It is also associated with psychiatric side effects, most commonly depression but also, more rarely, psychosis and unusual behaviours. Other rare side effects include hyperostosis and premature epiphyseal closure, which have been reported to be persistent. In the United States, a special procedure is required to obtain the pharmaceutical. In most other countries, a consent form is required which explains these risks. In other countries, it is prescribed like any other medicine from a dermatologist (after proper blood tests). Women taking isotretinoin must not get pregnant during and for one month after the discontinuation of isotretinoin therapy. | 11
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Sepsis can cause liver, kidney and heart damage. Respiratory failure - CAP patients often have dyspnea, which may require support. Non-invasive machines (such as bilevel positive airway pressure), a tracheal tube or a ventilator may be used. Pleural effusion and empyema - Microorganisms from the lung may trigger fluid collection in the pleural cavity, or empyema. Pleural fluid, if present, should be collected with a needle and examined. Depending on the results, complete drainage of the fluid with a chest tube may be necessary to prevent proliferation of the infection. Antibiotics, which do not penetrate the pleural cavity well, are less effective. Abscess - A pocket of fluid and bacteria may appear on X-ray as a cavity in the lung. Abscesses, typical of aspiration pneumonia, usually contain a mixture of anaerobic bacteria. Although antibiotics can usually cure abscesses, sometimes they require drainage by a surgeon or radiologist. Causes
Many different microorganisms can cause CAP. However, the most common cause is Streptococcus pneumoniae. Certain groups of people are more susceptible to CAP-causing pathogens - infants, adults with chronic conditions (such as chronic obstructive pulmonary disease), and senior citizens. Alcoholics and others with compromised immune systems are more likely to develop CAP from Haemophilus influenzae or Pneumocystis jirovecii. A definitive cause is identified in only half the cases. Neonates and infants
It is possible for a fetus to develop a lung infection before birth by aspirating infected amniotic fluid or through a blood-borne infection which crossed the placenta. Infants can also inhale contaminated fluid from the vagina at birth. | The most prevalent pathogen causing CAP in newborns is Streptococcus agalactiae, also known as group-B streptococcus (GBS). GBS causes more than half of CAP in the first week after birth. Other bacterial causes of neonatal CAP include Listeria monocytogenes and a variety of mycobacteria. CAP-causing viruses may also be transferred from mother to child; herpes simplex virus, the most common, is life-threatening, and adenoviridae, mumps and enterovirus can also cause pneumonia. Another cause of neonatal CAP is Chlamydia trachomatis, which, though acquired at birth, does not cause pneumonia until two to four weeks later. It usually presents with no fever and a characteristic, staccato cough. CAP in older infants reflects increased exposure to microorganisms, with common bacterial causes including Streptococcus pneumoniae, Escherichia coli, Klebsiella pneumoniae, Moraxella catarrhalis and Staphylococcus aureus. Maternally-derived syphilis is also a cause of CAP in infants. Viral causes include human respiratory syncytial virus (RSV), human metapneumovirus, adenovirus, human parainfluenza viruses, influenza and rhinovirus, and RSV is a common source of illness and hospitalization in infants. CAP caused by fungi or parasites is not usually seen in otherwise-healthy infants. Children
Although children older than one month tend to be at risk for the same microorganisms as adults, children under five years of age are much less likely to have pneumonia caused by Mycoplasma pneumoniae, Chlamydophila pneumoniae or Legionella pneumophila than older children. In contrast, older children and teenagers are more likely to acquire Mycoplasma pneumoniae and Chlamydophila pneumoniae than adults. | 11
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Tumoral calcinosis is a rare condition in which there is calcium deposition in the soft tissue in periarticular location, around joints, outside the joint capsule. They are frequently (0.5–3%) seen in patients undergoing renal dialysis. Clinically also known as hyperphosphatemic familial tumoral calcinosis (HFTC), is often caused by genetic mutations in genes that regulate phosphate physiology in the body (leading to too much phosphate (hyperphosphatemia)). Best described genes that harbour mutations in humans are FGF-23, Klotho (KL), or GALNT3. A zebrafish animal model with reduced GALNT3 expression also showed HFTC-like phenotype, indicating an evolutionary conserved mechanism that is involved in developing tumoral calcinosis. Clinical features
The name indicates calcinosis (calcium deposition) which resembles tumor (like a new growth). They are not true neoplasms – they dont have dividing cells. They are just deposition of inorganic calcium with serum exudate. Children and adolescents (6 to 25 years) are the most commonly affected. The symptom that the accumulations cause is not pain but swelling around joints. They have propensity to enlarge progressively and ulcerate the overlying skin and extrude. They are most common around shoulders, hips and elbows. Laboratory evaluation reveal normal serum calcium levels and hyperphosphatemia. Rarely ALP (alkaline phosphatase – an enzyme active at sites of bone formation) may be elevated. Treatment is normalization of serum phosphate levels and resection of lesions. Surgical removal should be complete and if part of it is left, recurrence is likely to occur. Cutting through the excised calcium deposition reveals semifluid calcium suspension in albumin encapsulated by fibrous tissue. | Ichthyosis hystrix is a group of rare skin disorders in the ichthyosis family of skin disorders characterized by massive hyperkeratosis with an appearance like spiny scales. This term is also used to refer to a type of epidermal nevi with extensive bilateral distribution. Types
Ichthyosis hystrix, Curth-Macklin type
The symptoms of ichthyosis hystrix Curth-Macklin are similar to epidermolytic hyperkeratosis (NPS-2 type) but there is no blistering and the hyperkeratosis is verrucous or spine-like. The hyperkeratosis is brown-grey in colour and is most obvious on the arms and legs. It is an autosomal dominant condition and can be caused by errors to the KRT1 gene. It is named after Helen Ollendorff Curth (1899-1982), a German-Jewish dermatologist, and Madge Thurlow Macklin (1893–1962), an American medical geneticist, and is one of the first syndromes named after two women. Ichthyosis hystrix, Lambert type
Also known as ichthyosis hystrix gravior or porcupine man. This disease is characterised by spiny scales which cover the entire body except the face, genitals, palms and soles. The only known cases were in Edward Lambert (known as the porcupine man) who was exhibited in front of the Royal Society in London in 1731 and three generations of his descendants. No cases of this disease are now known though some experts believe that it may have been a type of epidermolytic hyperkeratosis. From the history of the Lambert family the disease appears to have been an autosomal dominant condition. | 0-1
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In children, spontaneous resolution of EDP over the course of months to years is possible; however, this outcome is less likely if EDP presents in adulthood. : 224
See also
Lichen planus
List of cutaneous conditions
References
== External links == | Fedratinib, sold under the brand name Inrebic, is an anti-cancer medication used to treat myeloproliferative diseases including myelofibrosis. It is used in the form of fedratinib hydrochloride capsules that are taken by mouth. It is a semi-selective inhibitor of Janus kinase 2 (JAK-2). It was approved by the FDA on 16 August 2019.Myelofibrosis is a myeloid cancer associated with anemia, splenomegaly, and constitutional symptoms. Patients with myelofibrosis frequently harbor mutations which activate the JAK-STAT signaling pathway and which are sensitive to fedratinib. Phase I trial results focused on safety and efficacy of fedratinib in patients with high- or intermediate-risk primary or post–polycythemia vera/essential thrombocythemia myelofibrosis have been published in 2011. Medical uses
In the United States, fedratinib is indicated for the treatment of adults with intermediate-2 or high-risk primary or secondary (following polycythemia vera or essential thrombocythemia) myelofibrosis.In the European Union, fedratinib is indicated for the treatment of disease-related splenomegaly or symptoms in adults with primary myelofibrosis, following polycythaemia vera or essential thrombocythaemia, who are Janus kinase (JAK) inhibitor naïve or have been treated with ruxolitinib. Pharmacology
Mechanism of action
Fedratinib acts as a competitive inhibitor of protein kinase JAK-2 with IC50=6 nM; related kinases FLT3 and RET are also sensitive, with IC50=25 nM and IC50=17 nM, respectively. Significantly less activity was observed against other tyrosine kinases including JAK3 (IC50=169 nM). In treated cells the inhibitor blocks downstream cellular signalling (JAK-STAT) leading to suppression of proliferation and induction of apoptosis. History
Fedratinib was originally discovered at TargeGen. In 2010, Sanofi-Aventis acquired TargeGen and continued development of fedratinib until 2013. | 0-1
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Toxicity
Azacitidine causes anemia (low red blood cell counts), neutropenia (low white blood cell counts), and thrombocytopenia (low platelet counts), and patients should have frequent monitoring of their complete blood counts, at least prior to each dosing cycle. The dose may have to be adjusted based on nadir counts and hematologic response.It can also be hepatotoxic in patients with severe liver impairment, and patients with extensive liver tumors due to metastatic disease have developed progressive hepatic coma and death during azacitidine treatment, especially when their albumin levels are less than 30 g/L. It is contraindicated in patients with advanced malignant hepatic tumors.Kidney toxicity, ranging from elevated serum creatinine to kidney failure and death, have been reported in patients treated with intravenous azacitidine in combination with other chemotherapeutic agents for conditions other than myelodysplastic syndrome. Renal tubular acidosis developed in five patients with chronic myelogenous leukemia (an unapproved use) treated with azacitidine and etoposide, and patients with renal impairment may be at increased risk for renal toxicity. Azacitidine and its metabolites are primarily excreted by the kidneys, so patients with chronic kidney disease should be closely monitored for other side effects, since their levels of azacitidine may progressively increase.Based on animal studies and its mechanism of action, azacitidine can cause severe fetal damage. | Agenesis of the corpus callosum (ACC) is a rare birth defect in which there is a complete or partial absence of the corpus callosum. It occurs when the development of the corpus callosum, the band of white matter connecting the two hemispheres in the brain, in the embryo is disrupted. The result of this is that the fibers that would otherwise form the corpus callosum are instead longitudinally oriented along the ipsilateral ventricular wall and form structures called Probst bundles. In addition to agenesis, other degrees of callosal defects exist, including hypoplasia (underdevelopment or thinness), hypogenesis (partial agenesis) or dysgenesis (malformation).ACC is found in many syndromes and can often present alongside hypoplasia of the cerebellar vermis. When this is the case, there can also be an enlarged fourth ventricle or hydrocephalus; this is called Dandy–Walker malformation. Signs and symptoms
Signs and symptoms of ACC and other callosal disorders vary greatly among individuals. However, some characteristics common in individuals with callosal disorders include vision impairments, low muscle tone (hypotonia), poor motor coordination, delays in motor milestones such as sitting and walking, delayed toilet training and dysautonomic symptoms such as low perception of pain or chewing and swallowing difficulties.Laboratory research has demonstrated that individuals with ACC have difficulty transferring more complex information from one hemisphere to the other. They also have been shown to have some cognitive disabilities (difficulty in complex problem solving) and social difficulties (missing subtle social cues), even when their intelligence quotient is normal. | 0-1
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Researchers also did a cohort study on 19,397 babies, from their birth until age seven and discovered that high weight babies at four months were 1.38 times more likely to be overweight at seven years old compared to normal weight babies. High weight babies at the age of one were 1.17 times more likely to be overweight at age seven compared to normal weight babies. Medical illness
Cushings syndrome (a condition in which the body contains excess amounts of cortisol) may also influence childhood obesity. Researchers analyzed two isoforms (proteins that have the same purpose as other proteins, but are programmed by different genes) in the cells of 16 adults undergoing abdominal surgery. They discovered that one type of isoform created oxo-reductase activity (the alteration of cortisone to cortisol) and this activity increased 127.5 pmol mg sup when the other type of isoform was treated with cortisol and insulin. The activity of the cortisol and insulin can possibly activate Cushings syndrome.Hypothyroidism is a hormonal cause of obesity, but it does not significantly affect obese people who have it more than obese people who do not have it. In a comparison of 108 obese patients with hypothyroidism to 131 obese patients without hypothyroidism, researchers discovered that those with hypothyroidism had only 0.077 points more on the caloric intake scale than did those without hypothyroidism. Psychological factors
Researchers surveyed 1,520 children, ages 9–10, with a four-year follow up and discovered a positive correlation between obesity and low self-esteem in the four-year follow up. | In a fitness survey of 6,000 adults, researchers discovered that 25% of those who were considered active at ages 14 to 19 were also active adults, compared to 2% of those who were inactive at ages 14 to 19, who were now said to be active adults. Staying physically inactive leaves unused energy in the body, most of which is stored as fat. Researchers studied 16 men over a 14-day period and fed them 50% more of their energy required every day through fats and carbohydrates. They discovered that carbohydrate overfeeding produced 75–85% excess energy being stored as body fat and fat overfeeding produced 90–95% storage of excess energy as body fat.Many children fail to exercise because they spend long periods of time engaging in sedentary activities such as computer usage, playing video games or watching television. Technology has a large factor on the childrens activeness. Researchers provided a technology questionnaire to 4,561 children, ages 14, 16, and 18. They discovered children were 21.5% more likely to be overweight when watching 4+ hours of TV per day, 4.5% more likely to be overweight when using a computer one or more hours per day, and unaffected by potential weight gain from playing video games. A randomized trial showed that reducing TV viewing and computer use can decrease age-adjusted BMI; reduced calorie intake was thought to be the greatest contributor to the BMI decrease.Technological activities are not the only household influences of childhood obesity. Low-income households can affect a childs tendency to gain weight. | 11
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Diagnosis
Clinical evaluation
Female infants with classic CAH have ambiguous genitalia due to exposure to high concentrations of androgens in utero. CAH due to 21-hydroxylase deficiency is the most common cause of ambiguous genitalia in genotypically normal female infants (46XX). Less severely affected females may present with early pubarche. Young women may present with symptoms of polycystic ovarian syndrome (oligomenorrhea, polycystic ovaries, hirsutism).Males with classic CAH generally have no signs of CAH at birth. Some may present with hyperpigmentation, due to co-secretion with melanocyte-stimulating hormone, and possible penile enlargement. Age of diagnosis of males with CAH varies and depends on the severity of aldosterone deficiency. Boys with salt-wasting disease present early with symptoms of hyponatremia and hypovolemia. Boys with non-salt-wasting disease present later with signs of virilization.In rarer forms of CAH, males are undermasculinized and females generally have no signs or symptoms at birth. Laboratory studies
Genetic analysis can be helpful to confirm a diagnosis of CAH, but it is not necessary if classic clinical and laboratory findings are present. In classic 21-hydroxylase deficiency, laboratory studies will show:
Hypoglycemia (due to hypocortisolism) - One of cortisols many functions is to increase blood glucose levels. This occurs via a combination of several mechanisms, including (a) the stimulation of gluconeogesis (i.e. the creation of new glucose) in the liver, (b) the promotion of glycogenolysis (i.e. the breakdown of glycogen into glucose), and (c) the prevention of glucose leaving the bloodstream via the downregulation of GLUT-4 receptors (which normally promote movement of glucose from the bloodstream into adipose and muscle tissues). | Classification
Cortisol is an adrenal steroid hormone required for normal endocrine function. Production begins in the second month of fetal life. Poor cortisol production is a hallmark of most forms of CAH. Inefficient cortisol production results in rising levels of ACTH, because cortisol feeds back to inhibit ACTH production, so loss of cortisol results in increased ACTH. This increased ACTH stimulation induces overgrowth (hyperplasia) and overactivity of the steroid-producing cells of the adrenal cortex. The defects causing adrenal hyperplasia are congenital (i.e. present at birth). Cortisol deficiency in CAH is usually partial, and not the most serious problem for an affected person. Synthesis of cortisol shares steps with synthesis of mineralocorticoids such as aldosterone, androgens such as testosterone, and estrogens such as estradiol. The resulting excessive or deficient production of these three classes of hormones produce the most important problems for people with CAH. Specific enzyme inefficiencies are associated with characteristic patterns of over- or underproduction of mineralocorticoids or sex steroids. Since the 1960s, most endocrinologists have referred to the forms of CAH by the traditional names in the left column, which generally correspond to the deficient enzyme activity. As exact structures and genes for the enzymes were identified in the 1980s, most of the enzymes were found to be cytochrome P450 oxidases and were renamed to reflect this. In some cases, more than one enzyme was found to participate in a reaction, and in other cases, a single enzyme mediated in more than one reaction. Variation in different tissues and mammalian species also was found. | 11
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The vast majority of people who thought that they were possessed by the devil did not have hallucinations or other "spectacular symptoms" but "complained of anxiety, religious fears, and evil thoughts. ": 213 In 1584, a woman from Kent, England, named Mrs. Davie, described by a justice of the peace as "a good wife", was nearly burned at the stake after she confessed that she experienced constant, unwanted urges to murder her family. : 213 The English term obsessive–compulsive arose as a translation of German Zwangsvorstellung (obsession) used in the first conceptions of OCD by Carl Westphal. Westphals description went on to influence Pierre Janet, who further documented features of OCD. In the early 1910s, Sigmund Freud attributed obsessive–compulsive behavior to unconscious conflicts that manifest as symptoms. Freud describes the clinical history of a typical case of "touching phobia" as starting in early childhood, when the person has a strong desire to touch an item. In response, the person develops an "external prohibition" against this type of touching. However, this "prohibition does not succeed in abolishing" the desire to touch; all it can do is repress the desire and "force it into the unconscious." Freudian psychoanalysis remained the dominant treatment for OCD until the mid-1980s, even though medicinal and therapeutic treatments were known and available, because it was widely thought that these treatments would be detrimental to the effectiveness of the psychotherapy. : 210–211 In the mid-1980s, this approach changed, and practitioners began treating OCD primarily with medicine and practical therapy rather than through psychoanalysis. | Periorbital hyperpigmentation, also known as hereditary dark circles, is characterized by darker skin around the eyes caused by the presence of additional melanin. It is an extremely common hereditary human characteristic and is frequently found on individuals with dark skin. Periorbital hyperpigmentation is most prevalent within the 16–25 age group. See also
Eye shadow
Kohl (cosmetics)
Freckles
References
== External links == | 0-1
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The chapter contains the following sentence:
We must not, however, lay too much stress on the copious shedding of tears by the insane, as being due to the lack of all restraint; for certain brain-diseases, as hemiplegia, brain-wasting, and senile decay, have a special tendency to induce weeping. Terminology
