Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0019158 (hepatitis)
30,205 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

Congenital biliary atresia (CBA) is a disorder of early infancy that needs early and accurate diagnosis because the curability may be increased if surgical therapy (hepatic porto-enterostomy) is performed early in the disease course of this condition. But immediate differentiation of CBA from neonatal hepatitis (NH) is very difficult, and even special tests for differential diagnosis are of limited value because of some hazards, including the length of time required, technical difficulties, and so on. The data on 14 patients with CBA and 20 with NH admitted to Tenri Hospital were analyzed by two statistical methods (discriminant analysis and probit analysis) and then scores combining several simple variables were calculated. We tried to differentiate these two conditions by the scoring systems mentioned above, and checked the results of these methods against cases in other hospitals (CBA 6 cases and NH 15 cases). Our scoring systems presented here showed higher discriminant power than any single variable test, including the 131I-BSP excretion test, and can be used where there is no time for differential diagnosis.
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PMID:Differentiation of congenital biliary atresia and neonatal hepatitis by statistical methods. 642 77

Quantitative lipoprotein-X (Lp-X) was measured for diagnostic and postoperative examination of congenital biliary atresia (CBA). There was no significant difference in the levels in children with CBA (n = 23) or neonatal hepatitis (NH) (n = 14). However, a value over 200 mg/dl was found in 7 of CBA but not NH patients. As to 13 postoperative non-icteric patients with CBA, Lp-X was absent in only 2 patients. Thus, postoperative patients without jaundice do not always have a normal biliary secretion. From those results, estimation of Lp-X is both useful for diagnosis and postoperative evaluation of CBA.
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PMID:Clinical significance of lipoprotein-X in congenital biliary atresia. 646 60

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

A bile acid analysis by a fluorometric-enzymic method was carried out in serum from infants with neonatal hepatitis and biliary atresia. Serum bile acids were determined in 51 infants: 34 with extrahepatic biliary atresia and 17 with neonatal hepatitis syndrome. The following results were obtained: 1) There were no significant differences between neonatal hepatitis and biliary atresia concerning total serum bile acids. 2) The ratio of trihydroxylated to dihydroxylated bile acids in serum was less than one in 30 patients with biliary atresia and greater than one in 15 patients with neonatal hepatitis. 3) Conventional hepatic tests did not show any statistically significant difference between these types of patients. These results suggest that trihydroxy/dihydroxy bile acid ratio may be of clinical interest to differentiate biliary atresia from neonatal hepatitis in infants.
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PMID:[Serum profile of bile acids in children with neonatal hepatitis and extrahepatic biliary atresia]. 648 77

The relationship between reovirus type 3 and persistent infantile cholestasis was studied by measuring antibody to the virus in the sera of affected and control babies younger than 1 year of age. One hundred sixty-seven infants were divided into four groups: those with extrahepatic biliary atresia, idiopathic neonatal hepatitis, or other cholestatic disorders, and controls. When available, maternal sera obtained simultaneously with infant sera were also studied. The results indicate that 62% of babies with extrahepatic biliary atresia and 52% of infants with idiopathic neonatal hepatitis have reovirus 3 antibodies. In contrast, less than 12% of either normal infants or babies with other cholestatic disorders have antibodies. These observations suggest that perinatal infection with reovirus type 3 may serve as an initiating event in the genesis of two closely related forms of infantile obstructive cholangiopathy: extrahepatic biliary atresia and idiopathic neonatal hepatitis.
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PMID:Role of reovirus type 3 in persistent infantile cholestasis. 650 41

