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

Partial diversion of bile flow to an external stoma was performed in 6 patients with chronic intrahepatic cholestasis with severe pruritus that had been refractory to medical measures. Four patients with progressive intrahepatic cholestasis and 2 with arteriohepatic dysplasia were treated. Follow-up has been 3-8 yr. Patients with progressive intrahepatic cholestasis have been free of itching since surgery. Serum bile salt concentrations fell from 218-275 microM (normal less than 10) before to less than 10 microM after surgery. Biochemical tests of liver function and histology returned to normal or near normal. Patients with arteriohepatic dysplasia had persistent mild pruritus after surgery. Serum bile salt concentrations fell from 153-317 to 25-37 microM. There was little or no improvement in biochemical tests or histology. Bile volume and bile salt diverted were higher in patients with progressive intrahepatic cholestasis (7.3-13.0 ml/kg.day and 83-137 mumol/kg.day, respectively) than those with arteriohepatic dysplasia (3.2-4.5 ml/kg.day and 21-36 mumol/kg.day). The quality of life since surgery has been excellent in patients with progressive intrahepatic cholestasis, but not as optimal in those with arteriohepatic dysplasia. These findings suggest that partial external biliary diversion can provide effective relief from pruritus and perhaps reversal of liver disease in patients with progressive intrahepatic cholestasis. It should be used in patients with arteriohepatic dysplasia only in those with disabling pruritus.
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PMID:Partial external diversion of bile for the treatment of intractable pruritus associated with intrahepatic cholestasis. 337 8

The clinical, biochemical, and histological features of 27 children with syndromic paucity of the interlobular bile ducts are described. All presented in the first 5 months of life, 21 with jaundice, two with spontaneous bleeding due to vitamin K malabsorption in addition to jaundice, two with pruritus, and two with failure to thrive. Interlobular bile ducts were abundant in liver biopsies from five (18% of cases) in the first 6 months of life. The degree of portal fibrosis and cellular infiltrate was mild in all except three patients. Clinically significant heart lesions occurred in 52% but only 22% had peripheral pulmonary stenosis. Characteristic facial appearances were present in only 70%; embryotoxon and vertebral anomalies were present in 56 and 33%, respectively. Two infants died of cardiovascular complications, one of alimentary bleeding and one of progressive liver disease. Complications of vitamin K deficiency occurred in 15%, vitamin D deficiency in 30%, and vitamin E deficiency in 37%. Survivors at ages of 19 months to 16.5 years had considerable morbidity with pruritus occurring in 70%, jaundice in 48%, xanthomas in 30%, 74% having hepatomegaly and 63% splenomegaly. All had abnormal biochemical tests of liver function, 90% had growth retardation, and 50% developmental delay. We conclude that differentiation from extrahepatic biliary atresia can be difficult if biliary flow cannot be demonstrated. Prevention of fat-soluble vitamin deficiency is essential. Further research is required to decrease the morbidity associated with this syndrome in infancy.
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PMID:Syndromic paucity of the intrahepatic bile ducts: diagnostic difficulty; severe morbidity throughout early childhood. 368 72

1. Pruritus was assessed in 19 patients by measurement of nocturnal limb movement. 2. Serum (nine pruritic, ten non-pruritic) and interstitial fluid (five pruritic, three non-pruritic) bile acids were fractionated according to their mode of conjugation by using DEAP-Sephadex LH-20 and measured by gas chromatography-mass spectrometry. 3. No correlation was found between serum or interstitial fluid total bile acid or individual bile acid concentrations and pruritus. Bile acid profiles in the two groups of patients were similar and there was no correlation between pruritus and the conjugation pattern. 4. Te bile acid profile of interstitial fluid reflected that of serum and a linear relationship was found between serum and interstitial fluid bile acid concentrations (r ;.95, P less than 0.001). 5. The proportion of bile acid sulphate in interstitial fluid was significantly smaller than that in serum (P less than 0.025), where sulphates accounted for up to 46% of the total bile acids. 6. In three patients, a decrease in serum bile acid concentrations achieved by percutaneous transhepatic biliary drainage had little or no effect on pruritus. 7. These findings suggest that bile acids do not have a causative role in the pruritus of cholestatic liver disease.
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PMID:Bile acid profiles of human serum and skin interstitial fluid and their relationship to pruritus studied by gas chromatography-mass spectrometry. 708 67

Patients with uncontrollable pruritus secondary to cholestatic liver disease were subjected to plasma perfusion in an attempt to remove the toxins responsible for this symptom. The improvement in the degree of pruritus was dramatic and surprisingly long-lasting in five of six patients. One patient had a response more difficult to evaluate. Serum bile acids levels fell in all patients in whom pruritus improved but not in the patient who responded less favorably.
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PMID:Pruritus of cholestasis treated with plasma perfusion. 732 44