Historically, there have been a variety of terms used for the disorder, including pseudobulbar affect, pathological laughter and crying, emotional lability, emotionalism, emotional dysregulation, or more recently, involuntary emotional expression disorder. The term pseudobulbar (pseudo- + bulbar) came from the idea that the symptoms seemed similar to those caused by a bulbar lesion (that is, a lesion in the medulla oblongata). Terms such as forced crying, involuntary crying, pathological emotionality, and emotional incontinence have also been used, although less frequently. In popular culture
Arthur Fleck, the central character of the 2019 film Joker, displays signs of pseudobulbar affect, which are said to be what Joaquin Phoenix used as inspiration for his characters signature laugh. In the 2019 movie Parasite, the character Ki-woo sustains head trauma, and although it is not clearly mentioned that hes affected by pseudobulbar affect, he mentions not being able to stop laughing when thinking about all the events that occur in the movie. In the medical television show House, season 7, episode 8 ("Small Sacrifices"), the character Ramon Silva, played by Kuno Becker displays pseudobulbar affect, with uncontrollable incongruent laughter, while having the Marburg variety of multiple sclerosis. | Amnion nodosum are nodules found on the amnion, and is frequently present in oligohydramnios. The nodules are composed of squamous cell aggregates derived from the vernix caseosa on the fetal skin. Amnion nodosum is caused by the unexpected abrasion of amnion with depositions of the fetal surface cells and acellular debris on the eroded areas(due to moderate or severe oligohydramnios, the amnion can get "touch" with the fetal skin). Amnion nodosum and oligohydramnios are associated with pulmonary hypoplasia and renal agenesis. Amnion nodosum is granules on amnion whereas whitish nodules on the cord suggest a candidal infection. == References == | 0-1
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Although ecotourists claim to be educationally sophisticated and environmentally concerned, they rarely understand the ecological consequences of their visits and how their day-to-day activities append physical impacts on the environment. As one scientist observes, they "rarely acknowledge how the meals they eat, the toilets they flush, the water they drink, and so on, are all part of broader regional economic and ecological systems they are helping to reconfigure with their very activities." Nor do ecotourists recognize the great consumption of non-renewable energy required to arrive at their destination, which is typically more remote than conventional tourism destinations. For instance, an exotic journey to a place 10,000 kilometers away consumes about 700 liters of fuel per person.Ecotourism activities are, in and of themselves, issues in environmental impact because they may disturb fauna and flora. Ecotourists believe that because they are only taking pictures and leaving footprints, they keep ecotourism sites pristine, but even harmless-sounding activities such as nature hikes can be ecologically destructive. In the Annapurna Circuit in Nepal, ecotourists have worn down the marked trails and created alternate routes, contributing to soil impaction, erosion, and plant damage. Where the ecotourism activity involves wildlife viewing, it can scare away animals, disrupt their feeding and nesting sites, or acclimate them to the presence of people. In Kenya, wildlife-observer disruption drives cheetahs off their reserves, increasing the risk of inbreeding and further endangering the species. | Patients undergoing therapy to suppress the immune system are at risk for hemorrhagic cystitis due to either the direct effects of chemotherapy or activation of dormant viruses in the kidney, ureter, or bladder. Diagnosis
Diagnosis is made by history and examination.In immunocompromised patients, pus is present in the urine but often no organism can be cultured. In children, polymerase chain reaction sequencing of urine can detect fragments of the infectious agent.The procedure differs somewhat for women and men. Laboratory testing of urine samples now can be performed with dipsticks that indicate immune system responses to infection, as well as with microscopic analysis of samples. The presence of hematuria, or blood in the urine, may indicate acute UTIs, kidney disease, kidney stones, inflammation of the prostate (in men), endometriosis (in women), or cancer of the urinary tract. In some cases, blood in the urine results from athletic training, particularly in runners. Treatment
Unfortunately mesna is ineffective as a treatment once hemorrhagic cystitis has developed. Although rare, once a case of radiation-induced hemorrhagic cystitis is diagnosed there is no empirically-proven treatments to heal this type of condition, which can severely degrade a patients quality of life and might possibly lead to kidney failure with risk of death. Viral hemorrhagic cystitis in children generally spontaneously resolves within a few days. The first step in the treatment of HC should be directed toward clot evacuation. Bladder outlet obstruction from clots can lead to urosepsis, bladder rupture, and kidney failure. | 0-1
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Cat-scratch disease (CSD) or felinosis is an infectious disease that most often results from a scratch or bite of a cat. Symptoms typically include a non-painful bump or blister at the site of injury and painful and swollen lymph nodes. People may feel tired, have a headache, or a fever. Symptoms typically begin within 3–14 days following infection.Cat-scratch disease is caused by the bacterium Bartonella henselae which is believed to be spread by the cats saliva. Young cats pose a greater risk than older cats. Occasionally dog scratches or bites may be involved. Diagnosis is generally based on symptoms. Confirmation is possible by blood tests.The primary treatment is supportive. Antibiotics speed healing and are recommended in those with severe disease or immune problems. Recovery typically occurs within 4 months but can require a year. About 1 in 10,000 people are affected. It is more common in children. Signs and symptoms
Cat-scratch disease commonly presents as tender, swollen lymph nodes near the site of the inoculating bite or scratch or on the neck, and is usually limited to one side. This condition is referred to as regional lymphadenopathy and occurs 1–3 weeks after inoculation. Lymphadenopathy most commonly occurs in the axilla, arms, neck, or jaw, but may also occur near the groin or around the ear. A vesicle or an erythematous papule may form at the site of initial infection.Most people also develop systemic symptoms such as malaise, decreased appetite, and aches. Other associated complaints include headache, chills, muscular pains, joint pains, arthritis, backache, and abdominal pain. | In very rare cases, there may be conjoined twins, possibly impairing function of internal organs. Mortality rate (stillbirth)
Multiples are also known to have a higher mortality rate. It is more common for multiple births to be stillborn, while for singletons the risk is not as high. A literary review on multiple pregnancies shows a study done on one set each of septuplets and octuplets, two sets of sextuplets, 8 sets of quintuplets, 17 sets of quadruplets, and 228 sets of triplets. By doing this study, Hammond found that the mean gestational age (how many weeks when birthed) at birth was 33.4 weeks for triplets and 31 weeks for quadruplets. This shows that stillbirth happens usually 3–5 weeks before the woman reaches full term and also that for sextuplets or higher it almost always ends in death of the fetuses. Though multiples are at a greater risk of being stillborn, there is inconclusive evidence whether the actual mortality rate is higher in multiples than in singletons. Prevention in IVF
Today many multiple pregnancies are the result of in vitro fertilisation (IVF). In a 1997 study of 2,173 embryo transfers performed as part of in vitro fertilisation (IVF), 34% were successfully delivered pregnancies. The overall multiple pregnancy rate was 31.3% (24.7% twins, 5.8% triplets, and .08% quadruplets). Because IVFs are producing more multiples, a number of efforts are being made to reduce the risk of multiple births- specifically triplets or more. Medical practitioners are doing this by limiting the number of embryos per embryo transfer to one or two. | 0-1
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Pathophysiology
Congenital dysfibrinogenemia is most often caused by a single autosomal dominant missense mutation in the Aα, Bβ, or γ gene; rarely, it is caused by a homozygous or compound heterozygous missense mutation, a deletion, frameshift mutation, insert mutation, or splice site mutation in one of these genes. The most frequent sites for these mutations code for the N-terminus of the Aα chain or the C-terminus of the γ chain that lead to defective assembly of fibrin in early clot formation and thereby a bleeding predisposition. Two particular missense mutations represent the majority (74% in one study of 101 individuals) of all mutations associated with dysfibrinogenemia and therefore represent prime sites to examine in the initial testing of individuals having a congenital dysfibrinogenmia bleeding disorder. These mutations alter the codon coded for the amino acid arginine at either the 35th position of FGA (termed Arg35; see fibrinogen Metz1 and fibrinogen Bicetre in the Table below) and or the 301st position of FGG (termed Arg301; see fibrinogen Baltimore IV in the Table below).The following Table lists examples of mutations causing congenital dysfibrinogenemias. It gives: a) the mutated proteins trivial name; b) the gene mutated (i.e. FGA, FGB, or FGG), its mutation site (i.e. numbered nucleotide in the cloned gene), and the names of the nucleotides (i.e. | So the patient can in fact have his or her medication with food as long as the meal does not contain fat or oils (e.g., a cup of black coffee or toast with jam and no butter). Taking the medicines with food also helps ease the nausea that many patients feel when taking the medicines on an empty stomach. The effect of food on the absorption of INH is not clear: two studies have shown reduced absorption with food but one study showed no difference. There is a small effect of food on the absorption of PZA and of EMB that is probably not clinically important.It is possible to test urine for isoniazid and rifampicin levels to check for compliance. The interpretation of urine analysis is based on the fact that isoniazid has a longer half-life than rifampicin:
urine positive for isoniazid and rifampicin – patient probably fully compliant
urine positive for isoniazid only – patient has taken his medication in the last few days preceding the clinic appointment, but had not yet taken a dose that day. urine positive for rifampicin only – patient has omitted to take his medication the preceding few days, but did take it just before coming to clinic. urine negative for both isoniazid and rifampicin – patient has not taken either medicine for a number of daysIn countries where doctors are unable to compel patients to take their treatment (e.g., the UK), some say that urine testing only results in unhelpful confrontations with patients and does not help increase compliance. | 0-1
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An infantile hemangioma (IH), sometimes called a strawberry mark due to appearance, is a type of benign vascular tumor or anomaly that affects babies. Other names include capillary hemangioma, strawberry hemangioma,: 593 strawberry birthmark and strawberry nevus. and formerly known as a cavernous hemangioma. They appear as a red or blue raised lesion on the skin. Typically, they begin during the first four weeks of life, growing until about five months of life, and then shrinking in size and disappearing over the next few years. Often skin changes remain after they shrink. Complications may include pain, bleeding, ulcer formation, disfigurement, or heart failure. It is the most common tumor of orbit and periorbital areas in childhood. It may occur in the skin, subcutaneous tissues and mucous membranes of oral cavities and lips as well as in extracutaneous locations including the liver and gastrointestinal tract. The underlying reason for their occurrence is not clear. In about 10% of cases they appear to run in families. A few cases are associated with other abnormalities such as PHACE syndrome. Diagnosis is generally based on the symptoms and appearance. Occasionally medical imaging can assist in the diagnosis.In most cases no treatment is needed, other than close observation. It may grow rapidly, before stopping and slowly fading. Some are gone by the age of 2, about 60% by 5 years, and 90–95% by 9 years. | Sulfacetamide is a sulfonamide antibiotic. Uses
Sulfacetamide 10% topical lotion, sold under the brand name Klaron or Ovace, is approved for the treatment of acne and seborrheic dermatitis. When combined with sulfur, it is sold under the brand names Plexion, Clenia, Prascion, and Avar, which contain 10% sulfacetamide and 5% sulfur.Sulfacetamide has been investigated for use in the treatment of pityriasis versicolor and rosacea. It also has anti-inflammatory properties when used to treat blepharitis or conjunctivitis (in eye-drop solution). It is believed to work by limiting the presence of folic acid which bacteria need to survive. It has been suggested that sulfacetamide may also serve as a treatment for mild forms of hidradenitis suppurativa. Sulfacetamide has antibacterial activity and is used to control acne. Products containing sulfacetamide and sulfur (a keratolytic) are commonly promoted for the treatment of acne rosacea (rosacea with papules, pustules, or both). There are several prescription topical products containing sulfacetamide, such as foams, shampoos, cream and washes. Some research indicates that sulfacetamide derivatives may act as antifungals by an CYP51A1-independent mechanism. Structure and chemical properties
These are organic compounds containing an benzenesulfonamide moiety with an amine group attached to the benzene ring. The molecular formula is C8H10N2O3S. Its scientific name is N-(4-aminophenyl)sulfonylacetamide. At room temperature, it appears as a white powder. Degradation reactions and stability
Sulfacetamide is stable under normal temperatures and pressures. No dangerous reactions occur under known conditions of normal use. It is an important bacteriostatic agent that is commonly used in human and veterinary medicine. | 0-1
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Additional implicated genes include: SMAD4, BMPR1A, PTEN, GREM1, and MUTYH. The only validated genetic cause of SPS is RNF43. However, most individuals with SPS do not have a germline mutation in any of the associated genes, including RNF43. As the underlying genetic risks for SPS are not fully understood, genetic testing is not recommended. Both autosomal dominant and autosomal recessive patterns of inheritance have been reported, as well as sporadic. Individuals with SPS have serrated polyps, which include hyperplastic polyps, traditional serrated adenomas, and sessile serrated polyps. In addition to serrated polyps, adenomas are often identified. Diagnosis
The diagnosis of serrated polyposis syndrome is achieved when either one of two criteria are met: ≥5 serrated lesions/polyps proximal to the rectum (all ≥ 5 mm in size, with two lesions ≥10 mm), or >20 serrated lesions/polyps of any size distributed throughout the large bowel with 5 proximal to the rectum. Any serrated polyp is counted towards the diagnosis, including sessile serrated lesions, hyperplastic polyps, and traditional serrated adenomas. In addition, the cumulative number of serrated lesions is cumulative over time and includes multiple colonoscopies. The diagnosis of SPS is often overlooked, and requires lifelong tracking of cumulative serrated polyps. Treatment
Colonoscopy is the mainstay of treatment for SPS, which allows for the identification of polyps and removal. Polyp removal is recommended to prevent the development of colorectal cancer. If polyps are relatively few, then removal may be achieved with colonoscopy. After polyps are removed, repeat colonoscopy is recommended in 1 to 3 year intervals. | History
The condition was originally known as hyperplastic polyposis syndrome. When the syndrome was first recognized, only hyperplastic polyps were included in its definition, and the syndrome was believed to not be associated with a higher risk of colorectal cancer. In 1996, a case series revealed an association of the syndrome with cancer and serrated adenomas. Subsequently, other types of serrated polyps were found to occur in this condition, so the name was adjusted to the present "serrated polyposis syndrome. "The World Health Organization released diagnostic criteria in 2010, which were updated in 2010 and again in 2019. The 2010 classification defined SPS as meeting any of the following criteria: 1) five or more serrated polyps proximal to the sigmoid colon with 2 larger than 10 mm in size, 2) any serrated polyps found proximal to the sigmoid colon in a person with a first-degree relative with serrated polyposis, or 3) more than 20 serrated colon polyps. The updated 2019 classification revised the first criterion to include lesions in the sigmoid colon, and excluded very small polyps (<5 mm). The updated 2019 classification also removed the criterion that included any serrated lesions proximal to the sigmoid colon in a person with a first degree relative with SPS. Epidemiology
Data regarding overall prevalence of SPS is lacking, but it is estimated to occur in roughly 1 in 100,000. SPS is equally common among men and women. Most individuals with SPS are diagnosed between 40 and 60 years of age, with an average age of 55 years. | 11
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It is not useful for spasticity due to neurologic conditions such as cerebral palsy.A 2004 review found benefit for fibromyalgia symptoms, with a reported number needed to treat of 4.8 (meaning that 1 person out of every 4.8 benefits from treatment) for pain reduction, but no change in fatigue or tender points. A 2009 Cochrane review found insufficient evidence to justify its use in myofascial pain syndrome. It may also be used along with other treatments for tetanus. Side effects
Cyclobenzaprine results in increased rates of drowsiness (38%), dry mouth (24%), and dizziness (10%). Drowsiness and dry mouth appear to intensify with increasing dose. The sedative effects of cyclobenzaprine are likely due to its antagonistic effect on histamine, serotonin, and muscarinic receptors.Agitation is a common side effect observed, especially in the elderly. Some experts believe that cyclobenzaprine should be avoided in elderly patients because it can cause confusion, delirium, and cognitive impairment. In general, the National Committee for Quality Assurance recommends avoiding the use of cyclobenzaprine in the elderly because of the potential for more severe side effects.Dysphagia, a life-threatening side-effect, may rarely occur. Treatment protocols and support should follow the same as for any structurally related tricyclic, such as tricyclic antidepressants. Overdose
The most common effects of overdose are drowsiness and tachycardia. Rare but potentially critical complications are cardiac arrest, abnormal heart rhythms, severe low blood pressure, seizures, and neuroleptic malignant syndrome. Life-threatening overdose is rare, however, as the median lethal dose is about 338 milligrams/kilogram in mice and 425 mg/kg in rats. | The potential harm is increased when central nervous system depressants and antidepressants are also used; deliberate overdose often includes alcohol among other drugs. Interactions
Cyclobenzaprine has major contraindications with monoamine oxidase inhibitors (MAOIs). At least one study also found increased risk of serotonin syndrome when cyclobenzaprine was taken with the serotonergic drugs duloxetine or phenelzine.These substances may interact with cyclobenzaprine:
Central nervous system depressants (e.g. alcohol, opioids, benzodiazepines, nonbenzodiazepines, phenothiazines, carbamates, barbiturates, major tranquilizers)
Monoamine oxidase inhibitors taken within two weeks of cyclobenzaprine may result in serious, life-threatening side effects.Cyclobenzaprine may affect the medications used in surgical sedation and some surgeons request that patients temporarily discontinue its use prior to surgery. Pharmacology
Cyclobenzaprine is a centrally acting muscle relaxant. Cyclobenzaprine is a 5-HT2 receptor antagonist; it relieves muscle spasm through action on the central nervous system at the brain stem, rather than targeting the peripheral nervous system or muscles themselves. Pharmacodynamics
Pharmacokinetics
Cyclobenzaprine has an oral bioavailability of about 55% and approximately 93% is bound to proteins in plasma. The half-life of the drug is 18 hours and it has a plasma clearance of 0.7 litres per minute. Comparison to other medications
Cyclobenzaprine has been found to be not inferior to tizanidine, orphenadrine, and carisoprodol in the treatment of acute lower back pain, although none have been proven to be effective for long-term use (beyond two weeks of treatment). No differences in pain or spasm scores were noted among these agents, nor when compared to benzodiazepines. | 11
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CT scans can show brain bleeds, fractures of the skull, fluid build up in the brain that will lead to increased cranial pressure. MRI is able to better detect smaller injuries, detect damage within the brain, diffuse axonal injury, injuries to the brainstem, posterior fossa, and subtemporal and sub frontal regions. However, patients with pacemakers, metallic implants, or other metal within their bodies are unable to have an MRI done. Typically the other imaging techniques are not used in a clinical setting because of the cost, lack of availability. Management
