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Query: UMLS:C0033774 (pruritus)
 14,546 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

There have been a few reports of infants with severe neonatal cholestasis related to a defect in primary bile acid synthesis. To assess the importance of such deficiency among children with progressive intrahepatic cholestasis (Byler disease), screening for inborn errors in bile acid synthesis was performed by fast atom bombardment ionization-mass spectrometry of urine samples from 30 affected children. Bile acid analysis revealed a specific fast atom bombardment ionization-mass spectrometry profile for 3 beta-hydroxy-C27 steroid dehydrogenase/isomerase deficiency in five children who had jaundice, hepatosplenomegaly, and fatty stools beginning at ages ranging from 4 to 46 months. None of them had pruritus. Liver function tests showed persistently normal serum gamma-glutamyltransferase activity, low serum cholesterol and vitamin E levels, normal serum bile acid concentrations despite raised serum bilirubin levels, and decreased prothrombin time and clotting factor V. In four of the cases a similar disease was observed in siblings. Liver function returned to normal after oral ursodeoxycholic acid therapy. We conclude that 3 beta-hydroxy-C27-steroid dehydrogenase/isomerase deficiency should be considered when idiopathic cholestatic liver disease with clinical features akin to Byler disease is characterized by the association of normal serum gamma-glutamyltransferase activity, normal serum bile acid concentration, absence of pruritus, and a return to normal liver function during ursodeoxycholic acid therapy. Early identification of these children is essential because they benefit from bile acid therapy and might thus avoid the need for liver transplantation.
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PMID:A new cause of progressive intrahepatic cholestasis: 3 beta-hydroxy-C27-steroid dehydrogenase/isomerase deficiency. 791 5

Acute self-limited liver disease has been associated with tetracycline use. However, severe prolonged cholestatic hepatitis and bile duct paucity have not been previously attributed to tetracyclines. Hepatitis, characterized by prolonged jaundice, severe pruritus, and moderate increased transaminase values, occurred within 2 months of ingesting tetracyclines in two female patients. Serum bilirubin levels normalized 12 and 34 months after tetracycline ingestion. Liver histology revealed bile duct paucity, severe cholestasis, and minimal necrosis and inflammation. Tetracyclines may infrequently induce bile duct paucity and prolonged, severe, and reversible cholestasis.
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PMID:Tetracycline-induced bile duct paucity and prolonged cholestasis. 795

Pruritus in hepatic cholestasis has been suggested to be secondary to a high concentration of serum bile acids. Rifampicin, which inhibits the uptake of bile acids by hepatocytes, has been used to treat pruritus. To determine the efficacy of rifampicin as a treatment for refractory pruritus, the medical records of 33 children (median age 25 months, range 4-135; 19 boys) with chronic cholestasis liver disease (21 with Alagille's syndrome, eight with progressive intrahepatic cholestasis, one with extrahepatic biliary atresia, one with an inborn error of bile acid metabolism, and one with cryptogenic cirrhosis) were reviewed retrospectively. The median dose of rifampicin was 5(4-10) mg/kg/day. The median duration of intake was 36(4-120) weeks. Complete relief of pruritus was noted in five (15%) patients and a partial response in 12 (36%). Overall, no significant difference was noted in the laboratory parameters before and after treatment with rifampicin. In the 21 patients with Alagille's syndrome, however, a significant decrease in alkaline phosphatase was seen before and after one and six months of starting treatment. No adverse side effects were seen. Rifampicin appears to be effective in the treatment of refractory pruritus. A prospective study is warranted to assess further the effect of rifampicin treatment in children with hepatic cholestasis.
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PMID:Effect of rifampicin in the treatment of pruritus in hepatic cholestasis. 802 98

Serum levels of total alkaline phosphatase increase during pregnancy but the presently available methods are not very precise. We used the CHEM 1 Bayer Diagnostic test in all the consecutive patients admitted to our unit between June 1 and October 16, 1990. Exclusion criteria were twin pregnancies, pruritus, liver disease or parasitosis. A multifactorial analysis was used to discriminate between general, obstetric, pathologic and drug parameters. There were 373 serum samples, 91% were obtained during the last three months of pregnancy. The mean serum alkaline phosphatase level was 68.2 U/l and 75.9 U/l during the first two trimesters respectively and rose to 126.7, 178.8 and 234 U/l during the last three months respectively. The elevation was greater in cases of vomiting during the first trimester, alpha-methyldopa intake. It was less after hypodroxyapatite intake and was positively correlated with weight gain. Age, parity, pregestational weight, neonatal weight, other pathologies and other drug intake did not affect the results. Knowledge of these physiological levels will be useful for evaluating most liver diseases occurring during pregnancy. Assay of the enzyme fractions would be ordered secondarily.
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PMID:[Serum alkaline phosphatase levels in pregnancy]. 804 May 75

Primary biliary cirrhosis (PBC) is a chronic cholestatic liver disease characterized by inflammatory destruction of median size intrahepatic bile ducts. The characteristic histological process is described as chronic nonsuppurative destractive cholangitis (CNSDC). Our knowledge for the pathogenesis of PBC remains incomplete. However, immunological mechanisms seems to play one of the most important role. The immunohistochemical examination represents accumulation of stimmulated T lymphocytes in the portal area. Attachment of CD8 positive T cells to bile duct epithelial cells is observed. The animal model of PBC indicates autoreactive CD4 positive T cells seems to be important at the early stage of PBC and CD8 positive cytotoxic T cells are essential for the progression of the disease. PBC is histologically classified into four overlapping stages by Scheuer. Clinically, PBC is classified into asymptomatic PBC (aPBC), PBC with itching alone (s1PBC) and with jaundice (s2PBC).
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PMID:[Primary biliary cirrhosis (PBC): concept, pathogenesis and classification]. 811

