Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0008370 (cholestasis)
 9,378 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Extrahepatic and intrahepatic biliary obstruction of different etiology were studied in 62 patients, who were investigated for the presence of lipoprotein X (Lp-X). It was found present in 19 of 20 cholestasis by lithiasis, in all three primary biliary cirrhosis patients, in 2 of 4 cirrhosis, in 5 of 13 hepatitis, in all three benign recurrent intrahepatic cholestasis and in 1 of 2 recurrent juandice of pregnancy. It was found in a Dubin Johnson. Lp-X disappeared in 4 patients within two weeks after relief of the obstruction. It was found in patients with cholestatic hepatitis during the first week of jaundice. It was found in the first 48 hours in three patients with cholestasis by lithiasis. Lp-X does not help in differential diagnosis between extrahepatic and intrahepatic biliary obstruction, but the time of its appearance could contribute to it in some cases. A word of caution is raised in indicating surgery in a cholestatic patient without the presence of Lp-X.
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PMID:LP-X in cholestasis. 17 23

A case study of a family in which at least 3 members developed benig n recurrent intrahepatic cholestasis was undertaken. 9 members of the family had developed pruritus and/or jaundice during pregnancy. 2 others develope pruritus while taking oral contraceptives (OCs). 57 cases of benign recurrent intrahepatic cholestasis are noted, many of which occurred as a familial phenomenon. The data suggest a relationship between OC use, pregnancy and intrahepatic cholestasis. Th ere is also evidence for the possible interrelation between the 3 types of intrahepatic cholestasis, though a common origin for these diseases remains to be established.
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PMID:Familial benign recurrent intrahepatic cholestasis. Interrelation with intrahepatic cholestasis of pregnancy and from oral contraceptives? 93 78

Up to now about 100 cases of benign recurrent intrahepatic cholestasis have been reported. In this case we give a description of familial form of this disease, in which cholestasis was associated with inflammatory liver response. A brief review of this rare condition and differential diagnosis have been presented.
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PMID:[Recurrent benign intrahepatic cholestasis syndrome]. 152 40

The clinical usefulness of serum gamma-glutamyl transpeptidase (gamma GT) assay for the diagnosis of liver disease in children was assessed retrospectively in 398 children investigated from 1981 to 1986, in whom diagnosis was ascertained according to currently accepted criteria including liver histology in each case. Serum gamma GT activity was within normal limits in 10 controls, in 19 children with portal vein obstruction, and in 10 of 12 children with congenital hepatic fibrosis. Serum gamma GT was raised in all children with biliary atresia, sclerosing cholangitis, paucity of interlobular bile ducts, and alpha 1-antitrypsin deficiency with jaundice. Serum gamma GT was normal in spite of patent clinical signs of cholestasis in 3 patients with benign recurrent intrahepatic cholestasis, 1 infant with post-hemolytic neonatal cholestasis, and in 22 of 28 patients with progressive idiopathic cholestasis akin to Byler disease. In the latter group, children with raised serum gamma GT displayed extensive portal fibrosis and bile duct proliferation on liver histology, while this was not a prominent feature in children with normal serum gamma GT. These results indicate (a) the value and limits of the assay for serum gamma GT activity in children with liver disease, (b) that raised serum gamma GT may be considered a fairly reliable index of bile duct damage, and (c) that serum gamma GT may prove a useful tool in separating two forms of progressive idiopathic cholestasis, with or without bile duct involvement.
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PMID:Diagnostic value of serum gamma-glutamyl transpeptidase activity in liver diseases in children. 167 10

We present the clinical case of an 8 years old boy affected by episodes of severe recurrent jaundice, preceded by intense itching with clinical and biochemical signs of cholestasis, diagnosed as benign recurrent intrahepatic cholestasis (B.R.I.C.), or Summerskill's syndrome. This was first described by this author in 1959. The syndrome appears as a rare form of cholestatic jaundice of unknown pathogenesis, which in 80% of cases shows up before the age of 20. Its clinical characteristics are episodes of severe jaundice preceded by intense itching with biochemical signs of cholestasis which rise with no apparent cause and which recover spontaneously and are intervalled by asymptomatic periods which last months or years. During this time there is also a regression of the chemical and histological evidence of cholestasis. The diagnosis of B.R.I.C. can be made after having excluded the other congenital or acquired causes of intrahepatic cholestasis according to the recurrent character of the jaundice and to the hepatic biopsy.
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PMID:[Benign recurrent intrahepatic cholestasis. Description of a clinical case]. 189 86

The paucity of the intrahepatic bile ducts, also known as ductopenia, is a well recognized disorder in pediatric patients. Recently, however, a similar disorder has been reported in adults and termed idiopathic adulthood ductopenia (IAD). We describe a 30-year-old patient with a 15 year history of episodes of jaundice. During icteric episodes, serum levels of bilirubin and alkaline phosphatase were markedly elevated. Between attacks, totalling more than 30, the patient was asymptomatic, but bilirubin and alkaline phosphatase levels were mildly elevated. No neonatal jaundice was present in the patient's history. PBC, PSC and drug-induced cholestasis were excluded. Two needle biopsies of the liver, taken within a 13 year interval, were available. The lobular architecture appeared progressively disturbed by porto-centro-portal bridging septa. In both biopsies, a destructive cholangitis was found. In the last biopsy, the majority of the septal and interlobular ducts appeared severely damaged and, in three out of seven portal tracts, the interlobular bile duct had disappeared. In the parenchyma, the main feature was a severe mainly canalicular bilirubinostasis. The patient described illustrates that IAD may have a clinical picture indistinguishable from benign recurrent intrahepatic cholestasis. The etiology of the disease, in this as in other patients, remains unknown.
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PMID:Idiopathic adulthood ductopenia presenting with chronic recurrent cholestasis. A case report. 200 71