Most head injuries are of a benign nature and require no treatment beyond analgesics such as acetaminophen. Non-steroidal painkillers such as ibuprofen are avoided since they could make any potential bleeding worse. Due to the high risk of even minor brain injuries, close monitoring for potential complications such as intracranial bleeding. If the brain has been severely damaged by trauma, a neurosurgical evaluation may be useful. Treatments may involve controlling elevated intracranial pressure. This can include sedation, paralytics, cerebrospinal fluid diversion. Second-line alternatives include decompressive craniectomy (Jagannathan et al. found a net 65% favorable outcomes rate in pediatric patients), barbiturate coma, hypertonic saline, and hypothermia. Although all of these methods have potential benefits, there has been no randomized study that has shown unequivocal benefit. Clinicians will often consult clinical decision support rules such as the Canadian CT Head Rule or the New Orleans/Charity Head injury/Trauma Rule to decide if the patient needs further imaging studies or observation only. | : 625
Corpus callosum lipoma is a rare congenital brain condition that may or may not present with symptoms. This occurs in the corpus callosum, also known as the calossal commissure, which is a wide, flat bundle of neural fibers beneath the cortex in the human brain. Hibernomas are lipomas of brown fat. Intradermal spindle cell lipomas are distinct in that they most commonly affect women and have a wide distribution, occurring with relatively equal frequency on the head and neck, trunk, and upper and lower extremities. : 625
A fibrolipoma is a lipoma with focal areas of large amounts of fibrous tissue. A sclerotic lipoma is a predominantly fibrous lesion with focal areas of fat.Neural fibrolipomas are overgrowths of fibro-fatty tissue along a nerve trunk, which often leads to nerve compression. : 625 Pleomorphic lipomas, like spindle-cell lipomas, occur for the most part on the backs and necks of elderly men and are characterized by floret giant cells with overlapping nuclei. : 625
Spindle-cell lipomas are asymptomatic, slow-growing, subcutaneous tumors that have a predilection for the posterior back, neck, and shoulders of older men. : 625
Superficial subcutaneous lipomas, the most common type of lipoma, lie just below the surface of the skin. Most occur on the trunk, thigh, and forearm, although they may be found anywhere in the body where fat is located. Causes
The tendency to develop a lipoma is not necessarily hereditary, although hereditary conditions such as familial multiple lipomatosis might include lipoma development. | 0-1
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This is the second largest outbreak with (as of January 2020) over 3,400 confirmed or probable cases, including more than 2,200 who have died.An interim analysis by the Data Safety and Monitoring Board (DSMB) of the first 499 patient found that mAb114 and REGN-EB3 were superior to the comparator ZMapp. Overall mortality of patients in the ZMapp and Remdesivir groups were 49% and 53% compared to 34% and 29% for mAb114 and REGN-EB3. When looking at patients who arrived early after disease symptoms appeared, survival was 89% for mAB114 and 94% for REGN-EB3. While the study was not powered to determine whether there is any difference between REGN-EB3 and mAb114, the survival difference between those two therapies and ZMapp was significant. This led to the DSMB halting the study and PALM investigators dropping the remdesivir and ZMapp arms from the clinical trial. All patients in the outbreak who elect to participate in the trial will now be given either mAb114 or REGN-EB3.In August 2019, Congolese health officials announced it was more effective compared to two other treatments being used at the time.Among patients treated with it, 34% died; the mortality rate improved if the drug was administered soon after infection, in a timely diagnosis – critical for those infected with diseases like Ebola that can cause sepsis and, eventually, multiple organ dysfunction syndrome, more quickly than other diseases. The survival rate if the drug was administered shortly after the infection was 89%. | Atoltivimab/maftivimab/odesivimab was evaluated in 382 adult and pediatric participants with confirmed Zaire ebolavirus infection in one clinical trial (the PALM trial) and as part of an expanded access program conducted in the Democratic Republic of the Congo (DRC) during an Ebola virus outbreak in 2018/2019. In the PALM trial, the safety and efficacy of atoltivimab/maftivimab/odesivimab was evaluated in a multi-center, open-label, randomized controlled trial, in which 154 participants received atoltivimab/maftivimab/odesivimab (50 mg of each monoclonal antibody) intravenously as a single infusion, and 168 participants received an investigational control. The trial enrolled pediatric and adult participants (including pregnant women) with Zaire ebolavirus infection. All participants received standard, supportive care for the disease. The participants and the health care providers knew which treatment was being given. The primary efficacy endpoint was 28-day mortality. The primary analysis population was all participants who were randomized and concurrently eligible to receive either atoltivimab/maftivimab/odesivimab or the investigational control during the same time period of the trial. Of the 154 participants who received atoltivimab/maftivimab/odesivimab, 33.8% died after 28 days, compared to 51% of the 153 participants who received a control. In the expanded access program, an additional 228 participants received atoltivimab/maftivimab/odesivimab. Regulatory status
The FDA granted the application of atoltivimab/maftivimab/odesivimab-ebgn orphan drug and breakthrough therapy designations. The FDA granted the approval of Inmazeb to Regeneron Pharmaceuticals with an indication for the treatment of infection caused by Zaire ebolavirus in October 2020.The drug has also received orphan drug designation from the European Medicines Agency. References
Further reading
External links
"Atoltivimab". Drug Information Portal. U.S. National Library of Medicine. | 11
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Larva migrans can refer to:
Cutaneous larva migrans, a skin disease in humans, caused by the larvae of various nematode parasites
Visceral larva migrans, a condition in children caused by the migratory larvae of nematodes
Ocular larva migrans, an ocular form of the larva migrans syndrome that occurs when larvae invade the eye
Larva migrans profundus, also known as Gnathostomiasis | Serpiginous choroiditis, also known as geographic or helicoid choroidopathy, is an uncommon chronic progressive inflammatory disease affecting adult men and women equally in the second to seventh decades of life. Presentation
In this condition the posterior uveitis shows a geographic pattern. The inflammation begins in the juxtapapillary choroid and intermittently spreads centrifugally. The overlying retinal pigment epithelium and the outer retina are involved in the inflammatory process. A closely related condition is multifocal serpiginoid choroiditis. This is caused by tuberculosis.The distinction between these two conditions is important as the latter responds to anti tuberculosis treatment while the former does not. Diagnosis
Treatment
References
== External links == | 0-1
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By 2014 over 300 zinc containing enzymes have been identified, as well as over 1000 zinc containing transcription factors.Phytate was recognized as removing zinc from nutrients given to chicken and swine in 1960. That it can cause human zinc deficiency however was not recognized until Reinholds work in Iran in the 1970s. This phenomenon is central to the high risk of zinc deficiency worldwide. Soils and crops
Soil zinc is an essential micronutrient for crops. Almost half of the worlds cereal crops are deficient in zinc, leading to poor crop yields. Many agricultural countries around the world are affected by zinc deficiency. In China, zinc deficiency occurs on around half of the agricultural soils, affecting mainly rice and maize. Areas with zinc deficient soils are often regions with widespread zinc deficiency in humans. A basic knowledge of the dynamics of zinc in soils, understanding of the uptake and transport of zinc in crops and characterizing the response of crops to zinc deficiency are essential steps in achieving sustainable solutions to the problem of zinc deficiency in crops and humans. Biofortification
Soil and foliar application of zinc fertilizer can effectively increase grain zinc and reduce the phytate:zinc ratio in grain. People who eat bread prepared from zinc enriched wheat have a significant increase in serum zinc.Zinc fertilization not only increases zinc content in zinc deficient crops, it also increases crop yields. Balanced crop nutrition supplying all essential nutrients, including zinc, is a cost effective management strategy. | Low maternal zinc status has been associated with less attention during the neonatal period and worse motor functioning. In some studies, supplementation has been associated with motor development in very low birth weight infants and more vigorous and functional activity in infants and toddlers. Testosterone production
Zinc is required to produce testosterone. Thus, zinc deficiency can lead to reduced circulating testosterone, which could lead to sexual immaturity (Ananda Parsad, et al.) hypogonadism, and delayed puberty. Causes
Dietary deficiency
Zinc deficiency can be caused by a diet high in phytate-containing whole grains, foods grown in zinc deficient soil, or processed foods containing little or no zinc. Conservative estimates suggest that 25% of the worlds population is at risk of zinc deficiency.In the U.S., the Recommended Dietary Allowance (RDA) is 8 mg/day for women and 11 mg/day for men. RDA for pregnancy is 11 mg/day. RDA for lactation is 12 mg/day. For infants up to 12 months the RDA is 3 mg/day. For children ages 1–13 years the RDA increases with age from 3 to 8 mg/day. The following table summarizes most of the foods with significant quantities of zinc, listed in order of quantity per serving, unfortified. Note that all of the top 10 entries are meat, beans, or nuts. Recent research findings suggest that increasing atmospheric carbon dioxide concentrations will exacerbate zinc deficiency problems in populations that consume grains and legumes as staple foods. | 11
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These results were later supported in trials from the United Kingdom, but not in more recent studies from France and China. Genetic polymorphism appears to be associated with inter-individual variability in metabolic response to HRT in postmenopausal women. The WHI reported statistically significant increases in rates of breast cancer, coronary heart disease, strokes and pulmonary emboli. The study also found statistically significant decreases in rates of hip fracture and colorectal cancer. "A year after the study was stopped in 2002, an article was published indicating that estrogen plus progestin also increases the risks of dementia." The conclusion of the study was that the HRT combination presented risks that outweighed its measured benefits. The results were almost universally reported as risks and problems associated with HRT in general, rather than with Prempro, the specific proprietary combination of CEEs and MPA studied. After the increased clotting found in the first WHI results was reported in 2002, the number of Prempro prescriptions filled reduced by almost half. Following the WHI results, a large percentage of HRT users opted out of them, which was quickly followed by a sharp drop in breast cancer rates. The decrease in breast cancer rates has continued in subsequent years. An unknown number of women started taking alternatives to Prempro, such as compounded bioidentical hormones, though researchers have asserted that compounded hormones are not significantly different from conventional hormone therapy.The other portion of the parallel studies featured women who were post hysterectomy and so received either placebo progestogen or CEEs alone. | Skin and vaginal routes of hormone therapy are not subject to first pass metabolism, and so lack the anabolic effects that oral therapy has on liver synthesis of vitamin K-dependent clotting factors, possibly explaining why oral therapy may increase blood clot formation.While a 2018 review found that taking progesterone and estrogen together can decrease this risk, other reviews reported an increased risk of blood clots and pulmonary embolism when estrogen and progestogen were combined, particularly when treatment was started 10 years or more after menopause and when the women were older than 60 years.The risk of venous thromboembolism may be reduced with bioidentical preparations, though research on this is only preliminary. Stroke
Multiple studies suggest that the possibility of HRT related stroke is absent if therapy is started within five years of menopause, and that the association is absent or even preventive when given by non-oral routes. Ischemic stroke risk was increased during the time of intervention in the WHI, with no significant effect after the cessation of therapy and no difference in mortality at long term follow up. When oral synthetic estrogen or combined estrogen-progestogen treatment is delayed until five years from menopause, cohort studies in Swedish women have suggested an association with hemorrhagic and ischemic stroke. Another large cohort of Danish women suggested that the specific route of administration was important, finding that although oral estrogen increased risk of stroke, absorption through the skin had no impact, and vaginal estrogen actually had a decreased risk. | 11
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Therefore, it shows that rehabilitation effort needs to be continuous for word-finding abilities to improve from the baseline. The studies show that verbs are harder to recall or repeat, even with rehabilitation.Other methods in treating anomic aphasia include circumlocution-induced naming therapy (CIN), wherein the patient uses circumlocution to assist with his or her naming rather than just being told to name the item pictured after given some sort of cue. Results suggest that the patient does better in properly naming objects when undergoing this therapy because CIN strengthens the weakened link between semantics and phonology for patients with anomia, since they often know what an object is used for, but cannot verbally name it.Anomia is often challenging for the families and friends of those affected by it. One way to overcome this is computer-based treatment models, effective especially when used with clinical therapy. Leemann et al. provided anomic patients with computerized-assisted therapy (CAT) sessions, along with traditional therapy sessions using treatment lists of words. Some of the patients received a drug known to help relieve symptoms of anomia (levodopa), while others received a placebo. The researchers found that the drug had no significant effects on improvement with the treatment lists, but almost all of the patients improved after the CAT sessions. They concluded that this form of computerized treatment is effective in increasing naming abilities in anomic patients.Additionally, one study researched the effects of using "excitatory (anodal) transcranial direct current stimulation" over the right temporoparietal cortex, a brain area that seems to correlate to language. | Additionally, patients are able to speak with correct grammar; the main problem is finding the appropriate word to identify an object or person.Sometimes, subjects may know what to do with an object, but still not be able to give a name to the object. For example, if a subject is shown an orange and asked what it is called, the subject may be well aware that the object can be peeled and eaten, and may even be able to demonstrate this by actions or even verbal responses; however, they cannot recall that the object is called an "orange". Sometimes, when a person with this condition is multilingual, they might confuse the language they are speaking in trying to find the right word (inadvertent code-switching). Management
No method is available to completely cure anomic aphasia. However, treatments can help improve word-finding skills. Although a person with anomia may find recalling many types of words to be difficult, such as common nouns, proper nouns, verbs, etc., many studies have shown that treatment for object words, or nouns, has shown promise in rehabilitation research. The treatment includes visual aids, such as pictures, and the patient is asked to identify the object or activity. However, if that is not possible, then the patient is shown the same picture surrounded by words associated with the object or activity. Throughout the process, positive encouragement is provided. The treatment shows an increase in word finding during treatment; however, word identifying decreased two weeks after the rehabilitation period. | 11
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Society and culture
Economic burden
Overall, hepatitis accounts for a significant portion of healthcare expenditures in both developing and developed nations, and is expected to rise in several developing countries. While hepatitis A infections are self-limited events, they are associated with significant costs in the United States. It has been estimated that direct and indirect costs are approximately $1817 and $2459 respectively per case, and that an average of 27 work days is lost per infected adult. A 1997 report demonstrated that a single hospitalization related to hepatitis A cost an average of $6,900 and resulted in around $500 million in total annual healthcare costs. Cost effectiveness studies have found widespread vaccination of adults to not be feasible, but have stated that a combination hepatitis A and B vaccination of children and at risk groups (people from endemic areas, healthcare workers) may be.Hepatitis B accounts for a much larger percentage of health care spending in endemic regions like Asia. In 1997 it accounted for 3.2% of South Koreas total health care expenditures and resulted in $696 million in direct costs. A large majority of that sum was spent on treating disease symptoms and complications. Chronic hepatitis B infections are not as endemic in the United States, but accounted for $357 million in hospitalization costs in the year 1990. | Pemphigoid nodularis is a cutaneous condition that is a variant of bullous pemphigoid that has skin lesions mimicking prurigo nodularis.The antibody involved is IgG. See also
Adult linear IgA disease
List of cutaneous conditions
== References == | 0-1
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All individuals tested with classical early-onset, SLC6A3-related DTDS display raised homovanillic acid (HVA) levels with normal 5-hydroxyindoleacetic acid (5-HIAA, a serotonin metabolite) levels. DTDS patients demonstrate a HVA:5-HIAA ratio of 5.0-13.0, above the normal range of 1.0-4.0. In addition, the patients have normal pterin profiles.Mutant constructs of human dopamine transporter can be used for in vitro functional analysis of any particular mutants dopamine uptake and cocaine-analogue binding. Treatment
A multidisciplinary approach to long-term management of this progressive disorder is optimal. Treatment can be split into three categories: treatment of DTDS manifestations, prevention of secondary complications, and monitoring of overall disease progression. Manifestations
Treatment to control the chorea and dyskinesia in the early stages of DTDS is done with tetrabenazine and benzodiazepines. The dystonia is more difficult to control and the first-line agents include pramipexole and ropinirole; adjuncts include trihexyphenidyl, baclofen, gabapentin, and clonidine for severe dystonia, and chloral hydrate and benzodiazepines for exacerbations of dystonia or status dystonicus. Prevention of secondary complications
Regular physiotherapy is recommended to reduce the risk of contractures, shortening and hardening of the muscles. Feeding may become difficult and alternative feeding strategies including nasogastric tubes or percutaneous endoscopic gastrostomies may become necessary due to progressive bulbar dysfunction. The influenza vaccine is used in combination with prophylactic antibiotics to prevent chest infections. Monitoring
Patients are evaluated every six to 12 months for early evidence of hip dislocation and spinal deformities. Risk of pulmonary aspiration is evaluated with regular swallowing assessments. Nutritional assessments are performed regularly to ensure adequate caloric intake. | Treatment
Preliminary experiments have examined the potential of drugs to act as pharmacological chaperones of DAT to rescue DTDS mutations. Bupropion and ibogaine appear to promote DAT maturation and functional activity by acting as pharmacological chaperones in the endoplasmic reticulum. These two drugs rescue DAT maturation and functional activity of two DTDS-associated mutations (A314V and R445C). Preclinical experiments in mice have demonstrated efficacy and safety of a potential treatment using stereotactic injection of AAV vector to deliver working copies of the DAT gene. Support organizations
A variety of support and advocacy groups exist to connect with other patients and families or provide valuable services. International Parkinson and Movement Disorder Society
Parkinson & Movement Disorder Alliance
Parkinsons Foundation
Pediatric Neurotransmitter Disease Association
== References == | 11
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It is the third most common intestinal tumor in cats, after lymphoma and adenocarcinoma.Diagnosis and treatment are similar to that of the dog. Cases involving difficult to remove or multiple tumors have responded well to strontium-90 radiotherapy as an alternative to surgery. The prognosis for solitary skin tumors is good, but guarded for tumors in other organs. Histological grading of tumors has little bearing on prognosis. References
External links
