Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0023890 (cirrhosis)
 42,195 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

An 11-year-old girl developed cholangiocellular carcinoma in association with biliary cirrhosis due to congenital biliary atresia. An exploratory laparotomy and an operative cholangiogram at 3 months of age had confirmed the diagnosis of extrahepatic biliary atresia. A liver biopsy specimen taken at 6 months of age showed biliary cirrhosis. The subsequent clinical course was characterized by persistent moderate jaundice, anemia, malnutrition, rickets, pathologic fractures, and recurrent gastrointestinal bleeding. The presence of cholangiocellular carcinoma of the liver with advanced biliary cirrhosis was established at an exploratory laparotomy a week before her death. We discuss here the pathogenesis of biliary cirrhosis and carcinoma of the liver; there may be a relation between the two in the childhood population.
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PMID:Cholangiocarcinoma associated with biliary cirrhosis due to congenital biliary atresia. 19 70

Lipoprotein-X (LP-X) in the serum of infants with persistent jaundice is indicative of cholestasis. In early infancy biliary atresia and biliary agenesis are the most common cause of cholestasis, whereas neonatal hepatitis is a less frequent cause of cholestasis. The authors introduced and described the qualitative and quantitative methods of LP-X determination for diagnostic purposes. LP-X estimations were carried out in 9 children with persistent jaundice. LP-X was found to be present in 4 infants-in 2 with complete absence of extrahepatic biliary tracts, in 1 with extrahepatic biliary atresia and in 1 with hypoplastic extrahepatic biliary tract. LP-X was also found in a 5 year old boy with mechanical occlusion of bile ducts caused by a malignant tumor ( rhabdomyoblastoma ), and in 3 year old girl with inborn enzymatic liver dysfunction. In this case LP-X concentration was estimated before and after 3 week course of cholestyramine, after which there was a 35% decrease in the LP-X concentration. In a 4 month old child LP-X was not found in spite of the absence of extra and intrahepatic biliary tracts. This finding may be explained by the far advanced hepatic cirrhosis. The authors stress the importance of introducing of LP-X estimation in the differential diagnosis of jaundice in early infancy.
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PMID:[Lipoprotein X (LP-X) in the differential diagnosis of cholestasis in children, with special reference to biliary atresia]. 26 31

The portoenterostomy (Kasai) procedure in infants with biliary atresia has dramatically altered the outlook for this heretofore fatal disease. When performed on infants under three months of age, bile drainage can be achieved in a majority of the patients. Since 1972, 37 infants have been treated with this operation at our institution. Diagnostic operative cholangiography and liver biopsy are recommended if the cause of conjugated hyperbilirubinemia is presumed to be obstructive. When biliary atresia is encountered, identification of the atretic ducts with transection high in the porta hepatis is carried out. Thirty-two infants have had the portojejunostomy, while five, in whom the proximal hepatic ducts were atretic but the gall bladder and distal ducts were patent, underwent portocholecystostomy. Examination of the resected fibrous duct tissue revealed a statistically significant correlation between ductal histology and postoperative outcome. Extended bile drainage has been achieved in 26 of 37 patients. Seventeen exhibit near normal growth and development four months to five years postoperatively. Seven have died with progressive liver disease despite bile drainage. Two additional patients died, jaundice free, from unrelated causes. Despite bile drainage, progressive hepatic fibrosis has been confirmed by serial biopsies in 14 patients. This finding indicates that biliary obstruction is not the sole component in the development of biliary cirrhosis. These data suggest that extrahepatic biliary atresia is a dynamic obliterative process, which can be favorably modified in approximately 50% of the infants by early surgical treatment.
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PMID:The portoenterostomy procedure for biliary atresia: a five year experience. 56 61

The prognosis of 97 patients with congenital biliary atresia treated by hepatic portoenterostomy was studied. In 37 of 97 patients (38%), good bile excretion was observed after surgery, and jaundice cleared. Sixteen of the 37 patients developed ascending cholangitis; 11, esophageal varices; and nine died. Seven died of ascending cholangitis and two of hepatic failure. Fourteen patients had an uneventful course. In the remaining 60 patients, bile excretion was scanty, and the disease progressed to biliary cirrhosis; 52 died. Causes of death were hepatic failure (53%), intracranial hemorrhage (16%), rupture of esophageal varices (12%), severe dehydration (9%), and postoperative complications (9%). The age at death ranged from 2 to 52 months, with an average at 16 months.
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PMID:Congenital biliary atresia. Analysis of 97 cases with reference to prognosis after hepatic portoenterostomy. 82 37

