Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0033774 (pruritus)
 14,546 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A case of familial cholestasis with cirrhosis is described in a 8 months old boy, presenting with hepatosplenomegaly, portal hypertension, dramatic pruritus, and fluctuating icterus of early post-natal onset. Biological data include positive hepatocyte retention test, with mild hepatocyte cytolysis, without patent hepatocyte insufficiency. The discrepancy between the clinical symptoms and a slight elevation of bilirubin partially conjugated, the absence of elevated blood cholesterol, the absence of evidence of antigen or antibody of virus A or B, the marked elevation of blood biliary acid lead to the suspicion of Byler disease. A liver biopsy with ultrastructural study shows a thickening of the ectoplasm, and the presence of microfilament material in the lumen of partially broken villi. Comparisons are made with the 4 other cases of Byler disease with E.M. study documented in the literature.
 ...
PMID:[Byler's disease. Ultrastructural study. Apropos of a case in an infant]. 654 4

Studies of 14 North American Indian children with a familial type of severe neonatal cholestasis are described. Jaundice occurred during the neonatal period in 9 children, but disappeared before the end of the 1st yr. Progressive liver damage was documented by the persistence of high levels of alkaline phosphatase, moderate elevation of transaminases, and severe pruritus. Serum bile acids were constantly elevated (3.0-119.5 microgram/ml). Early portal hypertension and variceal bleeding necessitated portal-systemic shunts in 7 children. By light microscopy, the early stage was characterized by hepatitis with giant-cell transformation and biliary stasis. Later on, portal fibrosis became evident and was followed by cirrhosis. By electron microscopy bile canaliculi appeared slightly dilated with preservation or only partial loss of microvilli. They were surrounded by a prominent pericanalicular filamentous web. Immunofluorescence studies indicated the presence of action-containing microfilaments. This group of children might represent a human model of microfilament dysfunction-induced cholestasis.
 ...
PMID:Severe familial cholestasis in North American Indian children: a clinical model of microfilament dysfunction? 689 6

On the initiative of the Dutch Surgical Society a consensus meeting was held on December 3rd, 1993 in Utrecht, the Netherlands by the National Organisation for Quality Assurance in Hospitals (CBO), on the diagnosis and treatment of haemorrhoids. The following statements were formulated. Haemorrhoids are vascular cushions, covered by mucosa, originating from the plexus rectalis superior, and are part of the normal anatomy of man. Complaints from haemorrhoids occur if they prolapse. The usual 4-grade classification of haemorrhoids has no direct impact on their treatment. Portal hypertension is not a cause of haemorrhoids. Blood loss, a sensation of prolapse, pruritus and soiling are non-specific symptoms of haemorrhoids. Anaemia may only be attributed to haemorrhoids after other pathology has been excluded. Acute massive anorectal blood loss is frequently caused by traumatic damage to the rectum. Anticoagulant therapy is a risk factor. The presence of unexplained perianal skin lesions neccessitates further proctologic investigation. Haemorrhoids are not palpable on rectal digital examination. In patients under 50 with anorectal blood loss and a history of haemorrhoids, a proctoscopic examination is sufficient. Anorectal blood loss in patients over 50 requires exclusion of higher pathology. The regulation of defaecation and eating habits can have a preventive effect on the development of haemorrhoids. Conservative measures form the basis of treatment for haemorrhoidal complaints. Local antihaemorrhoidal treatment can only be expected to give short-term relief and is not a causal therapy. Barron elastic band ligation and sclerosing, in addition to infrared coagulation are treatment modalities in the outpatient setting that are very effective, inexpensive and optimally patient-friendly.(ABSTRACT TRUNCATED AT 250 WORDS)
 ...
PMID:[Consensus hemorrhoids (Dutch Society for Surgery)]. 783 Aug 34

Idiopathic neonatal hepatitis is one of the more important causes of neonatal cholestasis. It is regarded one of the clinical presentations of 'idiopathic obstructive cholangiopathy', just like extrahepatic biliary atresia. Is it not possible to discriminate between intrahepatic and extrahepatic causes of neonatal cholestasis, or between idiopathic neonatal hepatitis and metabolic, infectious, or toxic causes, by using clinical or laboratory parameters. Liver histology is slightly more helpful: giant cell formation, focal liver necrosis, and lymphocytic and neutrophilic infiltration may be found in idiopathic neonatal hepatitis. In infectious hepatitis liver pathology mostly is only a lesser part of the symptomatology. Sporadic idiopathic neonatal hepatitis has a better prognosis than familial; about 75% of children with sporadic hepatitis experience complete recovery as compared to less than 25% of children with familial hepatitis. Therapy is confined to the prevention and treatment of complications such as itching, portal hypertension and variceal bleeding, and (fat) malabsorption.
 ...
PMID:[Idiopathic neonatal hepatitis]. 812 25

Schistosoma mansoni schistosomiasis is widespread in Latin America and Black Africa. Infestation is through the skin where it produces erythema with pruritus. Invasion is manifested by mild or sometimes fairly marked systemic disorders. The gastrointestinal disorders observed in the established phase have no characteristic features. The major complication is portal hypertension due to the portal vein fibrosis provoked by schistosomatic granules with an egg at their centre. The diagnosis rests on the finding of eggs in the faeces and on biopsy of the rectal mucosa. The present treatments are effective and well tolerated, but a portocaval anastomosis may be necessary.
 ...
PMID:[Symptoms and development of Schistosoma mansoni infestation]. 834 4