Mast Cell Tumors from The Pet Health Library
Mast Cell Tumors in Dogs from Pet Cancer Center
Mast Cell Tumors in Cats from Pet Cancer Center | These groups include:
Those who have used topical corticosteroids over a prolonged period of time
Those who have used corticosteroids to cover a large surface area
Those with broken skin barrier or extensive abrasions
Those who have recently undergone stress (such as illness, trauma, surgery)
Children under the age of 12HPA axis suppression is preventable by supplementation with glucocorticosteroids. If HPA axis suppression occurs, it is often reversed shortly after discontinuation of treatment. Pharmacology
Mechanism of action
Pharmacokinetics
Absorption of topical corticosteroids depends on several factors such as the vehicle, or delivery system used by the drug, the integrity of the epidermal barrier, and whether or not an occlusive bandage is used in combination with the drug.The absorption of topical betamethasone dipropionate is theoretically minuscule; however, if absorbed it follows the same pharmacokinetic profile that is typical of systemic corticosteroids. It is metabolized primarily by the liver by hydrolysis to its metabolites B17P (primary) and betamethasone and the 6β-hydroxy derivatives of those metabolites, and it is excreted primarily by the kidneys. Chemistry
Betamethasone dipropionate is a white to almost white crystalline powder. Regulation
Betamethasone dipropionate was patented by Merck in 1987, as an augmented cream/lotion, Diprolene in the U.S., and Disprosone in Europe. These patents expired in 2003 and 2007 respectively leading to generic production of betamethasone dipropionate. During this time other topical corticosteroids such as triamcinolone acetonide and clobetasol propionate also became available as generic creams. | 0-1
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The surgical technique is analogous to radial polydactyly, in which the level of duplication and anatomical components should guide operative treatment. The pedicled ulnar extra digit can be removed by suture ligation to devise the skin bridge of the newborn child. This might be easier than an excision of the extra digit when the child is 6 to 12 months old. Ligation occludes the vascular supply to the duplicated digit, resulting in dry gangrene and subsequent autoamputation. This must be done with consideration of the presence of a neurovascular bundle, even in very small skin bridges. When the ligation is done inappropriately it can give a residual nubbin. Also, a neuroma can develop in the area of the scar. An excision can prevent the development of a residual nubbin and the sensitivity due to a neuroma. For infants with ulnar type B polydactyly the recommended treatment is ligation in the neonatal nursery. Studies have shown that excision of the extra digit in the neonatal nursery is a safe and simple procedure with a good clinical and cosmetic outcome. Radial polydactyly
Because neither of the two thumb components is normal, a decision should be taken on combining which elements to create the best possible composite digit. Instead of amputating the most hypoplastic thumb, preservation of skin, nail, collateral ligaments and tendons is needed to augment the residual thumb. Surgery is recommended in the first year of life, generally between 9 and 15 months of age. Surgical options depend on type of polydactyly. | A nematode infection is a type of helminthiasis caused by organisms in the nematode phylum.An example is enterobiasis. Several antinematodal agents are available. References
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Many of these patients experience their seizures only during sleep. They can have seemingly bizarre features as they originate from the frontal lobe of the brain. Often, individuals with ring chromosome 20 syndrome are initially found to have complex partial seizures of frontal lobe origin, though imaging studies do not show a corresponding structural brain abnormality. In certain patients, these seizures may secondarily generalized. Individuals from the ages of 0–17 years should be considered for ring 20 chromosome analysis if they have: predominantly complex partial seizures, medically refractory cryptogenic epilepsy, Lennox-Gastaut-like features with no cause identified, frequent subtle nocturnal seizures, an EEG showing prolonged high voltage frontally dominant slowing intermixed with spikes or sharp waves, an EEG showing overlapping features of continuous slow spike and wave discharges in sleep (CSWS) and electrical status epilepticus in sleep (ESES), and/or subsequent cognitive impairment/learning difficulties/mild retardation.These patients will typically have a normal childhood development until onset of epilepsy and lack evidence of dysmorphism or other congenital malformations. Genetics
Rather than the typical linear pattern of a chromosome, deletion of the endings of a chromosome can lead to ring formation. A chromosome has two arms, one long and one short. Deletion of the short arm of chromosome 20 does not appear to result in epilepsy; however, terminal deletion of the long arm is associated with epilepsy. Therefore, some gene loss from the terminal segment could be responsible for the manifestation of epilepsy in ring chromosome 20 syndrome. The most common breakpoint in patients is in the q13.33 region of chromosome 20. | As chromosomes occur in pairs, the affected individual usually has one normal chromosome and the other replaced by a ring chromosome. However, this is not usually the case for every cell in the patient. In most individuals with ring chromosome 20 syndrome, a certain percentage have two normal chromosome 20s and the remainder have one normal and one ring 20 chromosome. This mixture is called mosaicism. A higher degree (percentage) of mosaicism appears to be associated with earlier age of seizure onset and presence of malformations. Yet, the degree of mosaicism does not determine response to drug treatment. Since the range in age of onset of seizures and IQ for a given mosaic ratio is relatively wide, prediction of phenotype based on the mosaicism ratio is somewhat limited. Occasionally, there is a variation, where the ring chromosome 20 syndrome is characterized by an extra ring chromosome in addition to the two normal ones and hence gives rise to a partial trisomy or supernumerary ring 20. This supernumerary ring 20 can cause multiple anomalies but no epilepsy, and this resulting syndrome should be differentiated from ring chromosome 20 syndrome. This confusion has persisted even in the literature. The genes in the q13.33 region of chromosome 20 have yet to be fully delineated. However, this telomeric region includes two genes that are related to other distinct epilepsy syndromes, autosomal dominant nocturnal frontal lobe epilepsy and benign familial neonatal convulsions. | 11
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Because all three are involved in forming the hexameric glycoprotein fibrinogen, mutations in any one of the three genes can cause the deficiency. Diagnosis
Treatment
The most common type of treatment is cryoprecipitate or fibrinogen concentrate drip to increase fibrinogen levels to normal during surgical procedures or after trauma. RiaSTAP, a factor I concentrate, was approved by the U.S. FDA in 2009 for use when the fibrinogen level was below 50 mg/deciliter of plasma. Recently, antifibrinolytics have also been used to inhibit fibrinolysis (breaking down of the fibrin clot). In the case of dysfibrinogenemia that manifests by thrombosis, anticoagulants can be used. Due to the inhibited clotting ability associated with a- and hypofibrinogenemia, physicians advise against the use of Aspirin as it inhibits platelet function. See also
Congenital afibrinogenemia
Hypodysfibrinogenemia
References
== External links == | Costello syndrome, also called faciocutaneoskeletal syndrome or FCS syndrome, is a rare genetic disorder that affects many parts of the body. It is characterized by delayed development and intellectual disabilities, distinctive facial features, unusually flexible joints, and loose folds of extra skin, especially on the hands and feet. : 571 Heart abnormalities are common, including a very fast heartbeat (tachycardia), structural heart defects, and overgrowth of the heart muscle (hypertrophic cardiomyopathy). Infants with Costello syndrome may be large at birth, but grow more slowly than other children and have difficulty feeding. Later in life, people with this condition have relatively short stature and many have reduced levels of growth hormones. It is a RASopathy.Beginning in early childhood, people with specific mutations on the Costello syndrome gene variant have an increased risk of developing certain cancerous and noncancerous tumors. Small growths called papillomas are the most common noncancerous tumors seen with this condition. They usually develop around the nose and mouth. The most frequent cancerous tumor associated with Costello syndrome is a soft tissue tumor called a rhabdomyosarcoma. Other cancers also have been reported in children and adolescents with this disorder, including a tumor that arises in developing nerve cells (neuroblastoma) and a form of bladder cancer (transitional cell carcinoma). Costello syndrome was discovered by Jack Costello, a New Zealand paediatrician, in 1977. He is credited with first reporting the syndrome in the Australian Paediatric Journal, Volume 13, No.2 in 1977. | 0-1
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Adverse effects
Like most pharmacological therapeutic options, there are risks that should be considered. For these drugs in particular, vasodilation can be associated with some adverse effects which might include orthostatic hypotension, reflex tachycardia, headaches and palpitations. Tolerance may also develop over time due compensatory response of the body, as well as depletion of -SH groups of glutathione which are essential for the metabolism of the drugs to their active forms. Potential side effects:
Verapamil: hypotension, bradycardia, constipation
Diltiazem: hypotension, bradycardia, risk of heart block
Nifedipine: hypotension
Propranolol: asystole, asthma attacks
Contraindications
Organic nitrates should not be taken with PDE5 inhibitors (i.e. sildenafil) since both NO and PDE5 inhibitors increase cyclic GMP levels and the sum of their pharmacodynamic effects will greatly exceed the optimal therapeutic levels. What you could see upon taking both medications at the same time, as caused by the much higher induction of relaxation of smooth muscle cells, include a severe drop in blood pressure. Beta-receptor antagonists should be avoided in patients with reactive pulmonary disease to avoid asthma attacks. Also Beta-receptor antagonists should be avoided in patients with AV node dysfunction and/or patients on other medications which might cause bradycardia (i.e. calcium channel blockers). The potential for these contraindications and drug-drug interaction could lead to asystole and cardiac arrest. Certain calcium channel blocker should be avoided with some beta-receptor blockers since they may cause severe bradycardia and other potential side effects. | Vasospasm refers to a condition in which an arterial spasm leads to vasoconstriction. This can lead to tissue ischemia and tissue death (necrosis). Cerebral vasospasm may arise in the context of subarachnoid hemorrhage. Symptomatic vasospasm or delayed cerebral ischemia is a major contributor to post-operative stroke and death especially after aneurysmal subarachnoid hemorrhage. Vasospasm typically appears 4 to 10 days after subarachnoid hemorrhage. Along with physical resistance, vasospasm is a main cause of ischemia. Like physical resistance, vasospasms can occur due to atherosclerosis. Vasospasm is the major cause of Prinzmetals angina. Pathophysiology
Normally endothelial cells release prostacyclin and nitric oxide (NO) which induce relaxation of the smooth muscle cells, and reduce aggregation of platelets. Aggregating platelets stimulate ADP to act on endothelial cells and help them induce relaxation of the smooth muscle cells. However, aggregating platelets also stimulate thromboxane A2 and serotonin which can induce contraction of the smooth muscle cells. In general, the relaxations outweighs the contractions. In atherosclerosis, a dysfunctional endothelium is observed on examination. It does not stimulate as much prostacyclin and NO to induce relaxation on smooth muscle cells. Also there is not as much inhibition of aggregation of platelets. In this case, the greater aggregation of platelets produce ADP, serotonin, and thromboxane A2. However the serotonin and the thromboxane A2 cause more contraction of the smooth muscle cells and as a result contractions outweigh the relaxations. Complications
Vasospasm can occur in a wide variety of peripheral vascular beds under poorly understood mechanisms. | 11
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Stage 2: Light aerobic activity such as walking or stationary cycling
Stage 3: Sport-specific activities such as running drills and skating drills
Stage 4: Non-contact training drills (exercise, coordination, and cognitive load)
Stage 5: Full-contact practice (requires medical clearance)
Stage 6: Return to full-contact sport or high-risk activities (requires medical clearance)At each step, the person should not have worsening or new symptoms for at least 24 hours before progressing to the next. If symptoms worsen or new symptoms begin, athletes should drop back to the previous level for at least another 24 hours.Intercollegiate or professional athletes, are typically followed closely by team athletic trainers during this period but others may not have access to this level of health care and may be sent home with minimal monitoring. Medications
Medications may be prescribed to treat headaches, sleep problems and depression. Analgesics such as ibuprofen can be taken for headaches, but paracetamol (acetaminophen) is preferred to minimize the risk of intracranial hemorrhage. Concussed individuals are advised not to use alcohol or other drugs that have not been approved by a doctor as they can impede healing. Activation database-guided EEG biofeedback has been shown to return the memory abilities of the concussed individual to levels better than the control group.About one percent of people who receive treatment for mTBI need surgery for a brain injury. | New "Head Impact Telemetry System" technology is being placed in helmets to study injury mechanisms and may generate knowledge that will potentially help reduce the risk of concussions among American Football players.Educational interventions, such as handouts, videos, workshops, and lectures, can improve concussion knowledge of diverse groups, particularly youth athletes and coaches. Strong concussion knowledge may be associated with greater recognition of concussion symptoms, higher rates of concussion reporting behaviors, and reduced body checking-related penalties and injuries, thereby lowering risk of mTBI.Due to the incidence of concussion in sport, younger athletes often do not disclose concussions and their symptoms. Common reasons for non-disclosure include a lack of awareness of the concussion, the belief that the concussion was not serious enough, and not wanting to leave the game or team due to their injury. Self-reported concussion rates among U-20 and elite rugby union players in Ireland are 45–48%, indicating that many concussions go unreported. Changes to the rules or enforcing existing rules in sports, such as those against "head-down tackling", or "spearing", which is associated with a high injury rate, may also prevent concussions. Treatment
Adults and children with a suspected concussion require a medical assessment to confirm the diagnosis of concussion and rule out more serious head injuries. After life-threatening head injuries, injuries to the cervical spine, and neurological conditions are ruled out, exclusion of neck or head injury, observation should be continued for several hours. | 11
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Firstly, an infected T cell can transmit virus directly to a target T cell via a virological synapse. Secondly, an antigen-presenting cell (APC), such as a macrophage or dendritic cell, can transmit HIV to T cells by a process that either involves productive infection (in the case of macrophages) or capture and transfer of virions in trans (in the case of dendritic cells). Whichever pathway is used, infection by cell-to-cell transfer is reported to be much more efficient than cell-free virus spread. A number of factors contribute to this increased efficiency, including polarised virus budding towards the site of cell-to-cell contact, close apposition of cells, which minimizes fluid-phase diffusion of virions, and clustering of HIV entry receptors on the target cell towards the contact zone. Cell-to-cell spread is thought to be particularly important in lymphoid tissues, where CD4+ T cells are densely packed and likely to interact frequently. Intravital imaging studies have supported the concept of the HIV virological synapse in vivo. The many dissemination mechanisms available to HIV contribute to the viruss ongoing replication in spite of anti-retroviral therapies. Genetic variability
HIV differs from many viruses in that it has very high genetic variability. | See also
Nasal septum deviation
References
Different surgical treatments for nasal septal perforation and their outcomes
Septal Perforation Closure Utilizing the External Septorhinoplasty Approach
A Review of 25-Year Experience of Nasal Septal Perforation Repair
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A 2020 study conducted by INRIX, private company that analyzes traffic patters, behaviors and congestion, showed that as traffic levels returned to normal during the three-month period August to October 2020, growth in collisions (57%), outpaced the growth in miles traveled (22%) resulting in a higher than normal collision rate during this period.In France, the Ministry of Interior reported that traffic incidents, crash-related injuries, and fatalities dropped in 2020 compared with 2019. Fatalities dropped 21.4%, injuries dropped 20.9%, and incidents overall dropped 20%. In the same report, the ministry reported that the number of vehicles on the road dropped by 75%, which would indicate that the rate (incidents per vehicle-mile) in fact increased. Other
Other possibly hazardous factors that may alter a drivers soundness on the road include:
Irritability
Following specifically distinct rules too bureaucratically, inflexibly or rigidly when unique circumstances might suggest otherwise
Sudden swerving into somebodys blind spot without first clearly making oneself visible through the wing mirror
Unfamiliarity with ones dashboard features, center console or other interior handling devices after a recent car purchase
Lack of visibility due to windshield design, dense fog or sun glare
People-watching. Traffic safety culture, a variety of aspects of safety culture could impact on the number of crashes. Prevention
A large body of knowledge has been amassed on how to prevent car crashes, and reduce the severity of those that do occur. | For the inside jab, as the opponent throws the jab, rotate your body counter-clockwise, lean slightly to your left putting more weight on your lead leg. Its possible to just lean without rotating, but rotating helps the movement of your guard. Raise your rear hand ready for the opponent to throw a left hook. Common mistakes
There are many different mistakes you can make when trying to slip a punch:
Slipping too early
Slipping too wide
Slipping inside the cross
Only moving your head
Dropping your guard
How to master slipping
The best method to mastering slipping is practice. Now just saying practice seems simple. But the practice needs to be with a worthy opponent. Preferably someone that is taller than you and has a longer reach. Another method is a slip bag you can hang up and move back and forth. This helps you improve movement, timing, and eye coordination while performing a slip. Repetition and patience is key to mastering slipping. References
External links
https://www.mightyfighter.com/how-to-slip-punches/
https://lawofthefist.com/complete-guide-to-slipping-punches-in-boxing/ | 0-1
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Several techniques followed and materials used included metal loops, pins, kangaroo tendons and screws. Significant advances in surgery occurred in the 1950s with the development of banding after Herbert A. Haxton and others demonstrated that the patella was not just to protect the knee but important for straightening the leg. Subsequently, the three surgical treatments for displaced patella fractures became tension banding, partial patellectomy and total patellectomy, the decision of which to use tailored to the individual. References
== External links == | That is, it is a selective mixed agonist–antagonist of the estrogen receptors (ERs), with estrogenic actions in some tissues and antiestrogenic actions in other tissues. The medication has estrogenic effects in bone, partial estrogenic effects in the uterus and liver, and antiestrogenic effects in the breasts. The affinity of toremifene for the ER is similar to that of tamoxifen. In studies using rat ER, toremifene had about 1.4% and tamoxifen had about 1.6% of the affinity of estradiol for the ER. The affinities (Ki) of toremifene at the human ERs have been reported as 20.3 ± 0.1 nM for the ERα and 15.4 ± 3.1 nM for the ERβ. In other rat ER studies, toremifene had 3–9% of the affinity of estradiol for the ER while its metabolites N-desmethyltoremifene and 4-hydroxytoremifene had 3–5% and 64–158% of the affinity of estradiol for the ER, respectively. The affinity of another metabolite, 4-hydroxy-N-desmethyltoremifene, was not assessed. 4-Hydroxytoremifene showed about 100-fold higher antiestrogenic potency than toremifene in vitro in one study, but not in another. 4-Hydroxy-N-desmethyltoremifene has also been found to be strongly antiestrogenic in vitro. The metabolites of toremifene, particularly 4-hydroxytoremifene, may contribute importantly to the clinical activity of the medication. On the other hand, some authorities consider toremifene not to be a prodrug.Toremifene is very similar to tamoxifen and shares most of its properties. There are some indications that toremifene may be safer than tamoxifen as it is not a hepatocarcinogen in animals and may have less potential for genotoxicity. | 0-1