Ursodeoxycholic acid was administered to a patient with benign recurrent intrahepatic cholestasis to prevent cholestatic episodes. A detailed study of bile acid metabolism in this patient was carried out in the anicteric and icteric phases before and after ursodeoxycholic acid (750 mg/day) administration. Urinary, biliary and serum bile acids were measured by gas chromatography-mass spectrometry and by high-performance liquid chromatography techniques. During the anicteric phase the daily urinary excretion and serum concentrations of bile acids were within normal ranges, indicating normal hepatic uptake and secretion of bile acids during the cholestasis-free period. Only slight qualitative differences from normal individuals were observed; the relative proportions of deoxycholic acid in the bile and serum were higher, and 12-oxo-lithocholic acid was the predominant urinary bile acid. During the icteric phase a marked increase in the urinary excretion of primary bile acids and C-1, C-2, C-4 and C-6 hydroxylated metabolites was found. Serum bile acid concentrations increased before the rise in bilirubin, suggesting an acute disturbance in bile acid transport at the onset of the cholestatic attack. After ursodeoxycholic acid administration in the anicteric phase, bile became enriched with the exogenous bile acid, but little qualitative change was found in the other metabolites present in the urine, serum or bile during the anicteric or icteric phases. Prolonged administration of ursodeoxycholic acid failed to prevent recurrence of a cholestatic episode, suggesting that in benign recurrent intrahepatic cholestasis, oral ursodeoxycholic acid may be of little benefit in the treatment or prevention of cholestasis despite marked enrichment of the bile acid pool with this hydrophilic bile acid.
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PMID:Failure of ursodeoxycholic acid to prevent a cholestatic episode in a patient with benign recurrent intrahepatic cholestasis: a study of bile acid metabolism. 163 61

S-Adenosylmethionine (SAMe) proved to be effective in antagonizing bile secretion impairment induced by a wide range of hepatotoxins, including ethynylestradiol, taurolithocholate, chlorpromazine and alpha-naphthyl-isothiocyanate. The anticholestatic activity of SAMe may result from its role in the intermediate metabolism as this molecule is involved in transmethylation and transsulfuration reactions. Clinical experience, carried-out on more than 1,000 cholestatic patients, supports preclinical data. In particular, controlled clinical trials have documented that intravenous SAMe (800 mg/day) induced a significant decrease of biochemical parameters of cholestasis (serum total and conjugated bilirubin, serum total bile salts, and aminotransferases), as well as a significant improvement of pruritus in women with ICP compared with placebo. In addition, other studies provided the evidence that both parenteral (800 mg/day) and oral SAMe (1600 mg/day) significantly improves subjective (pruritus, fatigue, and general discomfort) and objective (serum total and conjugated bilirubin, and serum alkaline phosphatase) parameters of cholestasis in patients with intrahepatic cholestasis complicating chronic liver diseases compared with placebo. In all these trials, SAMe treatment has been well tolerated at the same extent as placebo. In conclusion, experimental and clinical investigations indicate that SAMe represents an effective and safe approach to the management of intrahepatic cholestasis.
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PMID:A review of the studies on the clinical use of S-adenosylmethionine (SAMe) for the symptomatic treatment of intrahepatic cholestasis. 217 53

Authors report 6 cases of benign recurrent intrahepatic cholestasis (BRIC), a rare disease of unknown etiology first described 30 years ago by Summerskill and Walshe, and thought to represent a study model for human cholestasis. Clinical, biochemical and pathologic findings of BRIC are briefly summarized in this paper in order to emphasize some triggering factors of the cholestatic attack (e.g., flu-like episodes and pregnancy), the diagnostic problems and the importance to avoid a surgical procedure. Finally, the 'state of the art' of the pathogenesis of BRIC is briefly summarized.
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PMID:Benign recurrent intrahepatic cholestasis. A clinico-pathologic study. 235 3

S-Adenosylmethionine (800 mg i.v. per day) was used to treat two brothers and a brother and sister from each of two kindreds with benign recurrent intrahepatic cholestasis. Symptoms, routine tests of liver function, concentrations of total bile acids, and the oral clearances of [11,12-2H]chenodeoxycholic acid and [24-13C]cholic acid were determined before and after treatment with S-adenosylmethionine. S-Adenosylmethionine did not ameliorate symptoms or biochemical parameters of cholestasis but reduced bile acid clearances in 3 of 4 subjects. Transaminase levels in both subjects of one kindred rose during treatment. These limited, preliminary observations suggest that S-adenosylmethionine may be ineffective in the therapy of benign recurrent intrahepatic cholestasis and may be hepatotoxic in some patients.
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PMID:Benign recurrent intrahepatic cholestasis: treatment with S-adenosylmethionine. 201 88


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