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)

Seven women, mean age 47.7 years, with primary biliary cirrhosis (6 patients in the II-III stage and I patient in IV stage of the disease) were treated in the course of 16 months with ursodeoxycholic acid (Ursofalk) 500 mg daily. At the end of the 3-d month of treatment the itching had passed in 3 of the patients and in the remaining 4 patients it had substantially decreased. In all patients the subjective complaints, dyspeptic syndrome, appetite and sleep improved. The serum concentrations of bilirubin, copper and cholesterol started to decrease and the serum activity of the enzymes alkaline phosphatase, ALAT and ASAT also decreased. In one patient the treatment was discontinued in the 6-th month because of allergic reaction. After 16 month treatment in the 6 patients who completed the treatment the itching passed and the working capacity improved. The serum concentrations of bilirubin, cholesterol, copper and IgG significantly fell (p less than 0.01), the serum activity of alkaline phosphatase, gamma glutamyl transpeptidase, ALAT and ASAT fell near the upper normal range. The hepatomegaly, splenomegaly, McLagan's flocculation test, serum concentration of IgM and the titer of the specific antimitochondrial antibodies (M2) did not change in spite of the treatment. The results show the ursodeoxycholic acid as a perspective therapeutic means for primary biliary cirrhosis which lowers or overcomes the syndrome of intrahepatic cholestasis and limits the activity of the cirrhotic process in the liver. Ursodeoxycholic acid is well tolerated.
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PMID:[The treatment of primary biliary liver cirrhosis with ursodeoxycholic acid (preliminary report)]. 177 66

Patients with jaundice and hyperbilirubinemia over 34 mumol/l have been examined by different methods in order to assess the diagnostic value of the methods. 340 patients were examined clinically and by laparoscopy, 168 patients and 92 healthy persons were examined by 10 laboratory indices, 639 patients--by ultrasonography, 95 patients--by scintigraphy, 116 patients--by computer tomography, 83 patients--by endoscopic retrograde cholangio-pancreatography (ERCPG), 17 patients--by percutaneous transhepatic cholangiography (PTC), 70 patients--by directed liver biopsy. In the patients with cholestasis the 5'-nucleotidase, alkaline phosphatase, glutamyl transpeptidase (lipoprotein X is positive in 92% of the patients) and cholesterol are increased most. The extrahepatic obstructions are diagnosed by ultrasonography in 94.8% of the patients (the biliary ducts are dilated), in 88.7% of the patients the localization of the obstruction and in 74.7% of the patients the cause of the obstruction are found. In parenchymal jaundice the sonography reveals the disease which has caused jaundice in 62.1% of the patients. The scintigraphy gives correct diagnosis in 50% of the patients with hepatitis and jaundice, in 78% of the patients with cirrhosis and jaundice and in 87.5% of the patients with liver cancer. The computer tomography reveals the obstructive jaundice in 94.7% of the patients and the focal processes in the liver in 96.7% of the patients. The ERCPG gives a clear picture of the biliary ducts in 72.28% and of the pancreatic duct in 83.13% of the patients with jaundice, simultaneously the biliary and the pancreatic ducts--in 45.78% of the patients and correct diagnosis in 83.1% of the patients examined.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:[Differential diagnosis of jaundice]. 343 27

The deliberate ingestion of carbon tetrachloride by a 48-year-old woman provided an opportunity to study the sequential biochemical changes of a severe but self-limited event of hepatocellular damage. The initial phase of cellular injury characterised by high levels of serum aspartate aminotransferase, ferritin and bile acids was followed by a period of partial cholestasis. This was indicated by a marked decrease in secondary bile acids and small rises in alkaline phosphatase and 5' nucleotidase. Improving liver function and regeneration became evident during this period and was associated with abnormal levels of gamma glutamyl transpeptidase. A late rise in serum ferritin and alphafetoprotein may represent a non-specific inflammatory reaction.
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PMID:Serum bile acids and other liver function tests in hepatocellular damage from carbon tetrachloride ingestion. 615 37