Liver specimens from 68 cases of biliary atresia were histologically studied in reference to postoperative prognosis. The histological findings were classified into 4 to 5 groups by the degree of fibrosis, bile duct proliferation, bile plugs, giant cell transformation, and intralobular cholestasis. Furthermore, morphometrical studies of interstitial area, bile duct (ductules), intrahepatic portal vein, and intrahepatic artery were done and correlated with the age of patients and with operative results. From this study, it was shown that 1) marked fibrosis (cirrhosis) and interstitial areas occupying more than 35% of the sectional area of the liver specimen, 2) severe ductular proliferation, shown by proliferated bile ducts occupying more than 20% of interstitial areas, 3) no or slight intralobular cholestasis and 4) marked interlobular cholestasis are considered to predict poor postoperative bile excretion.
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PMID:Histopathological studies on the prognosis of biliary atresia. 91 63

Since 1974, 16 consecutive infants with biliary atresia have been treated by hepatic portoenterostomy employing an exteriorized Roux-en-Y intestinal segment (Miluliez). Simultaneous, sequential analyses of bile pigments and lipids in serum and biliary drainage were performed. In the 11 patients with sustained bile drainage, progressive increases in bile volume, bilirubin and biliary lipid concentrations correlated well with their subsequent return toward normal in the serum. Despite relief of biliary obstruction, four patients have had progressive liver cirrhosis. The other 7 have residual liver damage which has been stable, or in two instances, improved, at late biopsy. The clinical and biochemical results suggest that both obstructive and parenchymal factors are operative in infants with biliary atresia.
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PMID:Biliary lipid excretion after hepatic portoenterostomy. 96 1

Nineteen cases of choledochal cyst are reviewed. Two distinct groups of patients were identified. Patients under one year of age, initially diagnosed as having biliary atresia, had a higher mortality rate, a higher incidence of severe cirrhosis with portal hypertension, and associated atresia or stenosis in the biliary tree. The second group, presenting between 3 and 20 years of age with more classic symptoms, had mild cirrhosis without portal hypertension and had associated choledocholithiasis and pancreatitis. It is suggested that the younger patients had a congenital form of cystic bile duct dilatation and that the older patients had an acquired form, perhaps related to a common channel with reflux of pancreatic juice into the common bile duct. Postoperative follow-up supports the current view that choledochocyst-jejunostomy with choleystectomy has a lower rate of long-term complications than does choledochocyst-duodenostomy.
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PMID:Choledochal cyst: a review of 19 cases. 99 19

The use of animal models in the experimental production of liver diseases similar to those of man is still in its infancy. There is a need to discover new models more closely related to counterpart syndromes in man in the fields of hepatorenal syndrome, neonatal jaundice, Wilson's disease, cholelithiasis, viral hepatitis, biliary atresia, and cirrhosis, to mention only a few. With the continued indiscriminate inbreeding of companion animals as well as the planned inbreeding of laboratory animals, there is little doubt that many more will soon be available. The current availability of mutant rats and sheep with bilirubin transport defects has allowed for a better understanding of how organic anions are transported by the liver. Many other currently available experimental animal models herein briefly reviewed have been only superficially studied. It is the intent of this chapter to provide for post-doctoral students an appreciation for the many animal model systems available for experimental hepatic research.
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PMID:Experimental liver diseases. 110 63

Fourteen patients with "noncorrectable" biliary atresia are living without jaundice for more than 2 yr after hepatic portoenterostomy or its modification. Retardation of physical growth was observed in one of them, and mental retardation in another, both of which seemed irrelevant to biliary atresia. Serial tests for liver function after operation revealed early recovery of serum bilirubin, transminase, and turbidity, and delayed improvement of alkaline phosphatase. Postoperative needle biopsy of the liver disclosed that changes in hepatic parenchyma and ductular proliferation were rapidly improved after successful operation. Improvement of fibrosis of the liver was delayed, and it was not satisfactory in patients whose preoperative changes in the liver were severe or in whom ascending cholangitis had been a frequent complication. Histologic features of hepatic cirrhosis were observed in the liver in three cases, in two of which there had been frequent episodes of cholangitis. Only one of these showed clinical signs of portal hypertension. Functional and morphologic cure can be achieved in "noncorrectable" biliary atresia by hepatic portoenterostomy or its modifications, although varying degree of hepatic fibrosis may remain according to severity of preoperative changes of the liver and postoperative complication of ascending cholangitis.
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PMID:Follow-up studies of long term survivors after hepatic portoenterostomy for "noncorrectible" biliary atresia. 112 98

A series of 12 infants undergoing hepatic portoenterostomy for incorrectable-type biliary atresia is reviewed. There has been no evidence of a sustained postoperative increase in bile excretion or improvement in biliary cirrhosis in any patient. Survival statistics for the group as a whole are poor with a mean postop survival time of 11 months and a mean total survival time of 15.3 months. These survival times are worse than that previously reported for infants with untreated biliary atresia. Our disappointing results with hepatic portoenterostomy raise doubts concerning its value in the treatment of biliary atresia.
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PMID:Hepatic portoenterostomy: an assessment of its value in the treatment of biliary atresia. 113 Aug 77


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