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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
Chlorpromazine is classified as a low-potency typical antipsychotic. Low-potency antipsychotics have more anticholinergic side effects, such as dry mouth, sedation, and constipation, and lower rates of extrapyramidal side effects, while high-potency antipsychotics (such as haloperidol) have the reverse profile. Pharmacokinetics
Pharmacodynamics
Chlorpromazine is a very effective antagonist of D2 dopamine receptors and similar receptors, such as D3 and D5. Unlike most other drugs of this genre, it also has a high affinity for D1 receptors. Blocking these receptors causes diminished neurotransmitter binding in the forebrain, resulting in many different effects. Dopamine, unable to bind with a receptor, causes a feedback loop that causes dopaminergic neurons to release more dopamine. Therefore, upon first taking the drug, patients will experience an increase in dopaminergic neural activity. Eventually, dopamine production of the neurons will drop substantially and dopamine will be removed from the synaptic cleft. | The effect of this drug in emptying psychiatric hospitals has been compared to that of penicillin and infectious diseases. But the popularity of the drug fell from the late 1960s as newer drugs came on the scene. From chlorpromazine a number of other similar antipsychotics were developed. It also led to the discovery of antidepressants.Chlorpromazine largely replaced electroconvulsive therapy, hydrotherapy, psychosurgery, and insulin shock therapy. By 1964, about 50 million people worldwide had taken it. Chlorpromazine, in widespread use for 50 years, remains a "benchmark" drug in the treatment of schizophrenia, an effective drug although not a perfect one. The relative strengths or potencies of other antipsychotics are often ranked or measured against chlorpromazine in aliquots of 100 mg, termed chlorpromazine equivalents or CPZE.In the movie: "Shutter Island", chlorpromazine is presented as being the new medicament for psychosis treatment however with adverse effects like tremors or abstinence syndrome. Society and culture
Names
Brand names include Thorazine, Largactil, Hibernal, and Megaphen (sold by Bayer in West-Germany since July 1953).) Research
Chlorpromazine has tentative benefit in animals infected with Naegleria fowleri, and shows antifungal and antibacterial activity in vitro. Veterinary use
The veterinary use of chlorpromazine has generally been superseded by use of acepromazine.Chlorpromazine may be used as an antiemetic in dogs and cats, or, less often, as sedative before anesthesia. In horses, it often causes ataxia and lethargy, and is therefore seldom used.It is commonly used to decrease nausea in animals that are too young for other common anti-emetics. | 11
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Nasal polyps are common, particularly in children and teenagers. As the disease progresses, people tend to have shortness of breath, and a chronic cough that produces sputum. Breathing problems make it increasingly challenging to exercise, and prolonged illness causes those affected to be underweight for their age. In late adolescence or adulthood, people begin to develop severe signs of lung disease: wheezing, digital clubbing, cyanosis, coughing up blood, pulmonary heart disease, and collapsed lung (atelectasis or pneumothorax).In rare cases, cystic fibrosis can manifest itself as a coagulation disorder. Vitamin K is normally absorbed from breast milk, formula, and later, solid foods. This absorption is impaired in some CF patients. Young children are especially sensitive to vitamin K malabsorptive disorders because only a very small amount of vitamin K crosses the placenta, leaving the child with very low reserves and limited ability to absorb vitamin K from dietary sources after birth. Because clotting factors II, VII, IX, and X are vitamin K–dependent, low levels of vitamin K can result in coagulation problems. Consequently, when a child presents with unexplained bruising, a coagulation evaluation may be warranted to determine whether an underlying disease is present. Lungs and sinuses
Lung disease results from clogging of the airways due to mucus build-up, decreased mucociliary clearance, and resulting inflammation. In later stages, changes in the architecture of the lung, such as pathology in the major airways (bronchiectasis), further exacerbate difficulties in breathing. | Patients with sPCL may have short-lived responses to treatment regimens (as communicated in case reports) that include bortezomid but there are no established therapeutic regimens that have clearly been shown to improve their overall or median survival. See also
Plasma cell dyscrasia
Multiple myeloma
References
BibliographyGreer JP, Foerster J, and Lukens JN, "Wintrobes Clinical Hematology", Lippincott Williams & Wilkins 11th ed., 2003. Hoffman R, Benz E, Shattil S, Furie B, Cohen H, "Hematology: Basic Principles and Practice", Churchill Livingstone, 4th ed, 2004. Hoffbrand AV, Catovsky D, and Tuddenham E, "Postgraduate Haematology", Blackwell, 5th ed., 2005. Hoffbrand AV, Moss PAH, and Pettit JE, "Essential Haematology", Blackwell, 5th ed., 2006. == External links == | 0-1
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The second figure of importance in this era was Ambroise Paré (sometimes spelled "Ambrose"), a French army surgeon from the 1530s until his death in 1590. The practice for cauterizing gunshot wounds on the battlefield had been to use boiling oil; an extremely dangerous and painful procedure. Paré began to employ a less irritating emollient, made of egg yolk, rose oil and turpentine. He also described more efficient techniques for the effective ligation of the blood vessels during an amputation. Modern surgery
The discipline of surgery was put on a sound, scientific footing during the Age of Enlightenment in Europe. An important figure in this regard was the Scottish surgical scientist, John Hunter, generally regarded as the father of modern scientific surgery. He brought an empirical and experimental approach to the science and was renowned around Europe for the quality of his research and his written works. Hunter reconstructed surgical knowledge from scratch; refusing to rely on the testimonies of others, he conducted his own surgical experiments to determine the truth of the matter. To aid comparative analysis, he built up a collection of over 13,000 specimens of separate organ systems, from the simplest plants and animals to humans. He greatly advanced knowledge of venereal disease and introduced many new techniques of surgery, including new methods for repairing damage to the Achilles tendon and a more effective method for applying ligature of the arteries in case of an aneurysm. | The protozoan is in the smaller of its two forms, called an amastigote, which is round, non-motile, and only 3–7 micrometers in diameter. Inside the stomach of the sandfly, the amastigotes quickly transform into elongated and motile forms called the promastigotes. Promastigote is spindle-shaped, triple the size of the amastigote, and has a single flagellum that allows mobility. The promastigotes live extracellularly in the alimentary canal, reproducing asexually, then migrate to the proximal end of the gut where they become poised for a regurgitational transmission. As the fly bites, the promastigotes are released from the proboscis and introduced locally at the bite site.Once inside the human host, promastigotes invade macrophages. Inside the cells they transform back into the smaller amastigote form. The amastigotes replicate in the most hostile part of the macrophage cell, inside the phagolysosome, whose normal defensive response they are able to prevent. After repeated multiplication, they break down their host cell by sheer pressure of mass, but there is some recent speculation that they are able to leave the cell by triggering the exocytosis response of the macrophage. The daughter cells protozoans then migrate to fresh cells or through the bloodstream to find new hosts. In this way the infection is progressive, spreading to the hosts mononuclear phagocyte system, particularly the spleen and liver. The free amastigotes in peripheral tissues are then ingested by sandfly to enter another cycle. Regulatory T and B cells
The cell-mediated immunity (CMI) that kills Leishmania also produces inflammation. If the inflammation is excessive, it can cause tissue damage. | 0-1
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Melioidosis is an infectious disease caused by a gram-negative bacterium called Burkholderia pseudomallei. Most people exposed to B. pseudomallei experience no symptoms; however, those who do experience symptoms have signs and symptoms that range from mild such as fever and skin changes, to severe with pneumonia, abscesses, and septic shock that could cause death. Approximately 10% of people with melioidosis develop symptoms that last longer than two months, termed "chronic melioidosis".Humans are infected with B. pseudomallei by contact with contaminated soil or water. The bacteria enter the body through wounds, inhalation, or ingestion. Person-to-person or animal-to-human transmission is extremely rare. The infection is constantly present in Southeast Asia particularly in northeast Thailand and northern Australia. In temperate countries such as Europe and the United States, melioidosis cases are usually imported from countries where melioidosis is endemic. The signs and symptoms of melioidosis resemble tuberculosis and misdiagnosis is common. Diagnosis is usually confirmed by the growth of B. pseudomallei from an infected persons blood or other bodily fluid such as pus, sputum, and urine. Those with melioidosis are treated first with an "intensive phase" course of intravenous antibiotics (most commonly ceftazidime) followed by a several-months treatment course of co-trimoxazole. In countries with the advanced healthcare system, approximately 10% of people with melioidosis die from the disease. In less developed countries, the death rate could reach 40%.Efforts to prevent melioidosis include: wearing protective gear while handling contaminated water or soil, practising hand hygiene, drinking boiled water, and avoiding direct contact with soil, water, or heavy rain. | Other uses
It 2008, ESPN reported that four NFL players were being suspended under the steroid policy as a result of taking bumetanide. It is sometimes used for weight loss because, as a diuretic, it removes water, but it also masks other drugs, including steroids, by diluting the contents of the users urine, yielding a lower concentration of filtered substances, which makes them less likely to be detected. Bumetanide was an undisclosed active ingredient in the over-the-counter weight loss supplement StarCaps, which was removed from the market after its presence was discovered by the United States Food and Drug Administration. Side effects
Common side effects include dizziness, low blood pressure, low blood potassium, muscle cramps, and kidney problems. Other serious side effects may include hearing loss and low blood platelets. A large observational study concluded that people with a sulfonamide antibiotic allergy may be allergic to sulfonamide non-antibiotics, such as bumetanide, but this is likely due to certain people being at an increased risk in general to developing allergic reactions rather than cross-reactivity between sulfonamide-containing drugs. In smaller studies, the lack of cross-reactivity between sulfonamide antibiotics and sulfonamide non-antibiotics has been demonstrated. Blood tests are recommended regularly for those on treatment. Safety during pregnancy and breastfeeding is unclear. Mechanism of action
Bumetanide is a loop diuretic and works by decreasing the reabsorption of sodium by the kidneys. The main difference between bumetanide and furosemide is in their bioavailability and potency. | 0-1
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Lilly produced a large number of documents as part of the discovery phase of this litigation, which started in 2004; the documents were ruled to be confidential by a judge and placed under seal, and later themselves became the subject of litigation.In 2006, Lilly paid $700 million to settle around 8,000 of these lawsuits, and in early 2007, Lilly settled around 18,000 suits for $500 million, which brought the total Lilly had paid to settle suits related to the drug to $1.2 billion.A December 2006 New York Times article based on leaked company documents concluded that the company had engaged in a deliberate effort to downplay olanzapines side effects. The company denied these allegations and stated that the article had been based on cherry-picked documents. The documents were provided to the Times by Jim Gottstein, a lawyer who represented mentally ill patients, who obtained them from a doctor, David Egilman, who was serving as an expert consultant on the case. After the documents were leaked to online peer-to-peer, file-sharing networks by Will Hall and others in the psychiatric survivors movement, who obtained copies, in 2007 Lilly filed a protection order to stop the dissemination of some of the documents, which Judge Jack B. Weinstein of the Brooklyn Federal District Court granted. Judge Weinstein also criticized the New York Times reporter, Gottstein, and Egilman in the ruling. The Times of London also received the documents and reported that as early as 1998, Lilly considered the risk of drug-induced obesity to be a "top threat" to Zyprexa sales. | Bicipital tenosynovitis is tendinitis or inflammation of the tendon and sheath lining of the biceps muscle. It is often the result of many years of small tears or other degenerative changes in the tendon first manifesting in middle age, but can be due to a sudden injury. Calcification of the tendon, and osteophytes ("bone spurs") in the intertubercular groove can be apparent on x-rays. The condition (which can also occur in dogs) is commonly treated with physical therapy and cortisone
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In these cases, the dura and arachnoid are split into two separate dural and arachnoidal sacs, each surrounding the corresponding hemicord which are not necessarily symmetric. Each hemicord contains a central canal, one dorsal horn (giving rise to a dorsal nerve root), and one ventral horn (giving rise to a ventral nerve root.) One study showed the bony spur typically situated at the most inferior aspect of the dural cleft. They advised that if the imaging appears to show otherwise, a second spur (present in about 5% of patients with diastematomyelia) is likely to be present. The conus medullaris is situated below the L2 level in more than 75% of these diastematomyelia patients. Thickening of the filum terminale is seen in over half of the cases. While the level of the cleft is variable, it is most commonly found in the lumbar region. The two hemicords usually reunite caudally to the cleft. Occasionally, however, the cleft will extend unusually low and the cord will end with two separate coni medullarae and two fila terminale ("Diplomyelia"). Diagnosis
Adult presentation in diastematomyelia is unusual. With modern imaging techniques, various types of spinal dysraphism are being diagnosed in adults with increasing frequency. The commonest location of the lesion is at first to third lumbar vertebrae. Lumbosacral adult diastematomyelia is even rarer. Bony malformations and dysplasias are generally recognized on plain x-rays. MRI scanning is often the first choice of screening and diagnosis. | MRI generally give adequate analysis of the spinal cord deformities although it has some limitations in giving detailed bone anatomy. Combined myelographic and post-myelographic CT scan is the most effective diagnostic tool in demonstrating the detailed bone, intradural and extradural pathological anatomy of the affected and adjacent spinal canal levels and of the bony spur. Prenatal ultrasound diagnosis of this anomaly is usually possible in the early to mid third-trimester. An extra posterior echogenic focus between the fetal spinal laminae is seen with splaying of the posterior elements, thus allowing for early surgical intervention and have a favorable prognosis. Prenate ultrasound could also detect whether the diastematomyelia is isolated, with the skin intact or association with any serious neural tube defects. Progressive neurological lesions may result from the "tethering cord syndrome" (fixation of the spinal cord) by the diastematomyelia phenomenon or any of the associated disorders such as myelodysplasia, dysraphia of the spinal cord. Treatment
Surgery
Surgical intervention is warranted in patients who present with new onset neurological signs and symptoms or have a history of progressive neurological manifestations that can be related to this abnormality. The surgical procedure required for the effective treatment of diastematomyelia includes decompression (surgery) of neural elements and removal of bony spur. This may be accomplished with or without resection and repair of the duplicated dural sacs. Resection and repair of the duplicated dural sacs is preferred since the dural abnormality may partly contribute to the "tethering" process responsible for the symptoms of this condition. | 11
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Thromboangiitis obliterans, also known as Buerger disease (English ; German: [ˈbʏʁɡɐ]), is a recurring progressive inflammation and thrombosis (clotting) of small and medium arteries and veins of the hands and feet. It is strongly associated with use of tobacco products, primarily from smoking, but is also associated with smokeless tobacco. Signs and symptoms
There is a recurrent acute and chronic inflammation and thrombosis of arteries and veins of the hands and feet. The main symptom is pain in the affected areas, at rest and while walking (claudication). The impaired circulation increases sensitivity to cold. Peripheral pulses are diminished or absent. There are color changes in the extremities. The colour may range from cyanotic blue to reddish blue. Skin becomes thin and shiny. Hair growth is reduced. Ulcerations and gangrene in the extremities are common complications, often resulting in the need for amputation of the involved extremity. Pathophysiology
There are characteristic pathologic findings of acute inflammation and thrombosis (clotting) of arteries and veins of the hands and feet (the lower limbs being more common). The mechanisms underlying Buergers disease are still largely unknown, but smoking and tobacco consumption are major factors associated with it. It has been suggested that the tobacco may trigger an immune response in susceptible persons or it may unmask a clotting defect, either of which could incite an inflammatory reaction of the vessel wall. This eventually leads to vasculitis and ischemic changes in distal parts of limbs.A possible role for Rickettsia in this disease has been proposed. | Pterygium inversum unguis or ventral pterygium is characterized by the adherence of the distal portion of the nailbed to the ventral surface of the nail plate. : 788 The condition may be present at birth or acquired, and may cause pain with manipulation of small objects, typing, and close manicuring of the nail. : 788 secondary due to connective tissue disorders
See also
Pterygium unguis
Nail
List of cutaneous conditions
== References == | 0-1
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Balós concentric sclerosis is a disease in which the white matter of the brain appears damaged in concentric layers, leaving the axis cylinder intact. It was described by József Mátyás Baló who initially named it "leuko-encephalitis periaxialis concentrica" from the previous definition, and it is currently considered one of the borderline forms of multiple sclerosis. Balós concentric sclerosis is a demyelinating disease similar to standard multiple sclerosis, but with the particularity that the demyelinated tissues form concentric layers. Scientists used to believe that the prognosis was similar to Marburg multiple sclerosis, but now they know that patients can survive, or even have spontaneous remission and asymptomatic cases.The concentric ring appearance is not specific to Balós MS. Concentric lesions have also been reported in patients with neuromyelitis optica, standard MS, progressive multifocal leukoencephalopathy, cerebral autosomal dominant arteriopathy with subcortical infarcts, leukoencephalopathy, concomitant active hepatitis C and human herpes virus 6. Pathophysiology
The lesions of the Balós sclerosis belong to the MS lesion pattern III (distal oligodendrogliopathy). Balo concentric sclerosis is now believed to be a variant of pattern III multiple sclerosis and probably due to metabolic problems.The Baló lesions show veins at their center, like those of MS, some suggestive of microhemorrhages or small ectatic venules. Unlike MS, no cortical gray matter lesions appear. | Nature Clinical Practice Neurology. 3 (9): E1. doi:10.1038/ncpneuro0619. PMID 17805242. S2CID 33938721. == External links == | 11
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In hemoglobin H disease, red blood cells containing hemoglobin H inclusions can be visualized on the blood smear using new methylene blue or brilliant cresyl blue stain.Hemoglobin analysis is important for the diagnosis of alpha-thalassemia as it determines the types and percentages of types of hemoglobin present. Several different methods of hemoglobin analysis exist, including hemoglobin electrophoresis, capillary electrophoresis and high-performance liquid chromatography.Molecular analysis of DNA sequences (DNA analysis) can be used for the confirmation of a diagnosis of alpha-thalassemia, particularly for the detection of alpha-thalassemia carriers (deletions or mutations in only one or two alpha-globin genes). Treatment