Primary sclerosing cholangitis (PSC) is a cholestatic liver disease characterized by inflammation and obliterative fibrosis of bile ducts leading to their progressive destruction (1-4). As a consequence, cholestasis with elevated serum levels of alkaline phosphatase (AP) and gamme glutamyl transpeptidase (GGT) and bilirubin in more advanced disease is the most prominent feature of this disease. The diagnosis of PSC is primarily based on endoscopic retrograde cholangiography with demonstration of irregular strictures and dilatations. In liver biopsy, typical findings are portal and periodical inflammation and fibrosis. Since PSC is a focal disease, the characteristic histological findings may or may not be seen in a single liver biopsy. The cause of PSC is still unknown. The association with histocombatibility antigens indicates that immunological mechanisms may be involved but it is still unclear whether the disease is immunogenic. Alternatively, bacteria and bacterial toxins from the colon might play a role (3, 4). In 70% of cases PSC is associated with ulcerative colitis (5) and, therefore, in all patients with this intestinal disease who also have elevated levels of liver enzymes, a cholangiography should be performed. Recently, in up to 80% of patients with PSC anti-neutrophil-cytoplasmatic-antigens (ANCA) were found to be elevated (6) and, in future, this test may help to diagnose the disease more easily. Up to now, however, the disease is usually diagnosed at a relatively advanced stage when the patients have jaundice.
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PMID:Ursodeoxycholic acid in the treatment of primary sclerosing cholangitis. 782 95

Severe hepatitis C virus (HCV)-related fibrosing cholestatic hepatitis leading to early liver failure has been reported only exceptionally. Of 259 HCV-infected renal transplant (RT) patients in one hospital unit, four (1.5%) are described, representing the first series of this particular post-RT disease. Patient mean age was 55.7 yr. Three were men. All had pretransplant, hepatitis B surface antigen-negative and were anti-HCV antibodies positive. Three of them showed pretransplant mild liver enzyme abnormalities, and all received kidneys from HCV-negative donors. All were on steroids, cyclosporine, and azathioprine (AZA). The clinical pattern appeared early after RT (mean, 11.5 mo). In three patients, hyperbilirubinemia (6.5 to 20 mg/dl) and high alkaline phosphatase levels (428 to 859 IU/L) were observed. Also, in all subjects, high gamma glutamyl transpeptidase levels (639 to 4270 IU/L), mild aspartate aminotransferase and alanine aminotransferase abnormalities, and serum HCV RNA were observed. Liver biopsy revealed diffuse fibrosis, leukocyte infiltrates, and different degrees of cholestasis, with typical signs of HCV hepatitis in only one patient. Two patients developed subfulminant liver failure and died 2 and 3 mo after biopsy, respectively. One patient also suffered hepatic failure, receiving a liver transplant. The fourth is alive on dialysis awaiting a combined kidney and liver transplant. It is concluded that fibrosing cholestatic hepatitis is a new, early, and severe complication after RT in HCV(+) patients, which appears in patients with ongoing HCV infection under AZA therapy, despite a nonaggressive immunosuppressive protocol. Both HCV and AZA could play a concurrent role in the pathogenesis of this severe complication after RT.
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PMID:Fibrosing cholestatic hepatitis in hepatitis C virus-infected renal transplant recipients. 962 Dec 97

Seventeen patients with established fasciolosis and ten normal controls were enrolled in the study. The Fasciola patients were divided according to infection intensity into two groups (four patients with high intensity and thirteen patients with low intensity) as assessed by egg counts coupled with ultrasonography for detection of worms in the biliary system. Aspartate and alanine aminotransferases (AST and ALT) levels were similar to those of the controls, within the accepted normal limits, before and after treatment denoting absence of hepatocellular injury. Total serum bile acids, individual bile acids: cholic acid (CA) and chenodeoxycholic acid (CDCA), gamma glutamyl transpeptidase (GGT) and serum alkaline phosphatase (SAP) were significantly higher among all patients as compared to the controls denoting a degree of cholestatic lesion in those patients. Patients with high infection intensity revealed higher parameters than those with low intensity. The difference was not significant. One month after treatment, there was a significant improvement in the cholestasis indicating parameters in all Fasciola cases compared to the pretreatment ones. This indicates the effective role of the drug on the hepatobiliary function. However, the levels were still different from the controls. In Fasciola infection, total and individual serum bile acids in conjunction with GGT and SAP evaluate the hepatobiliary status and detect any minor abnormalities especially in anicteric subjects. Studied after treatment, they can be useful indices for assessment of the improvement.
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PMID:Human fasciolosis: a study on the relation of infection intensity and treatment to hepatobiliary affection. 1060 89