A genetic syndrome causing paucity of interlobular bile ducts (Alagille syndrome) is characterized by five main characteristics: typical, peculiar facies; chronic cholestasis; posterior embryotoxon; butterfly-like vertebral-arch defects; and cardiovascular malformations. In the complete form of the syndrome, all five features are observed. Four or less of these characteristics are present in the incomplete or partial forms of this syndrome. Other, less frequent characteristics (growth retardation, mental retardation, renal and bone abnormalities as well as a high-pitched voice) have also been observed. An autosomal dominant mode of genetic transmission with variable penetrance seems likely. Therapy consists of nutritional supplementation of medium-chain triglycerides, essential fatty acids and fat-soluble vitamins. Liver transplantation has been used successfully to treat patients with liver failure, portal hypertension or severe pruritus and xanthomatosis.
 ...
PMID:Alagille syndrome today. 888 70

Primary biliary cirrhosis (PBC) is likely an autoimmune disease that destroys the interlobular bile ducts. Although the term PBC implies cirrhosis, this is not always present. The condition may be entirely silent clinically, save for the hallmark mitochondrial antibodies in serum. The clinical spectrum of PBC ranges from asymptomatic anicteric cholestasis with or without extrahepatic manifestations to severe cholestasis with decompensated cirrhosis. It is uncertain whether or not the course of this disease is universally fatal. Currently, no specific features have been identified which predict progression from asymptomatic to symptomatic disease, although once hyperbilirubinemia is present, a rising level indicates a poor prognosis. The liver-specific complications include pruritus, abdominal pain, xantholasma, and portal hypertension. The latter is often an early feature, as the portal hypertension is presinusoidal in nature and, when present, does not always reflect the presence of cirrhosis. There are many extrahepatic features of PBC, the most common being metabolic, chiefly hypothyroidism and metabolic bone disease. Other common associations are rheumatologic, renal, pulmonary, neuromuscular, and dermatologic. The non-specific yet distressing symptom of fatigue affects up to two-thirds of PBC subjects, but its etiology remains obscure.
 ...
PMID:The clinical expression of primary biliary cirrhosis. 908 8

The incidence of liver metastasis is quite frequent in patients with advanced cancer. Some patients are asintomatic, but more often a correlation can be present between the clinical observation and the anatomic and functional alteration of the liver provoked by metastasis. Hepatomegaly may cause pain, dyspnea, hiccup. Biliary obstruction generates jaundice and itching. Portal hypertension may cause ascitis, encephalopathy, varices of the esophagus. Hepatic failure may produce symptoms like sopor, dysrasic oedema, coagulation problems, jaundice. The treatment of the symptoms listed above is crucial for the quality of life of these patients, and must be the mainstay of the therapeutic approach. This paper describes the palliative treatment of the clinical complications related to liver metastasis.
 ...
PMID:[Clinical features and symptomatic treatment of liver metastasis in the terminally ill patient]. 921 73

The results of liver transplantation in patients with PSC are excellent and the quality of life is markedly improved. Indeed, liver transplantation is the therapy of choice for patients with end-stage PSC. However, in an age of cost containment, it appears that there are several advantages to offering transplant to patients with PSC a little bit earlier rather than later in the course of their disease. It appears that we can further improve survival, decrease morbidity, decrease blood usage, and avoid the risk of developing a cholangiocarcinoma, which occurs sporadically but not infrequently in the PSC patient. In addition, avoidance of right upper quadrant surgery, such as biliary or shunt surgery, appears to offer several advantages by decreasing resource utilization and possibly decreasing mortality. Although the UNOS selection guidelines recommend transplantation of the sickest patient, there appears to be accumulating evidence that transplantation in patients earlier in the course of their end-stage liver disease may improve survival, decrease morbidity, and also importantly, decrease the cost associated with this expensive procedure. Ideally, we would recommend consideration for liver transplantation all PSC patients who have (1) a Mayo risk score of > 4.8 in whom malignancy is ruled out, (2) cirrhosis and complications of portal hypertension such as variceal bleeding, refractory ascites, or portosystemic encephalopathy, or (3) disabling symptoms such as fatigue, pruritus, or recurrent bacterial cholangitis. We believe that biliary surgery to treat dominant strictures should be avoided and that such strictures should be approached either endoscopically or radiographically, which should include brushings, biopsies, and histology to reasonably exclude the diagnosis of cholangiocarcinoma. Finally, we continue to search for risk factors and for early markers of cholangiocarcinoma so these patients can be identified early and this devastating complication can be avoided by early transplantation.
 ...
PMID:Liver transplantation for primary sclerosing cholangitis: impact of risk factors on outcome. 934 9

Cholestatic liver disease is primarily caused by impaired bile production on the level of hepatocytes and cholangiocytes. Clinically cholestasis can be divided into intrahepatic and extrahepatic forms based on the presence or absence of dilated bile ducts (sonography). Intrahepatic cholestasis is most frequently caused by end stage liver cirrhosis followed by primary cholangiopathies and canalicular transport defects in hepatocytes. The causes of the most important cholangiopathies, such as Primary Biliary Cirrhosis (PBC) and Primary Sclerosing Cholangitis (PSC) are so far not known. Therefore, drug therapy of cholestatic liver disease focuses on the improvement of symptoms such as fatigue, pruritus, abdominal discomfort, jaundice, xanthoma, hypercholesterolemia, portal hypertension, blood count abnormalities, osteoporosis/osteomalacia, and the prevention of complications such as bile-duct strictures in PSC and development of cholangiocarcinoma. The first choice drug in the treatment of cholestatic liver disease of various causes is urosodeoxycholic acid (UDCA), that has been shown to decrease bile acid toxicity in general and prolong the transplant free survival of patients with PBC. If cholestasis persists cirrhosis of the liver is the major complication and liver transplantation may be the definitive treatment in advanced cases of cholestatic liver disease.
 ...
PMID:[Cholestatic liver diseases]. 945 66


<< Previous 1 2 3 4 5 6 Next >>