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)

To assess whether hepatic peptidyl prolyl hydroxylase (PPH) activity could serve as a practical quantitative indicator of hepatic fibrosis or aid in the categorization, diagnosis or prognosis of hepatobiliary disorders in infancy and childhood, the activity of this enzyme has been determined prospectively by a tritium release method in 97 biopsies from 94 infants and children with the following conditions: acute hepatitis of infancy, 10 patients; extrahepatic biliary atresia, 13; previous hepatitis of infancy, 8; alpha-1-antitrypsin deficiency, 6; chronic active hepatitis, 17; chronic persistent hepatitis, 5; glycogen storage disease, 5; and 25 patients with a miscellanea of other liver disorders. PPH activity was considered in relation to diagnosis, biochemical and histological abnormality and subsequent prognosis over a 4-year period. Five liver biopsies which showed no histological abnormality were considered as "controls" having PPH values of 0.72 +/- 0.47 (mean +/- S.D.). PPH activity was significantly elevated in acute hepatitis of infancy, 9 of the 10 infants having PPH greater than 1.66 units (i.e., mean +/- 2 S.D. of the "control" value). Nine infants (70%) with extrahepatic biliary atresia also had PPH activity above this value, as did two with alpha-1-antitrypsin deficiency and 12 patients all in different diagnostic categories. PPH activity did not correlate with hepatic fibrosis as indicated by hepatic hydroxyproline concentration or by histological assessment, or with biochemical tests of liver function within any diagnostic group or in the series as a whole. PPH activity was similar in biopsies with and without histological features of cirrhosis.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Hepatic peptidyl prolyl hydroxylase activity and liver fibrosis--a prospective study of 94 infants and children with hepatobiliary disorders. 632 86

Liver transplantation in children is still rarely performed although the prognosis for children with benign and final-stage liver disease and individual cases with unresectable hepatomas is acceptable. This report is based on the cases of eight children who underwent liver transplantation, in five cases for end-stage cirrhosis and in three cases for hepatoma. There was early mortality in only one case while in all the others development and rehabilitation were remarkable. For patients with malignancies, tumour recurrence is a limiting factor. Transplantation in biliary atresia is complicated when carried out at late stages of the disease. In cases where the time of indication is sufficiently early, a long waiting period for a suitable donor organ must be expected, during which several potential candidates may succumb to the underlying disease. Increasing experience and improved immunosuppressive therapy signify that liver transplantation can be considered more often in end-stage liver diseases.
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PMID:Liver transplantation in children. 635 86

The results of liver transplantation in a total of 540 patients from four centers in the United States and Western Europe have been collated. Twenty-five per cent of all transplants were performed for neoplastic disease. One- and 3-year survivals for this group were approximately 26 and 12% overall, and survival differed little for patients transplanted before and after January 1, 1980. Among the 44% of patients transplanted for endstage cirrhosis, 3-year survival was lowest for patients with alcoholic cirrhosis (20%). Three-year survival was greater for patients with nonalcoholic cirrhosis (29% overall), did not differ markedly among the various subtypes, and was greater for patients transplanted after January 1, 1980 (42%), as compared with before (22%). Patients with biliary atresia, sclerosing cholangitis, and metabolic and miscellaneous disorders constituted the remaining 30% of patients; 3-year survival varied from about 20 to 44% overall for the various subgroups, with a consistent trend toward improved survival among patients transplanted after January 1, 1980. The use of cyclosporin may not wholly explain this improved survival among more recently transplanted patients. Quality of life for transplant recipients surviving at least 3 months, as judged by a limited amount of data regarding time-in-hospital and functional status, appears to be good.
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PMID:Human liver transplantation: analysis of data on 540 patients from four centers. 636 66

More and more refined laboratory investigations for differential diagnosis of neonatal direct hyperbilirubinemia have been developed, but not one of them is absolutely diagnostic. All of them take time and by the time the infant is referred to the surgeon it is often too late, as the prognosis for successful surgical correction becomes rapidly worse with increasing age. Most authorities have now agreed that in cases of extrahepatic biliary atresia cirrhosis has already well developed after the second month of life. The only virtually certain methods of diagnosis are direct inspection of the gall bladder, cholangiogram, and a sizeable liver biopsy. Our clinical experience has convinced us that laparoscopy with liver biospy and simultaneous cholangiography by transhepatic puncture of the gall bladder is the simplest, fastest and most certain method for diagnosis. It allows for early differentiation of direct hyperbilirubinemia in the neonatal age and should be carried out if the hyperbilirubinemia has persisted during the first month of life and before laboratory investigations are instituted.
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PMID:Early differential diagnosis between neonatal hepatitis and biliary atresia. 644 3

