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Query: UMLS:C0239946 (liver fibrosis)
 8,268 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

We present the case of a 56 year old woman with Caroli's disease associated to congenital liver fibrosis, renal nephrocalcinosis and cutaneous vasculitis of the legs. Clinical signs of portal hypertension were treated by a shunt technique. After an asymptomatic period, the patient suffers now from crisis of angiocholitis.
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PMID:[Caroli's syndrome: a variable entity]. 784 99

We report the case of a two-year-old girl presenting with recurrent episodes of fever and hepatomegaly. High resolution ultrasound (US) and computed tomography (CT) of the liver show dilated intrahepatic bile ducts with bridge formation and intraluminal protrusions. The extrahepatic bile duct is normal. The diagnosis of irregular dilated bile ducts with signs of cholangitis and cholangiolitis is proved by liver biopsy. The histologically associated congenital liver fibrosis in our patient enter into the Caroli's syndrome. We illustrate the high resolution US and the CT findings in this case.
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PMID:Caroli's syndrome. 956 64

The polycystic kidney (PCK) rat represents a liver and kidney cyst pathology corresponding to Caroli's disease with congenital hepatic fibrosis and autosomal recessive polycystic kidney disease. We previously reported that an epidermal growth factor receptor tyrosine kinase inhibitor, gefitinib (Iressa), significantly inhibited the abnormal growth of biliary epithelial cells of PCK rats in vitro. This study investigated the effects of gefitinib on cyst pathogenesis of the PCK rat both in vitro and in vivo. A three-dimensional culture model of biliary epithelial cells in the collagen gel matrix was used for in vitro analysis. For in vivo experiments, PCK and control rats were treated with gefitinib between 3 and 10 weeks of age. In vitro, gefitinib had strong inhibitory effects on biliary cyst formation of PCK rats. In vivo, treatment with gefitinib significantly inhibited the cystic dilatation of the intrahepatic bile ducts of PCK rats, which was accompanied by improvement of liver fibrosis. By contrast, no beneficial effects were observed on renal cyst development because of the treatment. These results suggest that signaling pathways mediated by epidermal growth factor receptor are involved in biliary dysgenesis of the PCK rat, with the mechanisms of cyst progression being different between the liver and kidney.
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PMID:Inhibition of intrahepatic bile duct dilation of the polycystic kidney rat with a novel tyrosine kinase inhibitor gefitinib. 1700 82

Hepatopulmonary Syndrome (HPS) is a potential complication of chronic liver disease and is more commonly seen in the adult population. Caroli Syndrome is a rare inherited disorder characterized by intrahepatic ductal dilation and liver fibrosis that leads to portal hypertension. In children with liver disease, HPS should be considered in the differential diagnosis of prolonged, otherwise unexplained, hypoxemia. The presence of HPS can improve patient priority on the liver transplantation wait list, despite their Pediatric End-Stage Liver Disease (PELD) score. We present a 6-year-old girl with Caroli Syndrome and End-Stage Renal Disease who presented with persistent hypoxemia. The goal of this report is to increase awareness of HPS in children.
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PMID:Severe Hepatopulmonary Syndrome in a Child with Caroli Syndrome. 2888 36