Treatment for alpha-thalassemia may include blood transfusions to maintain hemoglobin at a level that reduces symptoms of anemia. The decision to initiate transfusions depends on the clinical severity of the disease. Splenectomy is a possible treatment option to increase total hemoglobin levels in cases of worsening anemia due to an overactive or enlarged spleen, or when transfusion therapy is not possible. However, splenectomy is avoided when other options are available due to an increased risk of serious infections and thrombosis.Additionally, gallstones may be a problem that would require surgery. Secondary complications from febrile episode should be monitored, and most individuals live without any need for treatment. Additionally, stem cell transplantation should be considered as a treatment (and cure), which is best done in early age. | Interruptions for longer periods may require the dose of Remodulin to be re-titrated. In patients with mild or moderate liver dysfunction, the initial dose of Remodulin should be decreased to 0.625 ng/kg/min ideal body weight and should be increased cautiously. Remodulin has not been studied in patients with severe liver dysfunction. No studies have been performed in patients with kidney dysfunction. No specific advice about dosing in patients with renal impairment can be given. Inhaled form
The inhaled form of treprostinil was approved by the FDA in July 2009 and is marketed as the trade name Tyvaso. The inhaled form is used with a proprietary inhalation device supplied by the manufacturer. Patients use one ampule with inhalation solution a day, four times a day at least four hours apart. Oral form
The oral form of treprostinil was approved by the FDA in December 2013 and is marketed as the trade name Orenitram. Orenitram is taken 2 or 3 times daily with food. History
During the 1960s a U.K. research team, headed by Professor John Vane began to explore the role of prostaglandins in anaphylaxis and respiratory diseases. Working with a team from the Royal College of Surgeons, Vane discovered that aspirin and other oral anti-inflammatory drugs worked by inhibiting the synthesis of prostaglandins. This finding opened the door to a broader understanding of the role of prostaglandins in the body. Vane and a team from the Wellcome Foundation had identified a lipid mediator they called “PG-X,” which inhibited platelet aggregation. | 0-1
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Functional magnetic resonance imaging (fMRI) of Charles Bonnet Syndrome patients displays a relationship between visual hallucinations and activity in the ventral occipital lobe. A connection between age-related macular degeneration (AMD) and colored visual hallucinations has been presented. Color vision signals travel through the parvocellular layers of the lateral geniculate nucleus (LGN), later transmitting down the color regions of the ventral visual pathway. Due to cone photoreceptor damage located in the macula, there is a significant reduction of visual input to the visual association cortex, stirring endogenous activation in the color areas and thus leading to colored hallucinations. Patients with CBS alongside macular degeneration exhibit hyperactivity in the color areas of the visual association cortex (as shown in fMRIs). Those who have significant ocular disease yet maintain visual acuity may still be susceptible to CBS.The Deep Boltzmann Machine (DBM) is a way of utilizing an undirected probabilistic process in a neural framework. Researchers argue that the DBM has the ability to model features of cortical learning, perception, and the visual cortex (the locus of visual hallucinations). Compelling evidence details the role homeostatic operations in the cortex play in regards to stabilizing neuronal activity. By using the DBM, researchers show that when sensory input is absent, neuron excitability is influenced, thus potentially triggering complex hallucinations. A short-term change in the levels of feedforward and feedback flows of information may intensely affect the presence of hallucinations. In periods of drowsiness, CBS related hallucinations are more prone to arise. | Visual release hallucinations, also known as Charles Bonnet syndrome or CBS, are a type of psychophysical visual disturbance in which a person with partial or severe blindness experiences visual hallucinations. First described by Charles Bonnet in 1760, the term Charles Bonnet syndrome was first introduced into English-speaking psychiatry in 1982. A related type of hallucination that also occurs with lack of visual input is the closed-eye hallucination. Signs and symptoms
People with significant vision loss may have vivid recurrent visual hallucinations (fictive visual percepts). One characteristic of these hallucinations is that they usually are "lilliputian" (hallucinations in which the characters or objects are smaller than normal). Depending on the content, visual hallucinations can be classified as either simple or complex. Simple visual hallucinations are commonly characterized by shapes, photopsias, and grid-like patterns. Complex visual hallucinations consist of highly detailed representations of people and objects. The most common hallucination is of faces or cartoons. Sufferers understand that the hallucinations are not real, and the hallucinations are only visual, that is, they do not occur in any other senses (such as hearing, smell or taste). Visual hallucinations generally appear when the eyes are open, fading once the visual gaze shifts. It is widely claimed that sensory deprivation is instrumental in the progression of CBS. During episodes of inactivity, hallucinations are more likely to occur. | 11
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A cold injury is defined as one in which cold temperature is the agent that causes tissue damage. Types of cold injury include chilblains, trenchfoot, frostbite, and immersion foot. Notes
References
Whayne, Tom French; DeBakey, Michael E. (1984). Cold Injury, Ground Type (PDF). Medical Department, United States Army, in World War II. Washington, D.C.: U.S. Army Center of Military History. OCLC 19967629. Retrieved 25 June 2021. | It makes viewing stereograms possible.Monocular diplopia may be induced in many individuals, even those with normal eyesight, with simple defocusing experiments involving fine, high-contrast lines. Treatment
The appropriate treatment for binocular diplopia depends upon the cause of the condition producing the symptoms. Efforts must first be made to identify and treat the underlying cause of the problem. Treatment options include eye exercises, wearing an eye patch on alternative eyes, prism correction, and in more extreme situations, surgery or botulinum toxin.If diplopia turns out to be intractable, it can be managed as last resort by obscuring part of the patients field of view. This approach is outlined in the article on diplopia occurring in association with a condition called horror fusionis. See also
Binocular vision
Monocular rivalry
Orthoptics
References
Further reading
Fraine L (2012). "Nonsurgical management of diplopia". The American Orthoptic Journal. 62: 13–18. doi:10.3368/aoj.62.1.13. PMID 23002469. S2CID 218549699. External links
Deciphering Diplopia | 0-1
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If eflornithine is prescribed to a patient with Human African trypanosomiasis caused by a trypanosome that contains a mutated or ineffective TbAAT6 gene, then the medication will be ineffective against the disease. Resistance to eflornithine has increased the use of melarsoprol despite its toxicity, which has been linked to the deaths of 5% of recipient HAT patients. Excess facial hair in women
The topical cream is indicated for treatment of facial hirsutism in women. It is the only topical prescription treatment that slows the growth of facial hair. It is applied in a thin layer twice daily, a minimum of eight hours between applications. In clinical studies with Vaniqa, 81% percent of women showed clinical improvement after twelve months of treatment. Positive results were seen after eight weeks. However, discontinuation of the cream caused regrowth of hair back to baseline levels within 8 weeks.Vaniqa treatment significantly reduces the psychological burden of facial hirsutism. Chemo preventative therapy
It has been noted that ornithine decarboxylase (ODC) exhibits high activity in tumor cells, promoting cell growth and division, while absence of ODC activity leads to depletion of putrescine, causing impairment of RNA and DNA synthesis. Typically, drugs that inhibit cell growth are considered candidates for cancer therapy, so eflornithine was naturally believed to have potential utility as an anti-cancer agent. By inhibiting ODC, eflornithine inhibits cell growth and division of both cancerous and noncancerous cells. However, several clinical trials demonstrated minor results. | Market
Vaniqa, granted marketing approval by the US FDA, as well as by the European Commission among others, is currently the only topical prescription treatment that slows the growth of facial hair. Besides being a non-mechanical and non-cosmetic treatment, it is the only non-hormonal and non-systemic prescription option available for women with facial hirsutism. Vaniqa is marketed by Almirall in Europe, SkinMedica in the US, Triton in Canada, Medison in Israel, and Menarini in Australia.Ornidyl, the injectable form of eflornithine hydrochloride, is licensed by Sanofi-Aventis, but is currently discontinued in the US. In other animals
Eflornithine is effective in mice. Bacchi et al. 1980 found the drug to be curative in T. b. brucei infection of mouse and it is generally without toxicity. Klug et al. 2016 are of the opinion that this demonstrates good promise for oral treatment. However although Jansson et al. 2008 also effectively treated mice with it they found the pharmacokinetics of oral administration in rats very negative. Brun et al. 2010 are of the opinion that Janssons results have killed the prospects for oral treatment. References
External links
History of Drug Development for African Sleeping Sickness | 11
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This is due in part to the effect of inhibitory neurons, electrical changes within the excitatory neuron, and the negative effects of adenosine.Focal seizures begin in one area of the brain while generalized seizures begin in both hemispheres. Some types of seizures may change brain structure, while others appear to have little effect. Gliosis, neuronal loss, and atrophy of specific areas of the brain are linked to epilepsy but it is unclear if epilepsy causes these changes or if these changes result in epilepsy.The seizures can be described on different scales, from the cellular level to the whole brain. These are several concomitant factor, which on different scale can "drive" the brain to pathological states and trigger a seizure. Diagnosis
The diagnosis of epilepsy is typically made based on observation of the seizure onset and the underlying cause. An electroencephalogram (EEG) to look for abnormal patterns of brain waves and neuroimaging (CT scan or MRI) to look at the structure of the brain are also usually part of the initial investigations. While figuring out a specific epileptic syndrome is often attempted, it is not always possible. Video and EEG monitoring may be useful in difficult cases. | In the United Kingdom, a full national private pilot license requires the same standards as a professional drivers license. This requires a period of ten years without seizures while off medications. Those who do not meet this requirement may acquire a restricted license if free from seizures for five years. Support organizations
There are organizations that provide support for people and families affected by epilepsy. The Out of the Shadows campaign, a joint effort by the World Health Organization, the International League Against Epilepsy and the International Bureau for Epilepsy, provides help internationally. In the United States, the Epilepsy Foundation is a national organization that works to increase the acceptance of those with the disorder, their ability to function in society and to promote research for a cure. The Epilepsy Foundation, some hospitals, and some individuals also run support groups in the United States. In Australia, the Epilepsy Foundation provides support, delivers education and training and funds research for people living with epilepsy. International Epilepsy Day (World Epilepsy Day) began in 2015 and occurs on the second Monday in February.Purple Day, a different world-wide epilepsy awareness day for epilepsy, was initiated by a nine-year-old Canadian named Cassidy Megan in 2008, and is every year on March 26. Research
Seizure prediction and modeling
Seizure prediction refers to attempts to forecast epileptic seizures based on the EEG before they occur. As of 2011, no effective mechanism to predict seizures has been developed. | 11
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Signs and symptoms
Symptoms are pain and tenderness at the radial side of the wrist, fullness or thickening over the thumb side of the wrist, and difficulty gripping with the affected side of the hand. The onset is often gradual. Pain is made worse by movement of the thumb and wrist, and may radiate to the thumb or the forearm. Causes
The cause of de Quervains disease is not established. Evidence regarding a possible relation with activity and occupation is debated. A systematic review of potential risk factors discussed in the literature did not find any evidence of a causal relationship with activity or occupation. However, researchers in France found personal and work-related factors were associated with de Quervains disease in the working population; wrist bending and movements associated with the twisting or driving of screws were the most significant of the work-related factors. Proponents of the view that De Quervain syndrome is a repetitive strain injury consider postures where the thumb is held in abduction and extension to be predisposing factors. Workers who perform rapid repetitive activities involving pinching, grasping, pulling or pushing have been considered at increased risk. These movements are associated with many types of repetitive housework such as chopping vegetables, stirring and scrubbing pots, vacuuming, cleaning surfaces, drying dishes, pegging out washing, mending clothes, gardening, harvesting and weeding. | Mechanism of action
Alprazolam is classed as a high-potency triazolobenzodiazepine: a benzodiazepine with a triazole ring attached to its structure. As a benzodiazepine, alprazolam produces a variety of therapeutic and adverse effects by binding to the GABAA benzodiazepine receptor site and modulating its function; GABA receptors are the most prolific inhibitory receptor within the brain. The GABA chemical and receptor system mediates inhibitory or calming effects of alprazolam on the nervous system. Binding of alprazolam to the GABAA receptor, a chloride ion channel, enhances the effects of GABA, a neurotransmitter. When GABA binds the GABAA receptor the channel opens and chloride enters the cell which makes it more resistant to depolarisation. Therefore, alprazolam has a depressant effect on synaptic transmission to reduce anxiety.The GABAA receptor is made up of 5 subunits out of a possible 19, and GABAA receptors made up of different combinations of subunits have different properties, different locations within the brain, and, importantly, different activities with regard to benzodiazepines. Alprazolam and other triazolobenzodiazepines such as triazolam that have a triazole ring fused to their diazepine ring appear to have antidepressant properties. This is perhaps due to the similarities shared with tricyclic antidepressants, as they have two benzene rings fused to a diazepine ring. Alprazolam causes a marked suppression of the hypothalamic–pituitary–adrenal axis. The therapeutic properties of alprazolam are similar to other benzodiazepines and include anxiolytic, anticonvulsant, muscle relaxant, hypnotic and amnesic; however, it is used mainly as an anxiolytic. | 0-1
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Elevated levels of ΔFosB leads to increases in brain-derived neurotrophic factor (BDNF) levels, which in turn increases the number of dendritic branches and spines present on neurons involved with the nucleus accumbens and prefrontal cortex areas of the brain. This change can be identified rather quickly, and may be sustained weeks after the last dose of the drug. Transgenic mice exhibiting inducible expression of ΔFosB primarily in the nucleus accumbens and dorsal striatum exhibit sensitized behavioural responses to cocaine. They self-administer cocaine at lower doses than control, but have a greater likelihood of relapse when the drug is withheld. ΔFosB increases the expression of AMPA receptor subunit GluR2 and also decreases expression of dynorphin, thereby enhancing sensitivity to reward.DNA damage is increased in the brain of rodents by administration of cocaine. During DNA repair of such damages, persistent chromatin alterations may occur such as methylation of DNA or the acetylation or methylation of histones at the sites of repair. These alterations can be epigenetic scars in the chromatin that contribute to the persistent epigenetic changes found in cocaine addiction. Dependence and withdrawal
Cocaine dependence develops after even brief periods of regular cocaine use and produces a withdrawal state with emotional-motivational deficits upon cessation of cocaine use. During pregnancy
Crack baby is a term for a child born to a mother who used crack cocaine during her pregnancy. The threat that cocaine use during pregnancy poses to the fetus is now considered exaggerated. | This data has been assembled into The Clean Fuels Report comparison of fuel emissions and show that ethanol exhaust generates 2.14 times as much ozone as gasoline exhaust. When this is added into the custom Localized Pollution Index (LPI) of The Clean Fuels Report, the local pollution of ethanol (pollution that contributes to smog) is rated 1.7, where gasoline is 1.0 and higher numbers signify greater pollution. The California Air Resources Board formalized this issue in 2008 by recognizing control standards for formaldehydes as an emissions control group, much like the conventional NOx and Reactive Organic Gases (ROGs).World production of ethanol in 2006 was 51 gigalitres (1.3×1010 US gal), with 69% of the world supply coming from Brazil and the United States. More than 20% of Brazilian cars are able to use 100% ethanol as fuel, which includes ethanol-only engines and flex-fuel engines. Flex-fuel engines in Brazil are able to work with all ethanol, all gasoline or any mixture of both. In the US flex-fuel vehicles can run on 0% to 85% ethanol (15% gasoline) since higher ethanol blends are not yet allowed or efficient. Brazil supports this fleet of ethanol-burning automobiles with large national infrastructure that produces ethanol from domestically grown sugar cane. Sugar cane not only has a greater concentration of sucrose than corn (by about 30%), but is also much easier to extract. | 0-1
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Effusions are large, restricting range of motion but significant pain is not a feature. There is usually stiffness. Tenderness of the joint may or may not be present. Aspirated synovial fluid is usually sterile but will sometimes show elevated cell count (>100 cells/mL) with 50% being polymorphonuclear leukocytes.Onset of effusions are sudden with no particular trigger or stimulus. Each episode lasts for a few days to about a week and recurs in cycles of 7 to 11 days with extremes of 3 days to 30 days also reported. Sometimes the joint may begin to swell again as soon as the fluid has subsided. Where both knees are affected concurrently, as one joint ceases to swell the other may become involved.The cycle of joints swellings have been reported as being very regular, even predictable. This has been a characteristic feature of IH in many case reports. However, over the longer-term especially, these cycles of effusion and recovery may not be as constant as first reported.In women, many cases seem to begin at puberty. Episodes of knee swelling may coincide the menstrual cycle. In nearly all case reports, pregnancy seems to suppress the condition but after birth, during lactation, it returns.In the main, patients are mostly free of other symptoms. Fever is rare. There no signs of local inflammation or lymphatic involvement. Laboratory tests are generally normal or within reference limits. | Cause
The cause is unknown but allergic and auto-inflammatory mechanisms have been proposed.In a 1957 review of IH, Mattingly did not find evidence that the condition is inherited unlike Reimann who, in 1974, describes the condition as “heritable, non-inflammatory, and afebrile”.More recently, specific association with the Mediterranean fever gene, MEFV, has been proposed. So, with some individuals carrying gene mutations (MEFV and also TRAPS-related genes), the native immune system seems to plays a role in the development of IH, i.e. there is an auto-immune component to the condition. Pathophysiology
Involvement of mast cells has been reported reflecting a possible immunoallergic aspect to IH. Mattingly suggests that IH may be an unusual variant of rheumatoid arthritis, and some patients may go on to develop RA. Joint damage however does not generally occur and only the synovial membrane is affected by a ‘non-inflammatory oedema’.With regard to the periodic nature of effusions, Reimann theorises that:
“…either an inherent rhythm or a feedback mechanism (Morley, 1970) excites bioclocks in the hypothalamus or in the synovial membrane (Richter, 1960). These Zeitgebers provoke sudden accumulation of plasma in the lining and spaces of joints, tendon and ligament sheaths.”
