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)

We carried out a retrospective investigation of the 89 patients with extrahepatic biliary atresia born in The Netherlands during a 10 year period. Of these 89 patients 10 had a diagnostic laparotomy only. Eight patients had an anastomosis between the proximal bile duct and the intestine, and the remaining 71 had hepatic portoenterostomies. Bile drainage was re-established in 46 (65%). After successful hepatic portoenterostomy the development of cholangitis was the most important determinant of long term survival; five year survival was 54% in the 19 patients who had cholangitis and 91% in the 27 who did not. In the whole group of 71 patients the five year survival was 47%. Seventeen patients were at least 5 years of age at the time of writing, three of whom had had liver transplantation. Three patients have cirrhosis and hyperbilirubinaemia, and the other 11 have normal bilirubin concentrations and normal or slightly raised transaminase activities. To improve these results early surgical intervention in all children with extrahepatic biliary atresia is necessary, as are better methods of prophylaxis and treatment of cholangitis.
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PMID:Prognosis of extrahepatic biliary atresia. 293 Feb 27

The accuracy of ultrasound assessment of portal vein patency has been defined by comparing it with the results of arterial portography in 115 cases. The accuracy of arterial portography was confirmed in 21 cases where orthotopic liver transplantation was performed and used as a 'bench-mark' against which to assess the ultrasound findings. Ultrasound correctly assessed portal vein patency in 87.5% of patients. It was more accurate in assessing patency (90%) than occlusion (68%). Ultrasound correctly assessed portal vein patency in 90% of cases of cirrhosis and hepatic malignancy. Difficulties occurred in children with biliary atresia particularly following the Kasai operation (37.5% accuracy). In the absence of previous surgery to the portal vein or biliary system, ultrasound is comparable to arterial portography and can be used as the sole means of assessment.
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PMID:Assessment of portal vein patency: comparison of arterial portography and ultrasound scanning. 305 95

Pulmonary hypertension is a rare complication of portal hypertension. Reports of childhood cases especially rare. This report describes an autopsy case of a 6-year-old boy with congenital biliary atresia followed by liver cirrhosis in whom severe hypertensive pulmonary arterial changes, including medial hypertrophy, intimal fibrosis and plexiform lesions were demonstrated. Fresh and organizing fibrin-platelet thrombi as well as probable organized thrombi with recanalization were occasionally found in the pulmonary vasculature, but it was thought that they had probably been formed locally as a late complication rather than being of thromboembolic origin. Retrospectively, the chest roentgenograms had revealed abnormalities suggestive of pulmonary hypertension since infancy, but the patient showed no apparent symptoms of it during life. Previously reported childhood cases of pulmonary hypertension associated with portal hypertension were briefly reviewed. Although the mechanism is presently not known, it is suggested that patients with portal hypertension, even in early childhood, are at risk of developing this unusual complication.
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PMID:Pulmonary hypertension associated with portal hypertension in childhood. Case report of a 6-year-old child and review of the literature. 305 10

In this decade liver transplantation has been established as the preferred treatment for children and adults with irreversible end-stage liver disease. Biliary atresia in children and nonalcoholic cirrhosis in adults are the most common indications for the procedure. Transplantation currently plays only a minor role in the treatment of hepatic malignant disease. Blood group compatibility between donor and recipient is preferred, but histocompatibility matching (tissue typing) currently has no significant role in the selection of recipients. Approximately 70% of recipients survive for 1 year, and these patients have an excellent prospect of long-term survival. The emerging evidence indicates that the quality of life and rehabilitation of most liver recipients are good. The current success of liver transplantation can be attributed to critical selection of recipients, modern anesthetic and surgical techniques, improved perioperative care, accurate diagnosis of rejection and superior immunosuppression with cyclosporine.
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PMID:Liver transplantation: current concepts. 328 10

This report reviews the results of some paediatric surgical departments and points out the unsolved problems in biliary atresia disease. The authors conclude that a 5-year survival rate of 60% may be achieved in long-term follow-up, but a complete cure is observed only in 30%. Children who develop a cirrhosis and portal hypertension without or in spite of bile flow can benefit only by liver transplantation. As a result of long-term clinical experience conditions are defined that should be taken in consideration in the surgical treatment of bile duct atresia. In respect of liver transplantation the disadvantages of an external bile draining fistula to prevent cholangitis, an extensive mobilisation of the liver for HPE procedure, and the disadvantages of reoperation are discussed. By avoiding these disadvantages liver transplantation procedure will be facilitated and a 1-2 year survival rate of 80% may be achieved.
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PMID:Liver transplantation for biliary atresia: indications and results. 329 71