At least four bilirubin fractions can be separated and identified by liquid chromatography, the least understood being the "delta" fraction (B delta), which apparently is covalently bound to albumin. To learn more about the incidence and significance of B delta, we assayed serum from 539 infants and children, both by the chromatographic method and the routine colorimetric Jendrassik-Grof method. The proportion of B delta appeared to correlate with both age and disease course. For infants younger than 28 days B delta generally was less than 2% of total bilirubin; for hyperbilirubinemic older infants and children the median B delta value was 35%. High B delta (greater than 50% of total bilirubin) in newborns was associated with intra- and extra-hepatic cholestasis, biliary cirrhosis, biliary atresia, and hepatitis. Among older infants and children, a proportion of low B delta (less than 10%) was found in hemolytic anemias, sepsis, shock, and other non-hepatic jaundice. In several cases, when low B delta was accompanied by increased conjugated bilirubin, the prognosis was very poor. Delayed clearance of B delta from the circulation together with its reactivity in direct diazo methods may interfere with interpretation of values for conjugated bilirubin as measured by classical methods.
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PMID:Delta bilirubin in serum of pediatric patients: correlations with age and disease. 646 72

Prior to the Kasai procedure of hepatic porto-enterostomy in 1959, biliary atresia was a bleak chapter in paediatric surgery. It was only after many years, however, that the procedure became widely accepted. During the past 8 years 23 cases of biliary atresia have been treated at the Royal Alexandra Hospital for Children (RAHC). Twenty-one had the noncorrectable type of atresia. Sustained drainage was obtained in ten patients, all of whom had the noncorrectable type anomaly. Nine are still alive, eight having survived more than one year after operation and all are free from jaundice. Cholangitis has been a problem in six of these patients and six have evidence of fibrosis or cirrhosis on subsequent liver biopsy. Two patients have shown improvement in liver histology since operation. Sustained biliary drainage is related to age at operation, the size of biliary ductules at the porta and the subsequent development of cholangitis. Long term prognosis still remains uncertain.
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PMID:Biliary atresia: review of experience with twenty-three patients. 657 57

Life-threatening ascites developed in a 4-month-old infant with biliary atresia 1 month after hepatic portoenterostomy. Although initially ascribed to progressive liver failure, the ascites was subsequently found to be partially caused by portal vein thrombosis. Documented recanalization of the portal venous system was accompanied by resolution of the ascites. Refractory ascites in patients with biliary atresia generally signals end-stage cirrhosis and consideration of liver transplantation. Portal vein patency should be evaluated before referral of such patients for this procedure.
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PMID:Portal vein thrombosis following hepatic portoenterostomy. 664 51

A female child, 5 years and 5 months of age, who had had longstanding obstructive jaundice with hepatic dysfunction from the age of 6 months, underwent Y-hepaticojejunostomy according to Roux after dissection of the entire extrahepatic duct with severe periductal fibrosis. At that time, liver biopsy already showed progress to biliary cirrhosis with prominent ductal proliferation in Glisson's triad. Although good bile drainage to the intestine was obtained early after the operation, 2 years elapsed until disappearance of jaundice and ascites with improvement of liver function. The patient is now 13 years old and doing well with moderately impaired liver function. No oesophageal varices have been seen by endoscopy lately. The clinical course and data are suggestive of an abortive form of biliary atresia.
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PMID:Inflammatory stenosis of the whole extrahepatic bile duct in a child: a case report. 666 67

Extrahepatic biliary atresia is defined as partial or total absence of permeable bile duct between porta hepatis and the duodenum. The incidence varies from 1:8,000 to 1:10,000. Cholestasis is total and permanent. 131I Rose Bengal test and needle liver biopsy allow correct identification of 95% of cases before surgery. Before the surgical procedure described by Kasai in 1959, all patients died between 1 and 2 years of age. Through the use of different types of Kasai's procedures by experienced groups, 30 to 35% of patients are successfully operated on. In our group, 248 infants underwent surgery, 121 before 1977: 44 (36.6%) are alive at least 5 years after surgery. Growth is within normal limits in all children still alive, even in those who presented with numerous and severe episodes of cholangitis during the first two postoperative years. Portal hypertension was progressive in 23 children with splenomegaly and esophageal varices developing between 1 and 2 years of age. Five patients bled profusely between 2 and 4 years of age: portal systemic shunts were performed in 4. Surgical liver biopsies were performed in 20 children, 5 to 8 years old: cirrhosis was present in all and was micronodular in 13 and macronodular in 7. The most impressive histologic change was absence of biliary ducts or ductules. This histologic finding can be related with percutaneous transhepatic cholangiographic pictures. Despite the abnormal appearance of intrahepatic bile ducts and the constant presence of cirrhosis with portal hypertension, all 44 patients who are alive at least 5 years after surgery seem to lead almost normal lives.
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PMID:Extrahepatic biliary atresia. 669 74

Clinical, biological, and histological data are described in 20 patients at least 5 years after surgery for extrahepatic biliary atresia. Seventeen had an " uncorrectable " type and underwent hepatoportoenterostomy or hepatoportocholecystostomy , 3 patients had a "correctable" type and underwent cystojejunostomy . Clearing of jaundice was observed in all patients. Portal hypertension developed in 16 out of the 20. Liver specimens were obtained by surgical biopsy in all patients. Definite biliary cirrhosis was seen in all but two. Particular patterns were observed in some cases: biliary structures without bile stasis were absent in 8, while 4 displayed marked cell infiltration in portal areas, and 7 had distended vascular channels. The appearance of liver cells was always normal. No relationship could be clearly established between the degree of fibrosis and the earlier presence of cholangitis.
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PMID:Histological liver evaluation 5 years after surgery for extrahepatic biliary atresia: a study of 20 cases. 674 86


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