Diagnosis
There is no specific test for this condition. Diagnosis is based on signs and symptoms, and exclusion of other conditions. Differential diagnoses
Rheumatoid arthritis. Confusion with rheumatoid arthritis may be common even though IH is a non-inflammatory condition without the many signs and symptoms associated with RA. So far, an association with HLA-B27 gene in IH has not been reported. | 11
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Most pathologists use the World Health Organization classification system for testicular tumors:
Germ cell tumors
Precursor lesions
Intratubular germ cell neoplasia
Unclassified type (carcinoma in situ)
Specified types
Tumors of one histologic type (pure forms)
Seminoma
Variant - Seminoma with syncytiotrophoblastic cells
Spermatocytic tumor
Variant - spermatocytic tumor with sarcoma
Embryonal carcinoma
Yolk sac tumor
Trophoblastic tumors
Choriocarcinoma
Variant - monophasic choriocarcinoma
Placental site trophoblastic tumour
Cystic trophoblastic tumor
Teratoma
Variant - Dermoid cyst
Variant - Epidermoid cyst
Variant - Monodermal teratoma (Carcinoid), Primitive neuroectodermal tumor (PNET), Nephroblastoma-like tumor, others. | The parents of a child with an autosomal recessive disorder are carriers of one copy of the defective gene, but are usually not affected by the disorder.The prevalence of mutations associated with this condition reach 68% to 81% in certain arctic coastal populations, suggesting that the condition had some adaptive value in those habitats at some time. Fewer than 50 people have been identified with this condition. Diagnosis
Genetic testing is necessary for diagnosis. Differential diagnosis
This condition is sometimes mistaken for fatty acid and ketogenesis disorders such as Medium-chain acyl-coenzyme A dehydrogenase deficiency (MCAD), other long-chain fatty acid oxidation disorders such as Carnitine palmitoyltransferase II deficiency (CPT-II) and Reye syndrome. Treatment
Treatment for this condition is supportive. There is no cure. Affected people should eat a high-carbohydrate, low-fat diet and avoid fasting. See also
Primary carnitine deficiency
Carnitine palmitoyltransferase II deficiency
References
External links
This article incorporates public domain text from The U.S. National Library of Medicine | 0-1
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People with progressively worsening headache also warrant imaging, as they may have a mass or a bleed that is gradually growing, pressing on surrounding structures and causing worsening pain. People with neurological findings on exam, such as weakness, also need further workup.The American Headache Society recommends using "SSNOOP", a mnemonic to remember the red flags for identifying a secondary headache:
Systemic symptoms (fever or weight loss)
Systemic disease (HIV infection, malignancy)
Neurologic symptoms or signs
Onset sudden (thunderclap headache)
Onset after age 40 years
Previous headache history (first, worst, or different headache)Other red flag symptoms include:
Old headaches
Old headaches are usually primary headaches and are not dangerous. They are most often caused by migraines or tension headaches. Migraines are often unilateral, pulsing headaches accompanied by nausea or vomiting. There may be an aura (visual symptoms, numbness or tingling) 30–60 minutes before the headache, warning the person of a headache. Migraines may also not have auras. Tension-type headaches usually have bilateral "bandlike" pressure on both sides of the head usually without nausea or vomiting. However, some symptoms from both headache groups may overlap. It is important to distinguish between the two because the treatments are different. The mnemonic POUND helps distinguish between migraines and tension-type headaches. POUND stands for:
One review article found that if 4–5 of the POUND characteristics are present, a migraine is 24 times as likely a diagnosis than a tension-type headache (likelihood ratio 24). If 3 characteristics of POUND are present, migraine is 3 times more likely a diagnosis than tension type headache (likelihood ratio 3). | Stillbirth and an excess of amniotic fluid (polyhydramnios) are common during pregnancy with a HLS-affected foetus, with cases of up to 8 litres cited compared to the normal 1 litre. Less common symptoms such as abnormally small eyes and a broad nose are also possible. Cause
Genetic
HLS is caused by a genetic missense mutation of the HYLS1 gene, encoding for Hydrolethalus syndrome protein 1, on chromosome 11; a single base change to the amino acid sequence for HYLS1 in exon 6 involves the replacement of aspartic acid 211 with glycine (D211G) in the polypeptide chain. Exon 6 is the only protein coding exon in HYLS1; proper functioning of exons 1-5 ensures regulation and expression of the entire protein. HLS is an autosomal recessive syndrome; development is only possible if both parents carry the defective gene, and in that instance, the risk of the foetus developing the syndrome is 25%. HLS is a member of the Finnish disease heritage, with incidences more common in Finland than the rest of the world; roughly 1 in 20,000 developing foetuses are affected in Finland. Rare cases in other regions have also been documented, often with less severe phenotypes as a result of allele variability across countries, allowing survival of affected offspring for up to several months. Individuals of Finnish descent are advised to undergo genetic testing before attempting to conceive.Prior to the discovery of HLS, the HYLS1 gene was unknown, and similar genes within humans have not been identified. | 0-1
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The surgeon then uses a specially designed instrument to separate and remove the lenticule through the incision, leaving the anterior lamellae of the cornea intact. No excimer laser is used in the "ReLEx-procedures". Surface procedures
The excimer laser is used to ablate the most anterior portion of the corneal stroma. These procedures do not require a partial thickness cut into the stroma. Surface ablation methods differ only in the way the epithelial layer is handled. Photorefractive keratectomy (PRK) is an outpatient procedure generally performed with local anesthetic eye drops (as with LASIK/LASEK). It is a type of refractive surgery which reshapes the cornea by removing microscopic amounts of tissue from the corneal stroma, using a computer-controlled beam of light (excimer laser). The difference from LASIK is that the top layer of the epithelium is removed (and a bandage contact lens is used), so no flap is created. Recovery time is longer with PRK than with LASIK, though the outcome (after 3 months) is about the same (very good). More recently, customized ablation has been performed with LASIK, LASEK, and PRK. Transepithelial photorefractive keratectomy (TransPRK) is a laser-assisted eye surgery to correct refraction errors of human eye cornea. It employs excimer laser to ablate outer layer of cornea, epithelium, as well its connective tissue, stroma, to correct eye optical power. | It involves cutting a flap in the cornea and pulling it back to expose the corneal bed, then using an excimer laser to ablate the exposed surface to the desired shape, and then replacing the flap. The name LASIK was coined in 1991 by University of Crete and the Vardinoyannion Eye. The patents related to so-called broad-beam LASIK and PRK technologies were granted to US companies including Visx and Summit during 1990–1995 based on the fundamental US patent issued to IBM (1983) which claimed the use of UV laser for the ablation of organic tissues. In 1991, J.T. Lin, Ph.D. (a Chinese Physicist) was granted a US patent for a new technology using a flying-spot for customized LASIK currently used worldwide. The first US patent using an eye-tracking device to prevent decentration in LASIK procedures was granted to another Chinese Physicist, Dr. S. Lai in 1993. Techniques
Flap procedures
Excimer laser ablation is done under a partial-thickness lamellar corneal flap. Automated lamellar keratoplasty (ALK): The surgeon uses an instrument called a microkeratome to cut a thin flap of the corneal tissue. The flap is lifted like a hinged door, targeted tissue is removed from the corneal stroma, again with the microkeratome, and then the flap is replaced. Laser-assisted in situ Keratomileusis (LASIK): The surgeon uses either a microkeratome or a femtosecond laser to cut a flap of the corneal tissue (usually with a thickness of 100–180 micrometres). | 11
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Aspirin and other heart drugs also appear to help in the treatment of this disease, even in extreme cases. After the patient has been diagnosed, and myocardial infarction (heart attack) ruled out, the aspirin regimen may be discontinued, and treatment becomes that of supporting the patient. There is currently no internationally agreed protocol for treatment of this condition.While medical treatments are important to address the acute symptoms of takotsubo cardiomyopathy, further treatment includes lifestyle changes. It is important that the individual stay physically healthy while learning and maintaining methods to manage stress, and to cope with future difficult situations.Although the symptoms of takotsubo cardiomyopathy usually go away on their own and the condition completely resolves itself within a few weeks, some serious short and long-term complications can happen that must be treated. These most commonly include congestive heart failure and very low blood pressure, and less commonly include blood clotting in the apex of the left ventricle, irregular heart beat, and tearing of the heart wall. Heart failure
For patients in acute heart failure, ACE inhibitors, angiotensin receptor blockers, and beta blockers, are considered mainstays of heart failure treatment. But use of beta blockers specifically for takotsubo cardiomyopathy is controversial, because they may confer no benefit. Low blood pressure
For people with cardiogenic shock, medical treatment is based on whether a left ventricular outflow tract (LVOT) obstruction is present. Therefore, early echocardiography is necessary to determine proper management. | A 2009 large case series from Europe found that takotsubo cardiomyopathy was slightly more frequent during the winter season. This may be related to two possible/suspected pathophysiological causes: coronary spasms of microvessels, which are more prevalent in cold weather, and viral infections – such as Parvovirus B19 – which occur more frequently during the winter. Sex
Women, specifically postmenopausal women, are at greatest risk of developing TTS. This has led some researchers to theorize about the possible protective effects of estrogen in preventing TTS. Genetic risk factors
It is currently being investigated if certain genetic traits associated with catecholamine receptors found on cardiac muscle cells play a role in the development of TTS. There is limited evidence tying TTS directly to a specific genetic expression or mutation, however there is currently a widely held hypothesis supporting the idea of the interaction between environmental factors and the interplay of genetic predisposition leading to the susceptibility to microvascular alterations that contribute to the TTS disease process. Hormonal dysregulation
Certain endocrine diseases including pheochromocytoma and thyrotoxicosis have been identified as potential risk factors for TTS. The relationship between thyroid function and stress cardiomyopathy is marked by a dual phenotype, where both impending primary hyperthyroidism and a high set point of thyroid homeostasis (encoding type 2 allostatic load) are common phenomena. A multi-centre observation study found normal thyroid function to be the exception rather than the rule in TTS. Especially hyperthyroidism is highly prevalent in takotsubo cardiomyopathy, and it seems to predict a poor prognosis in terms of complications and mortality. | 11
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Treatment
Treatment for light bruises is minimal and may include RICE (rest, ice, compression, and elevation), painkillers (particularly NSAIDs) and, later in recovery, light stretching exercises. Particularly, immediate application of ice while elevating the area may reduce or completely prevent swelling by restricting blood flow to the area and preventing internal bleeding. Rest and preventing re-injury is essential for rapid recovery. Very gently massaging the area and applying heat may encourage blood flow and relieve pain according to the Gate control theory of pain, although causing additional pain may indicate the massage is exacerbating the injury. As for most injuries, these techniques should not be applied until at least three days following the initial damage to ensure all internal bleeding has stopped, because although increasing blood flow will allow more healing factors into the area and encourage drainage, if the injury is still bleeding this will allow more blood to seep out of the wound and cause the bruise to become worse. In most cases hematomas spontaneously revert, but in cases of large hematomas or those localized in certain organs (e.g., the brain), the physician may perform a puncture of the hematoma to allow blood to exit. History
Folk medicine, including ancient medicine of Egyptians, Greeks, Celts, Turks, Slavs, Mayans, Aztecs and Chinese, has used bruising as a treatment for some health problems. The methods vary widely and include cupping, scraping, and slapping. Fire cupping uses suction which causes bruising in patients. | Chloroquine retinopathy is a form of toxic retinopathy (damage of the retina) caused by the drugs chloroquine or hydroxychloroquine, which are sometimes used in the treatment of autoimmune disorders such as rheumatoid arthritis and systemic lupus erythematosus. This eye toxicity limits long-term use of the drugs. Presentation
The risk of toxicity is low for individuals without complicating conditions during the first five years of treatment using less than 6.5 mg/kg/day of hydroxychloroquine or 3 mg/kg/day of chloroquine, and/or cumulative doses of less than 1000 gram and 460 gram (total dose), respectively. Some physicians suggest that lean body weight is more accurate when calculating daily dosage.Most patients are routinely given 400 mg of hydroxychloroquine daily (or 250 mg chloroquine). This dose is considered acceptable. Early stage
The earliest signs of toxicity include bilateral paracentral visual field changes (best detected with a red test object) and a subtle granular depigmentation of the paracentral RPE. Advanced stage
With continued drug exposure, there is progressive development of a bilateral atrophic bulls-eye maculopathy and paracentral scotomata, which may in severe cases ultimately spread over the entire fundus, causing widespread retinal atrophy and visual loss. Pathophysiology
Both agents bind to melanin pigment in the RPE, and this may serve to concentrate the drugs or to prolong their adverse effects. Diagnosis
Patients and their primary care physicians must be made fully aware of the ophthalmic risks and the need for regular screening examinations to detect retinal toxicity at an early stage. | 0-1
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: 45–57 TCM focuses on tracing and correcting underlying disharmony, in terms of deficiency and excess, using the complementary and opposing forces of yin and yang (陰陽), to create a balanced flow of qi. Qi is believed to be cultivated and stored in three main dantian energy centers and to travel through the body along twelve main meridians (Jīng Luò 經絡), with numerous smaller branches and tributaries. The main meridians correspond to twelve main organs ) (Zàng fǔ 臟腑). Qi is balanced in terms of yin and yang in the context of the traditional system of Five Phases (Wu xing 五行). A person is believed to become ill or die when qi becomes diminished or unbalanced. Health is believed to be returned by rebuilding qi, eliminating qi blockages, and correcting qi imbalances. These TCM concepts do not translate readily to modern science and medicine. Daoism
In Daoism, various practices now known as Daoist qigong are claimed to provide a way to achieve longevity and spiritual enlightenment, as well as a closer connection with the natural world. Buddhism
In Buddhism meditative practices now known as Buddhist qigong are part of a spiritual path that leads to spiritual enlightenment or Buddhahood. Confucianism
In Confucianism practices now known as Confucian qigong provide a means to become a Junzi (君子) through awareness of morality. Contemporary qigong
In contemporary China, the emphasis of qigong practice has shifted away from traditional philosophy, spiritual attainment, and folklore, and increasingly to health benefits, traditional medicine and martial arts applications, and a scientific perspective. | A nuchal cord was diagnosed if the cord was visualized lying around at least 3 of the 4 sides of the neck. A cord was actually present at delivery in 52 of the 289 women. Only 18 of the 52 cords or 35% of the nuchal cords were detected on ultrasound done immediately before delivery, and 65% of nuchal cords were not detected. Of the 237 cases where there was no cord at delivery, ultrasound had false positive results, i.e. diagnosed a cord in 44 of the 237 cases (19%) in which there was no cord present at all. In this study, ultrasound was only 35% accurate at finding a single loop, and only 60% accurate at detecting a nuchal cord wrapped multiple times around the neck.In no study was it possible by ultrasound to distinguish between a loose or a tight cord, although at least 3 attempted to do so. Peregrine concludes that ultrasound diagnosis of nuchal cords will only be useful if doctors are able to do so reliably and predict which of those fetuses are likely to have a problem., However, perinatologists routinely look for umbilical cord issues in monoamniotic twins. Studies have shown an improvement in outcomes where cord entanglement was prenatally identified in these cases. Ultrasound measurement of the velocity of flow in the cord may be useful in the management of twins and chronically growth-retarded fetuses. Of course this depends on the training of the sonographer. | 0-1
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Methane and hydrogen are flammable, and so flatus can be ignited if it contains adequate amounts of these components.Not all humans produce flatus that contains methane. For example, in one study of the faeces of nine adults, only five of the samples contained archaea capable of producing methane. The prevalence of methane over hydrogen in human flatus may correlate with obesity, constipation and irritable bowel syndrome, as archaea that oxidise hydrogen into methane promote the metabolisms ability to absorb fatty acids from food.The remaining trace (<1% volume) compounds contribute to the odor of flatus. Historically, compounds such as indole, skatole, ammonia and short chain fatty acids were thought to cause the odor of flatus. More recent evidence proves that the major contribution to the odor of flatus comes from a combination of volatile sulfur compounds. Hydrogen sulfide, methyl mercaptan (also known as methanethiol), dimethyl sulfide, dimethyl disulfide and dimethyl trisulfide are present in flatus. The benzopyrrole volatiles indole and skatole have an odor of mothballs, and therefore probably do not contribute greatly to the characteristic odor of flatus. In one study, hydrogen sulfide concentration was shown to correlate convincingly with perceived bad odor of flatus, followed by methyl mercaptan and dimethyl sulfide. This is supported by the fact that hydrogen sulfide may be the most abundant volatile sulfur compound present. These results were generated from subjects who were eating a diet high in pinto beans to stimulate flatus production. | Thirdly, the volume of flatus produced by patients with irritable bowel syndrome who have pain and abdominal distension would be tolerated in normal subjects without any complaints of pain. Patients who complain of bloating frequently can be shown to have objective increases in abdominal girth, often increased throughout the day and then resolving during sleep. The increase in girth combined with the fact that the total volume of flatus is not increased led to studies aiming to image the distribution of intestinal gas in patients with bloating. They found that gas was not distributed normally in these patients: there was segmental gas pooling and focal distension. In conclusion, abdominal distension, pain and bloating symptoms are the result of abnormal intestinal gas dynamics rather than increased flatus production. Excessive volume