A brother and sister who suffered from pruritus since infancy developed hepatic cirrhosis early in life. Although this clinical picture has never been seen in Wilson's disease, Kayser-Fleischer rings in the boy made further studies necessary. Oral radiocopper loading tests administered to both children and to their parents served to exclude Wilson's disease conclusively. Determinations of the concentrations and patterns of bile acids in the serum indicated that the abnormalities observed in these children are not related to errors in bile acid synthesis. Although a defect in bile acid transport is present, it appears to have occurred as a consequence of the liver disease.
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PMID:Familial cholestatic cirrhosis associated with Kayser-Fleischer rings. 736 85

A patient with Sulindac-induced hepatitis is presented. Sulindac was confirmed as the cause of the patient's liver disease when hepatic dysfunction abruptly recurred after an inadvertent reexposure to Sulindac and quickly resolved when the drug was withdrawn. The prompt reappearance of fever, chills, pruritus, rash, tender hepatomegaly, and abnormal liver tests after only two doses of Sulindac suggested a hypersensitivity reaction.
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PMID:Sulindac (Clinoril) hepatitis. 745 Apr 49

Pruritus is a challenging clinical problem which often complicates chronic cholestatic liver disease. For practical purposes, cholestasis may be defined as impaired hepatocellular secretion of bile and is a feature of a wide variety of liver diseases. Cholestasis is usually suspected clinically when a patient presenting with jaundice or pruritus is found to have an elevation in serum alkaline phosphatase activity disproportionate to increases in serum aminotransferase levels. Early imaging by ultrasonography, computerized tomography, or cholangiography is important to address the possibility of remediable biliary tract obstruction. The majority of patients who develop problematic pruritus due to chronic cholestasis will have one of several diseases: primary biliary cirrhosis, primary sclerosing cholangitis, drug-induced cholestasis, autoimmune chronic active hepatitis, or alcoholic liver disease. Specific aetiological diagnosis is usually possible when history and physical examination are complemented, as appropriate, by serological testing, hepatobiliary imaging, and liver biopsy. This review does not address issues in diagnosis, but concentrates upon the management of pruritus, a potentially disabling complication of prolonged cholestasis.
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PMID:Pruritus of chronic cholestasis. 758 73

A locus for progressive familial intrahepatic cholestasis (PFIC), also known as Byler disease, has been mapped to a 19 cM region of chromosome 18 by a search for shared segments, using patients from the Amish kindred in which the disorder was originally described. A similar liver disease, benign recurrent intrahepatic cholestasis (BRIC), recently has been mapped to the same region, suggesting that these two diseases are caused by mutations in the same gene. Although PFIC and BRIC are clinically distinct diseases, episodic attacks of jaundice and pruritus, with elevated concentrations of bile acid in serum, are seen in both disorders. In PFIC patients, these attacks result in progressive liver damage and death. The clinical and biochemical features of PFIC and BRIC are suggestive of a defect in primary bile acid secretion. The biology of bile secretion is of great interest because of its vital importance in digestion of dietary fats as well as in secretion of xenobiotics and metabolic waste products. Cloning of the gene (or genes) responsible for PFIC and BRIC will likely provide important insights into this pathway.
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PMID:Mapping of a locus for progressive familial intrahepatic cholestasis (Byler disease) to 18q21-q22, the benign recurrent intrahepatic cholestasis region. 765 58

The spectrum of liver disease is extremely wide, with many of the underlying disorders having acute and chronic presentations. Most of the underlying pathogenetic mechanisms are accounted for by autoimmune disease, viral infection and toxic insult. The management strategy of any liver disease is a combination of treating the symptoms and complications that arise, as well as drug therapies relevant to the specific underlying diagnosis. Encephalopathy, ascites, spontaneous bacterial peritonitis, variceal bleeding and pruritus are the main complications at which drug therapy is directed, although in some cases it represents only 1 aspect of the overall management. Drug therapy per se is largely ineffective in acute liver failure with the possible exception of acetylcysteine, but many drugs are used in the management of the constituent components of this complex medical emergency. Treatments for specific liver conditions are expanding, especially in the areas of autoimmune and viral disease. The increasing availability and success of liver transplantation has tended to change the emphasis of management, and it is often not appropriate to exhaust the treatment options before referring the patient for transplantation. A comprehensive review of all liver disease is beyond the scope of this article, but hopefully the important principles of management and commonly occurring clinical decisions are discussed.
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PMID:Hepatic disorders. Features and appropriate management. 770 18

Pruritus in cholestatic liver disease can be difficult to treat. It may be related to impaired excretion of large opioid peptides. Buprenorphine, a drug with partial opiate antagonist properties, was used in a double-blind trial of five patients with uncontrollable itching. One patient improved clinically, one patient had good relief from pruritus and three had intolerable side-effects. Buprenorphine may be of limited use in intractable pruritus.
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PMID:Buprenorphine and hepatic pruritus. 784 99


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