Pruritus is a severe and troublesome symptom in patients with cholestasis and is often difficult to treat. Propofol was recently shown to be efficient in the treatment of pruritus secondary to spinal morphine administration. In a prospective, randomized, double-blind, cross-over and placebo controlled study, 20 patients received 1 dose of propofol (15 mg) and 1 dose of Intralipid (1.5 mg) during a 2-day study period. Pruritus was assessed by a visual analogue scale from 0 (no pruritus) to 10 (most severe pruritus imaginable). Treatment success was defined as a decrease in pruritus of at least 4 points on the scale in 80% of the patients receiving propofol and in 15% of those receiving intralipid (p < 0.05). Discomfort on injection was observed in 15% under propofol treatment. In conclusion this study shows that subhypnotic doses of propofol are effective for the short-term symptomatic relief of pruritus associated with liver disease. At the dose administered, side effects were rare and minor.
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PMID:[Pruritus associated with liver disease: propofol, a new therapeutic approach?]. 819 Dec 69

Pruritus in patients with liver disease is often uncontrollable; however, washing daily with aqueous cream instead of soap has been found to significantly reduce the degree of itching. It also causes no side effects.
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PMID:The use of aqueous cream to relieve pruritus in patients with liver disease. 832 50

One hundred and one patients were included in a double-blind controlled trial to determine whether malotilate (diisopropyl 1,3-dithiol-2-ylidene malonate) is therapeutically effective in primary biliary cirrhosis. Fifty-two patients received malotilate (500 mg three times a day) and 49 patients placebo. The mean follow-up time was 28 months (range 6-46 months). The large majority of patients did not have advanced liver disease since only ten patients were in Child-Pugh class B and none in class C, and the median bilirubin and albumin at entry were normal. Malotilate had no clear effect on pruritus. In malotilate recipients the following statistically significant biochemical changes occurred: alkaline phosphatase decreased 21%, AST 20%, ALT 40%, IgA 12% and IgM 26%. In the placebo group no significant changes occurred. Evaluation of entry and 2-year liver biopsies indicated that malotilate diminished plasma cell and lymphocytic infiltrate and piece-meal necrosis, but had no effect on liver fibrosis. There was no difference in survival or in disease progression according to Child-Pugh criteria. In six patients receiving malotilate, but in none on placebo, treatment was discontinued due to suspected side effects. All patients recovered completely. We conclude that malotilate has an immune-modulating, anti-inflammatory but not anti-fibrotic effect in primary biliary cirrhosis. The clinical relevance of the observed benefits, however, appears too slight to recommend malotilate as single drug therapy in primary biliary cirrhosis.
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PMID:The results of a randomized double blind controlled trial evaluating malotilate in primary biliary cirrhosis. A European multicentre study group. 844 37

The pruritus of cholestasis is a difficult clinical problem to manage. The pathogenesis of this symptom is unknown. All conventional therapies have been unsuccessful in isolating a particular group of substances that could be implicated as direct or indirect causative agents. It should be emphasized that nonspecific treatment modalities that lower the plasma concentrations of a variety of substances (such as cholestyramine, colestipol, charcoal hemoperfusion, plasmapheresis, partial external diversion of bile, and drugs that induce hepatic enzymes), can potentially ameliorate the pruritus of cholestasis by lowering the circulating levels of an undefined pruritogen or a factor responsible for inducing the primary mechanism of the pruritus. The encouraging results reported by the use of antibiotic therapy for this condition merits investigation. Increased opioidergic neurotransmission is part of the syndrome of cholestasis and it contributes, at least in part, to the pruritus associated with it. Opiate antagonists seem to be an effective alternative therapy for some patients. Recent preliminary reports suggest that other neurotransmitter systems, eg, serotoninergic, may be involved in the pruritus of cholestasis. The need for quantitation of scratching activity cannot be over emphasized. The use of quantitative methodology offers the opportunity to facilitate the study of scratching behavior that is a biological phenomenon secondary to liver disease.
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PMID:The pruritus of cholestasis. 867 36

The authors describe a 62 year-old white male who was diagnosed as autoimmune hyperthyroidism and treated with methimazole and atenolol. Ten days later he showed itching, jaundice and choluria. All drugs were discontinued. The patient was given radioactive iodine. Two months later direct serum bilirubin levels reached 35 mg%. Endoscopic retrograde cholangiogram evidenced normal extrahepatic biliary ducts. The percutaneous liver biopsy showed marked cholestasis specially in the centrolobular zone with a slight infiltrate of mononuclear cells in the portal areas. Together with the liver disease the patient presented an anemic syndrome. Bone marrow aspiration showed rich cellularity, Perls staining showed 70% sideroblasts, with 10% ringed sideroblasts and increased extracorpuscular iron. The patient's evolution was satisfactory. Twenty months after the beginning of the disease clinical and biochemical tests were normal. A new bone marrow aspiration rendered normal. Hepatic cholestasis suffered by our patient was probably due to an adverse reaction of methimazole. Physiopathology of reversible sideroblastic anemia is discussed.
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PMID:[Acquired sideroblastic anemia and cholestasis in a hyperthyroid patient treated with methimazole and atenolol]. 873 82


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