The normal range of volumes of flatus in normal individuals varies hugely (476–1,491 mL/24 h). All intestinal gas is either swallowed environmental air, present intrinsically in foods and beverages, or the result of gut fermentation. Swallowing small amounts of air occurs while eating and drinking. This is emitted from the mouth by eructation (burping) and is normal. Excessive swallowing of environmental air is called aerophagia, and has been shown in a few case reports to be responsible for increased flatus volume. This is, however, considered a rare cause of increased flatus volume. Gases contained in food and beverages are likewise emitted largely through eructation, e.g., carbonated beverages. Endogenously produced intestinal gases make up 74 percent of flatus in normal subjects. | 11
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In retrospect, Smith played a major role in both medical research and raising awareness about coccidioidomycosis, especially when he became dean of the School of Public Health at the University of California at Berkeley in 1951. Coccidioides immitis was considered by the United States during the 1950s and 1960s as a potential biological weapon. The strain selected for investigation was designated with the military symbol OC, and initial expectations were for its deployment as a human incapacitant. Medical research suggested that OC might have had some lethal effects on the populace, and Coccidioides immitis started to be classified by the authorities as a threat to public health. However, Coccidioides immitis was never weaponized to the publics knowledge, and most of the military research in the mid-1960s was concentrated on developing a human vaccine. Currently, it is not on the U.S. Department of Health and Human Services or Centers for Disease Control and Preventions list of select agents and toxins. In 2002, Coccidioides posadasii was identified as genetically distinct from Coccidioides immitis despite their morphologic similarities and can also cause coccidioidomycosis. Research
As of 2013, there is no vaccine available to prevent infection with Coccidioides immitis or Coccidioides posadasii, but efforts to develop such a vaccine are underway. Other animals
Valley fever is not contagious. In dogs, the most common symptom of coccidioidomycosis is a chronic cough, which can be dry or moist. Other symptoms include fever (in approximately 50% of cases), weight loss, anorexia, lethargy, and depression. | It is generally associated with high doses (>4 mg/kg per hour or >67 mcg/kg per minute) and prolonged use (>48 hours) though it has been reported with high-dose short-term infusions.Additional proposed risk factors include a young age, critical illness, high fat and low carbohydrate intake, inborn errors of mitochondrial fatty acid oxidation, and concomitant catecholamine infusion or steroid therapy. Characteristics of PRIS include acute refractory bradycardia, severe metabolic acidosis, cardiovascular collapse, rhabdomyolysis, hyperlipidemia, renal failure, and hepatomegaly.The incidence of PRIS is unknown, but it is probably less than 1 percent. Mortality is variable but high (33 to 66 percent). Treatment involves discontinuation of the propofol infusion and supportive care. Risk Factors
Predisposing factors seem to include young age, severe critical illness of central nervous system or respiratory origin, exogenous catecholamine or glucocorticoid administration, inadequate carbohydrate intake and subclinical mitochondrial disease. Treatment
Treatment options are limited and are usually supportive, including hemodialysis with cardiorespiratory support. == References == | 0-1
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The letter x is used to represent the number of chromosomes in a single set:
haploid (one set; 1x)
diploid (two sets; 2x)
triploid (three sets; 3x), for example sterile saffron crocus, or seedless watermelons, also common in the phylum Tardigrada
tetraploid (four sets; 4x), for example Salmonidae fish, the cotton Gossypium hirsutum
pentaploid (five sets; 5x), for example Kenai Birch (Betula kenaica)
hexaploid (six sets; 6x), for example some species of wheat, kiwifruit
heptaploid or septaploid (seven sets; 7x)
octaploid or octoploid, (eight sets; 8x), for example Acipenser (genus of sturgeon fish), dahlias
decaploid (ten sets; 10x), for example certain strawberries
dodecaploid or duodecaploid (twelve sets; 12x), for example the plants Celosia argentea and Spartina anglica or the amphibian Xenopus ruwenzoriensis. Classification
Autopolyploidy
Autopolyploids are polyploids with multiple chromosome sets derived from a single taxon. Two examples of natural autopolyploids are the piggyback plant, Tolmiea menzisii and the white sturgeon, Acipenser transmontanum. Most instances of autopolyploidy result from the fusion of unreduced (2n) gametes, which results in either triploid (n + 2n = 3n) or tetraploid (2n + 2n = 4n) offspring. Triploid offspring are typically sterile (as in the phenomenon of triploid block), but in some cases they may produce high proportions of unreduced gametes and thus aid the formation of tetraploids. This pathway to tetraploidy is referred to as the triploid bridge. Triploids may also persist through asexual reproduction. In fact, stable autotriploidy in plants is often associated with apomictic mating systems. In agricultural systems, autotriploidy can result in seedlessness, as in watermelons and bananas. | In some situations, polyploid crops are preferred because they are sterile. For example, many seedless fruit varieties are seedless as a result of polyploidy. Such crops are propagated using asexual techniques, such as grafting. Polyploidy in crop plants is most commonly induced by treating seeds with the chemical colchicine. Examples
Triploid crops: some apple varieties (such as Belle de Boskoop, Jonagold, Mutsu, Ribston Pippin), banana, citrus, ginger, watermelon, saffron crocus, white pulp of coconut
Tetraploid crops: very few apple varieties, durum or macaroni wheat, cotton, potato, canola/rapeseed, leek, tobacco, peanut, kinnow, Pelargonium
Hexaploid crops: chrysanthemum, bread wheat, triticale, oat, kiwifruit
Octaploid crops: strawberry, dahlia, pansies, sugar cane, oca (Oxalis tuberosa)
Dodecaploid crops: some sugar cane hybridsSome crops are found in a variety of ploidies: tulips and lilies are commonly found as both diploid and triploid; daylilies (Hemerocallis cultivars) are available as either diploid or tetraploid; apples and kinnow mandarins can be diploid, triploid, or tetraploid. Fungi
Besides plants and animals, the evolutionary history of various fungal species is dotted by past and recent whole-genome duplication events (see Albertin and Marullo 2012 for review). Several examples of polyploids are known:
autopolyploid: the aquatic fungi of genus Allomyces, some Saccharomyces cerevisiae strains used in bakery, etc. allopolyploid: the widespread Cyathus stercoreus, the allotetraploid lager yeast Saccharomyces pastorianus, the allotriploid wine spoilage yeast Dekkera bruxellensis, etc. paleopolyploid: the human pathogen Rhizopus oryzae, the genus Saccharomyces, etc.In addition, polyploidy is frequently associated with hybridization and reticulate evolution that appear to be highly prevalent in several fungal taxa. | 11
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In the posterior thigh it first gives off branches to the short head of the biceps femoris and then divides into the tibial (L4-S3) and common fibular nerves (L4-S2). The fibular nerve continues down on the medial side of biceps femoris, winds around the fibular neck and enters the front of the lower leg. There it divides into a deep and a superficial terminal branch. The superficial branch supplies the fibularis muscles and the deep branch enters the extensor compartment; both branches reaches into the dorsal foot. In the thigh, the tibial nerve gives off branches to semitendinosus, semimembranosus, adductor magnus, and the long head of the biceps femoris. The nerve then runs straight down the back of the leg, through the popliteal fossa to supply the ankle flexors on the back of the lower leg and then continues down to supply all the muscles in the sole of the foot. The pudendal (S2-S4) and coccygeal nerves (S5-Co2) supply the muscles of the pelvic floor and the surrounding skin.The lumbosacral trunk is a communicating branch passing between the sacral and lumbar plexuses containing ventral fibers from L4. The coccygeal nerve, the last spinal nerve, emerges from the sacral hiatus, unites with the ventral rami of the two last sacral nerves, and forms the coccygeal plexus. Lower leg and foot
The lower leg and ankle need to keep exercised and moving well as they are the base of the whole body. | Cerebro-costo-mandibular syndrome is a very rare genetic disorder which is characterized by jaw/chin, palate and rib abnormalities. Signs and symptoms
The following list comprises the most common symptoms people with this disorder exhibit:
Severe micrognathia
Thorax in the shape of a bell
Cleft palate
Neonatal respiratory difficulties
Rib gapsCommon (but not the most) symptoms include:
External auditory canal atresia
Hearing loss
Failure to thrive
Glossoptosis
Intellectual disabilities
Fetal growth delays
Kyphosis
Short height
TracheomalaciaNot common but also not rare symptoms include:
Fifth finger clinodactyly
Cerebral calcification
Hydranencephaly
Meningocele
Microcephaly
Polycystic kidney dysplasia
Myelomeningocele
Porencephalic cyst
Short, hard palate
Spina bifida
Ventricular septal defect
Webbed neck
Causes
This disorder is caused by autosomal dominant mutations in the SNRPB gene, in chromosome 20. Epidemiology
Only 110 cases have been described in medical literature. == References == | 0-1
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Some therapies do attempt to cure pedophilia, but there are no studies showing that they result in a long-term change in sexual preference. Michael Seto suggests that attempts to cure pedophilia in adulthood are unlikely to succeed because its development is influenced by prenatal factors. Pedophilia appears to be difficult to alter but pedophiles can be helped to control their behavior, and future research could develop a method of prevention.There are several common limitations to studies of treatment effectiveness. Most categorize their participants by behavior rather than erotic age preference, which makes it difficult to know the specific treatment outcome for pedophiles. Many do not select their treatment and control groups randomly. Offenders who refuse or quit treatment are at higher risk of offending, so excluding them from the treated group, while not excluding those who would have refused or quit from the control group, can bias the treated group in favor of those with lower recidivism. The effectiveness of treatment for non-offending pedophiles has not been studied. Cognitive behavioral therapy
Cognitive behavioral therapy (CBT) 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. | Cotards syndrome exists in three stages:
Germination stage: symptoms of psychotic depression and of hypochondria appear;
Blooming stage: full development of the syndrome and delusions of negation; and;
Chronic stage: continued severe delusions along with chronic psychiatric depression.Cotards syndrome withdraws the person with the condition from other people due to neglect of their personal hygiene and physical health. Delusions of negation of self prevent the patient from making sense of external reality, which then produces a distorted view of the external world. Such delusions of negation are usually found in schizophrenia. Although a diagnosis of Cotards syndrome does not require the patient to have had hallucinations, the strong delusions of negation are comparable to those found in schizophrenic patients. Distorted reality
The article Betwixt Life and Death: Case Studies of the Cotard Delusion (1996) describes a contemporary case of Cotards delusion which occurred in a Scotsman whose brain was damaged in a motorcycle accident:
[The patients] symptoms occurred in the context of more general feelings of unreality and [of] being dead. In January 1990, after his discharge from hospital in Edinburgh, his mother took him to South Africa. He was convinced that he had been taken to Hell (which was confirmed by the heat) and that he had died of sepsis (which had been a risk early in his recovery), or perhaps from AIDS (he had read a story in The Scotsman about someone with AIDS who died from sepsis), or from an overdose of a yellow fever injection. | 0-1
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A major point of contention is whether transplantation should be allowed at all if the patient is not yet fully biologically dead, and if brain death is acceptable, whether the persons whole brain needs to have died, or if the death of a certain part of the brain is enough for legal and ethical and moral purposes. Most organ donation for organ transplantation is done in the setting of brain death. However, in Japan this is a fraught point, and prospective donors may designate either brain death or cardiac death – see organ transplantation in Japan. In some nations such as Belgium, France, Netherlands, New Zealand, Poland, Portugal, Singapore and Spain, everyone is automatically an organ donor unless they opt out of the system. Elsewhere, consent from family members or next-of-kin is required for organ donation. The non-living donor is kept on ventilator support until the organs have been surgically removed. If a brain-dead individual is not an organ donor, ventilator and drug support is discontinued and cardiac death is allowed to occur. In the United States, where since the 1980s the Uniform Determination of Death Act has defined death as the irreversible cessation of the function of either the brain or the heart and lungs, the 21st century has seen an order-of-magnitude increase of donation following cardiac death. In 1995, only one out of 100 dead donors in the nation gave their organs following the declaration of cardiac death. | Expansive legal definitions of death, such as Spain uses, also increase the pool of eligible donors by allowing physicians to declare a patient to be dead at an earlier stage, when the organs are still in good physical condition. Allowing or forbidding payment for organs affects the availability of organs. Generally, where organs cannot be bought or sold, quality and safety are high, but supply is not adequate to the demand. Where organs can be purchased, the supply increases. Iran adopted a system of paying kidney donors in 1988 and within 11 years it became the only country in the world to clear its waiting list for transplants. Healthy humans have two kidneys, a redundancy that enables living donors (inter vivos) to give a kidney to someone who needs it. The most common transplants are to close relatives, but people have given kidneys to other friends. The rarest type of donation is the undirected donation whereby a donor gives a kidney to a stranger. Less than a few hundred of such kidney donations have been performed. In recent years, searching for altruistic donors via the internet has also become a way to find life saving organs. | 11
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Surgery is most likely to improve vision if there was some remaining vision before surgery, and if surgery is undertaken within a week of the onset of symptoms.Those with relatively mild visual field loss or double vision only may be managed conservatively, with close observation of the level of consciousness, visual fields, and results of routine blood tests. If there is any deterioration, or expected spontaneous improvement does not occur, surgical intervention may still be indicated. If the apoplexy occurred in a prolactin-secreting tumor, this may respond to dopamine agonist treatment.After recovery, people who have had pituitary apoplexy require follow-up by an endocrinologist to monitor for long-term consequences. MRI scans are performed 3–6 months after the initial episode and subsequently on an annual basis. If after surgery some tumor tissue remains, this may respond to medication, further surgery, or radiation therapy with a "gamma knife". Prognosis
In larger case series, the mortality was 1.6% overall. In the group of patients who were unwell enough to require surgery, the mortality was 1.9%, with no deaths in those who could be treated conservatively.After an episode of pituitary apoplexy, 80% of people develop hypopituitarism and require some form of hormone replacement therapy. The most common problem is growth hormone deficiency, which is often left untreated but may cause decreased muscle mass and strength, obesity and fatigue. 60–80% require hydrocortisone replacement (either permanently or when unwell), 50–60% need thyroid hormone replacement, and 60–80% of men require testosterone supplements. | Many pituitary tumors (25%) are found to have areas of hemorrhagic infarction on MRI scans, but apoplexy is not said to exist unless it is accompanied by symptoms.In some instances, lumbar puncture may be required if there is a suspicion that the symptoms might be caused by other problems (meningitis or subarachnoid hemorrhage). This is the examination of the cerebrospinal fluid that envelops the brain and the spinal cord; the sample is obtained with a needle that is passed under local anesthetic into the spine. In pituitary apoplexy the results are typically normal, although abnormalities may be detected if blood from the pituitary has entered the subarachnoid space. If there is remaining doubt about the possibility of subarachnoid hemorrhage (SAH), a magnetic resonance angiogram (MRI with a contrast agent) may be required to identify aneurysms of the brain blood vessels, the most common cause of SAH.Professional guidelines recommend that if pituitary apoplexy is suspected or confirmed, the minimal blood tests performed should include a complete blood count, urea (a measure of renal function, usually performed together with creatinine), electrolytes (sodium and potassium), liver function tests, routine coagulation testing, and a hormonal panel including IGF-1, growth hormone, prolactin, luteinizing hormone, follicle-stimulating hormone, thyroid-stimulating hormone, thyroid hormone, and either testosterone in men or estradiol in women.Visual field testing is recommended as soon as possible after diagnosis, as it quantifies the severity of any optic nerve involvement, and may be required to decide on surgical treatment. | 11
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