ARC syndrome (OMIM 208085) is an autosomal recessive multisystem disorder characterized by neurogenic arthrogryposis multiplex congenita, renal tubular dysfunction and neonatal cholestasis with bile duct hypoplasia and low gamma glutamyl transpeptidase (gGT) activity. Platelet dysfunction is common. Affected infants do not thrive and usually die in the first year of life. To elucidate the molecular basis of ARC, we mapped the disease to a 7-cM interval on 15q26.1 and then identified germline mutations in the gene VPS33B in 14 kindreds with ARC. VPS33B encodes a homolog of the class C yeast vacuolar protein sorting gene, Vps33, that contains a Sec1-like domain important in the regulation of vesicle-to-target SNARE complex formation and subsequent membrane fusion.
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PMID:Mutations in VPS33B, encoding a regulator of SNARE-dependent membrane fusion, cause arthrogryposis-renal dysfunction-cholestasis (ARC) syndrome. 1505 68

We describe cholestasis as a result of bile duct abnormalities in 8 children with portal vein obstruction. In a clinical, biochemical and radiological investigation of 121 children with cavernous transformation of the portal vein seen between 1986 and 2000, 8 presented with jaundice, pruritus, and/or raised serum aminotransferases and/or gamma glutamyl transpeptidase (gamma GT) activities. Each displayed dilation and narrowing of intra- and/or extrahepatic bile ducts. Surgical decompression of the portal system (portal-systemic or Rex anastomosis) resulted in the regression of the signs of cholestasis in all children. We conclude that children with portal vein obstruction may exhibit clinically significant cholestasis as a result of external compression of the bile duct by the cavernoma.
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PMID:Cholestasis in children with portal vein obstruction. 1581 69

A 38-year-old Ghanaian man presented with a 6-month history of worsening pruritus, jaundice, and ascites. He was previously fit and well and rarely drank alcohol. Screening tests for chronic liver disease including viral, autoimmune, and other metabolic causes including iron overload were unremarkable. A liver biopsy performed at the referring hospital demonstrated intralobular cholestasis and cirrhosis. He was listed for liver transplantation but subsequently developed sepsis with multiple organ failure and died. The sickle solubility test was positive. Blood smear showed cells consistent with liver failure and no sickle cells. Hemoglobin electrophoresis revealed HbA2 2.8%, HbF 0.5%, and HbS greater than HbA (49.6% vs. 41.3%) in the absence of blood transfusion. Sequence analysis of the beta-globin genes showed he was a compound heterozygote for the Hbs mutation at codon 6 (CAG --> GTG) and a novel mutation at position 844 of intron 2 (betaIVS2-844 C --> A). A diagnosis of sickle hepatopathy causing decompensated cirrhosis was made. This case is unusual insomuch as this patient was asymptomatic for over 35 years and represents a novel presentation of sickle cell disease. Sickle cell disease should be considered in appropriate patients when unusual presentations of liver disease arise.
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PMID:Sickle liver disease--an unusual presentation in a compound heterozygote for HbS and a novel beta-thalassemia mutation. 1756 24

Biliary strictures (BS), a major complication after orthotopic liver transplantation (OLT), cause morbidity, mortality, graft loss, and increased costs. The virtually unchanged incidence of BS (approximately 10%-25%) suggests that they are not simply "technical" in origin, but probably represent a mucosa ischemic injury inherent in the transplantation procedure. To study risk factors for BS, we analyzed 403 OLTs performed between January 1, 1997 and December 31, 2006, at a single center, excluding cases of regraft or death within 1 month. The average time to the diagnosis of the BS was 253 days (range, 7-1002 days). Upon univariate analysis, the absence of flushing of donor bile ducts, an imported versus a locally procured liver, and rejection were risk factors for BS. In contrast, the following factors were protective: donor cardiac arrest followed by resuscitation (suggesting an ischemic preconditioning effect) as well as addition of epoprostenol to and pressurization of the preservation solution. Patients with higher postoperative peak values of transaminases, bilirubin, alkaline phosphatase, and gamma glutamyl transpeptidase were at greater risk for later development of BS. Donor hypotension, donor age, donor intensive care unit (ICU) stay, type of preservation, positive cross-match, cold and warm ischemia times, sequential versus simultaneous portal/arterial reperfusion, as well as cytomegalovirus (CMV) infection were not risk factors for BS. Upon multivariate analysis, only epoprostenol and pressurization offered protection from BS. In conclusion, this study 2 novel points: (1) patients with high(er) transaminase values and cholestasis early postoperatively are at greater risk to develop later BS and require close monitoring and (2) donor maneuvers for better flushing and preserving peribiliary vascular plexus and biliary mucosa (epoprostenol and pressurization of preservation solution) offer protection from BS.
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PMID:Biliary strictures after liver transplantation: risk factors and prevention by donor treatment with epoprostenol. 1985 57


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