Recent advances of pediatric endoscopy were discussed. Progresses of technique and improvements of instrument have made it possible to scope safely resulting in the increase of pediatric endoscopies. Gastric endoscopy has become useful to find the peptic ulcer of the stomach in the newborn babies who showed hematemesis. ERCP has been done successfully in the newborn babies making it possible to differentiate congenital biliary atresia from infantile hepatitis. Colonofiberscopy has become reliable and useful procedure to search the source of rectal bleeding but still had some problems to be resolved. General anesthesia has been widely used at the performance of pediatric endoscopy. In some institutes, local anesthesia has been recommended in the patients over 8 years old. In 1983, 1161 pediatric endoscopies were performed in 24 institutes, which were twofold of those performed in 1978. Number of these cases is not enough for the training of pediatric endoscopist. Urgent theme is to establish the system for the education of "true" pediatric endoscopist.
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PMID:[Progress and focal points discussed at the Japanese Committee on Pediatric Endoscopy]. 650 83

The gamma-GT/ASAT (aspartate aminotransferase) and gamma-GT/ALAT (alanine aminotransferase) ratios were examined in 6 children with neonatal hepatitis (NH), 14 children with extrahepatic biliary atresia (EHBA), and 8 children with intrahepatic cholestasis (IHC) (of which 3 with the Aagenaes syndrome). A ratio above 1 is suggestive of EHBA. Both ratios differentiate very well between NH and EHBA, but there is considerable overlap in the enzyme ratios between the EHBA and the IHC groups. Gamma-GT/transaminase ratios may prove to be a useful indicator in the diagnostic work-up of children with liver and biliary tract disease, allowing for early surgery in patients with EHBA, and with a low risk of subjecting NH patients to unnecessary surgery. In our cases the gamma-GT/ALAT ratio separated better between EHBA and IHC than the gamma-GT/ASAT ratio. Furthermore, the case histories made EHBA seem unlikely in two out of three IHC patients with a gamma-GT/ALAT ratio above 1.
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PMID:Liver enzyme ratios in neonatal liver disease. 652 89

Seven patients with diagnosis of biliary atresia (BA) and two patients with neonatal hepatitis were studied by electron microscopy. In all patients the diagnosis was done by clinical examinations, laboratory assays, histological studies by punch or surgical biopsies and or surgical examinations combined with intraoperatory cholangiography. The ultrastructural alterations found in both groups of patients were essentially similar to those described in other forms of cholestasis. In patients suffering from biliary atresia the main features found at hepatocytic livel were: Finely granular deposits of electron dense substance sometimes conforming lamellar structures; 2) Some increase and vesiculation of smooth endoplasmic reticulum membranes; 3) Reductions in number and length of sinusoidal microvilli; in some areas was also detected basal membrane and an increase in the amount of collagen fibers in the space of Disse; 4) Marked bile canaliculi dilatations with reduction of microvilli and thickening of the pericanalicular surrounding area, some canaliculi were constitued by several hepatocytes. Ductules were found in only 3 cases with the following alterations: 1) Intracytoplasmic electron dense deposits of granular material without limiting membranes; 2) A marked increase in the number of microfilaments mainly located in the apical portion of the cell or in the vicinity of the nuclei; 3) Inflammatory cells in the duct epithelium or in direct contact with the hepatocytes. The ultrastructural findings in the two cases of neonatal hepatitis (NH) resembled those described in the biliary atresia.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:[Liver ultrastructure in congenital atresia of the extrahepatic bile ducts]. 653 1

Technetium-99m di-isopropylphenylcarbamoylmethylimidodiacetic acid cholescintigraphy, together with measurements of radioactivity in duodenal juice, was used to evaluate 23 infants with prolonged obstructive jaundice. Four patients proved to have biliary atresia. The remainder had neonatal hepatitis. There was distinct differentiation of biliary atresia from neonatal hepatitis when the time-activity curves were analyzed. In neonatal hepatitis the radioactivity in duodenal juice is obviously higher, peaking above 1500 cpm/100 microliter per mCi dose. In biliary atresia the pattern is flattened, with maximal activity below 500 cpm/100 microliter per mCi dose.
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PMID:Diagnosis of obstructive jaundice in infants: Tc-99m DISIDA in duodenal juice. 653 26


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