We performed a prospective study on 375 patients with liver disease, 60% female, for whom orthotopic liver transplantation (OLT) was considered during 1977-1985. Fifty-four per cent had cirrhosis, 8.5% congenital/hereditary disorders, 25% malignant tumour, 6% benign tumour, 2% Budd-Chiari syndrome, 1.5% acute hepatic failure, 3% other diagnoses, and 10% were under 15 years of age. As of July 1st, 1985, 99 patients (47 chronic active/inactive cirrhosis (CAC/CIC), 28 primary biliary cirrhosis (PBC), five hepatocellular carcinoma (HCC), 19 other diagnoses) were accepted for OLT (median age 40 years, 10% under age 15). By that date, 45 patients (median age 42), had had an OLT (20 CAC/CIC, 15 PBC, three biliary atresia, two HCC, five other diagnoses). Fifty-four per cent (201 patients) were rejected for transplantation. The primary reasons for rejection were: no indication (11%), age (5%), other surgical procedures possible (3%), severe liver failure (14%), extrahepatic spread of liver tumour (11%), cardiovascular or pulmonary problems (2%), severe hepatic bone disease (1%), and miscellaneous (7%). Thirty per cent of the patients with CAC/CIC, 38% with PBC, 88% with HCC and 71% with biliary atresia were rejected. In the CAC/CIC, PBC and biliary atresia patients severe liver failure was the most frequent reason for rejection (62%, 50% and 60%, respectively). In HCC, extrahepatic tumour spread was the most frequent reason (72%) for rejection. In this category only two patients (7%) ultimately underwent liver transplantation.
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PMID:Selection criteria and decisions in 375 patients with liver disease, considered for liver transplantation during 1977-1985. 330 82

Two hundred fifty pediatric (less than 18 years of age) patients underwent orthotopic liver transplantation because of end-stage liver disease and were given combination therapy with cyclosporine and prednisone. The most common indications for transplantation in decreasing order of frequency were biliary atresia, inborn errors of metabolism, and postnecrotic cirrhosis. The 5-year actuarial survival for the entire group was 69.2%. Age and diagnosis did not influence survival. Infections were the most common cause of death, followed by liver failure and cerebrovascular accident. The impact of retransplantation on survival depends on the indication. The survival is better when retransplantation is carried out after rejection than because of technical complications, and the latter has a better survival than does primary graft nonfunction. The difference in survival among these groups is statistically significant. The quality of life for 164 of 173 survivors is good to excellent; only nine children are currently experiencing medical problems. A persistent problem in pediatric transplantation is the scarcity of small donors.
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PMID:Indications for pediatric liver transplantation. 331 78

The extrahepatic biliary tract of a male infant, including both the left and right hepatic ducts, was proven to be patent up until his time of death, however, histologic examination of the liver revealed severe fibrosis of the portal areas with ductular proliferation, scattered bile thrombi, and subsequent biliary cirrhosis. In this case, since the obstructive lesion occurred at the secondary branching of the bilateral hepatic duct and because fewer changes were present in the interlobular bile ducts, it seemed possible to consider that the liver histology revealed findings similar to those of extrahepatic biliary atresia.
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PMID:A case report of intrahepatic biliary atresia showing an unusual liver histology. 339 52

A newly-born male affected by extrahepatic biliary atresia with an alpha-1 antitrypsin pi ZZ deficiency is presented. Parents, Pi MZ, showing no signs of affection either in liver or lungs. His two brothers and one sister died at nine months, 7 years and 22 months respectively. Two brothers showed cirrhosis of the liver, the sister showed extrahepatic biliary atresia, and in all three patients there was an alpha-1 antitrypsin deficiency. Authors want to emphasize the presence of extra-hepatic biliary atresia in both brother and sister who had also an alpha-1 antitrypsin deficiency because this is an infrequent association which requires different treatment.
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PMID:[Alpha 1-antitrypsin deficiency and atresia of the bile ducts]. 349 22

Of 46 liver transplantations performed, 15 patients are alive at follow-up intervals from 2 to 50 months. The most common indications for liver transplantation were biliary atresia, cirrhosis, and neoplasia. About 50% of the patients had pre- and/or postoperative angiograms. Additional material was available from pretransplant workups in patients subsequently shown to be unsuitable for transplantation. Of 13 preoperative angiographic studies, portal vein patency was confirmed in two, inferior vena caval (IVC) patency was confirmed in two, and diffuse neoplasm was identified in three. These patients subsequently had transplants. Conditions precluding liver transplantation in six patients were inadequate portal vein size (less than 5 mm) in two, occluded IVC or portal vein in one each, nonvisualized portal vein in one, and neoplasm localized to one lobe in one. Postoperatively 18 vascular studies identified hepatic arterial compromise in nine, postbiopsy arteriovenous fistula in two, and bleeding from a right adrenal artery in one. Postoperative venography showed thrombosis or occlusion of the IVC in five, portal vein thrombosis in one, and splenic vein thrombosis in one. Of the 18 postoperative angiograms, 12 demonstrated findings considered threatening to the transplants' survival. Six of 18 studies demonstrated findings considered compromising to the transplant. Of the nine postoperative cholangiographic studies, six diagnoses were considered threatening to transplant survival: obstruction of the biliary-enteric anastomosis in four, leakage from the biliary-enteric anastomosis in one, and an abscess from biliary leakage in one.
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PMID:Angiographic and interventional radiologic considerations in liver transplantation. 351 41


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