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)

Anomalous junction of the pancreaticobiliary ductal system (AJPBDS) was found not only in 6 of 28 children with congenital dilatation of the bile duct, but in 3 of 57 children with congenital biliary atresia, in 2 of 17 children with infantile hepatitis and in one child with chronic pancreatitis during the past 17 yr at the Kagoshima University Hospital. A new technique of selective choledochopancreatography has been developed in which the mid-choledochus is clamped and dye is selectively injected into the distal choledochus to demonstrate the pathology of the bile duct. This technique has been successful even in small children when other cholangiographic techniques have failed to show AJPBDS. AJPBDS can be divided into two main types: (1) with biliary tract dilatation and (2) without biliary tract dilatation. The preferred treatment for type 1 is considered to be Roux-en-Y limb hepaticojejunostomy with primary excision of the choledochal cyst in order to prevent reflux of pancreatic juice flow into the choledochus, postoperative gall stone formation, or cancer from arising in the remaining choledochal cyst. Hepatic portocholecystostomy for biliary atresia with patency between gall bladder and papilla duodeni associated with AJPBDS can not be recommended since it may cause dilatation or obstruction of the bile duct postoperatively. Further studies concerning the etiology or therapy for AJPBDS are necessary.
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PMID:Congenital biliary tract dilatation and anomalous junction of the pancreatico-biliary ductal system. 42 74

The relationship between neonatal hepatitis and biliary atresia has been extensively discussed. Clinical and laboratory evaluations are often unsuccessful in distinguishing between the two diseases. Laparotomy, cholangiogram, and liver biopsy are often required. Several authors have proposed that the two diseases may represent variable expressions of one entity. A case that illustrates infantile obstructive cholangiopathy is presented and the clinical implications are discussed.
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PMID:Infantile obstructive cholangiopathy. 43 76

Serum 25-hydroxy-vitamin D (25-OHD) concentrations were measured in 49 patients with hepatobiliary disease in infancy. Low mean values were found in groups of patients with biliary atresia, neonatal hepatitis, choledochal cyst, and chronic intrahepatic cholestatic syndrome. In the group of patients with surgically repaired biliary atresia, the mean value did not differ from normal. Parenteral vitamin D increased 25-OHD in serum in patients with biliary atresia, but did not do so in one patient with neonatal hepatitis. In contrast, oral vitamin D did not increase serum 25-OHD concentrations in patients with biliary atresia. It is concluded that the reduction of serum 25-OHD seen in biliary atresia was largely due to the malabsorption of vitamin D, while in neonatal hepatitis it was due to impairment of 25-hydroxylation of the vitamin.
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PMID:Serum 25-hydroxy-vitamin D in hepatobiliary disease in infancy. 47 12

Among 137 non-selected liver tissue specimens of 101 infants between 1968 and 1978 six cases of extrahepatic biliary atresia were found. For the pathologist examining the liver cylinder the differential diagnosis between biliary atresia and neonatal hepatitis may be extemely difficult. Findings typical of extrahepatic biliary atresia, such as obstruction of portal tracts, cannot be observed before the 4th or 6th week. Formerly, biliary atresia was considered as a congenital deformity. However, under morphologic aspects a positive syntrophy or a causative relationship between neonatal hepatitis and biliary atresia can at present not be excluded.
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PMID:[Histomorphological liver changes due to extrahepatic biliary stresia]. 54 64

Aetiological factors were sought prospectively in 55 babies with extrahepatic biliary atresia, in 105 with neonatal hepatitis, and in 11 with intrahepatic biliary atresia, seen as a result of nearly complete ascertainment of these conditions in the State of Victoria between 1963 and 1974. In neonatal hepatitis infective causes were shown in 22 babies, galactosaemia in 6 and alpha1-antitrypsin deficiency in 8; familial occurrence was noted in 10 further babies and unrelated second diseases were present in 24 of the remaining 59 babies. The only clues to aetiology in extrahepatic biliary atresia were a suspicion of time-space clusters, a deficiency of affected babies born to young primiparous women, and an unexpected number of spontaneous abortions in the histories given by the mothers. Genetic factors appeared to be important in intrahepatic biliary atresia, but are not reported in detail. Hypotheses for the aetiology of neonatal hepatitis and of extrahepatic biliary atresia are presented. Both are considered syndromes with multiple causes. Recurrence risks in sibs are discussed, and are 1 in 7 for neonatal hepatitis of unknown cause, negligible in extrahepatic biliary atresia, and usually 1 in 2 or 1 in 4 in intrahepatic biliary atresia, depending upon the family history.
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PMID:Studies of the aetiology of neonatal hepatitis and biliary atresia. 55 75

Operative liver biopsies and specimens of the extrahepatic ducts and porta hepatis have been studied in 12 cases of biliary atresia. In all cases, the liver showed giant cell transformation and inflammation with mononuclear cells and neutrophils. Most had other features of neonatal hepatitis, including necrosis of hepatocytes. In the intrahepatic bile ducts of all cases but one, the hepatic ducts and glands at the porta hepatis, and in the extrahepatic ducts where epithelium remained, there was degeneration of the epithelium and intramural inflammation. In the ducts at the porta hepatis and in 6 of 8 extrahepatic ducts where epithelium remained, there was extensive mural fibrosis compromising the diameter of the duct lumens. Three cases showed the inflammatory changes distal to sites of closure of the extrahepatic ducts. These findings demonstrate that in biliary atresia, hepatitis, intrahepatic cholangitis, and sclerosing cholangitis of the extrahepatic ducts all interact to produce acquired obstruction to bile flow.
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PMID:Biliary Atresia: histopathologic observations and reflections upon its natural history. 59 78

The concentration of individual bile acids in serum was measured in 18 neonates and infants with various cholestatic conditions (extrahepatic biliary atresia, neonatal hepatitis syndrome, chronic intrahepatic cholestasis and posthemolytic cholestasis). The cholate/chenodeoxycholate ratio in serum was smaller than one in all patients with neonatal hepatitis syndrome or extrahepatic biliary atresia, cholestatic conditions which were accompanied by signs of liver cell injury. It was greater than one in the patients with chronic intrahepatic cholestasis. Administration of cholestyramine to patients with patent extrahepatic bile ducts decreased the total concentration bile acids in serum and elevated the cholate/chenodeoxycholate ratio. Thus, cholestyramine administration may be of diagnostic value for evaluation of bile duct patency in cholestasis of infancy. Differences between the bile acid pattern in serum and bile were observed. Thus, the cholate/chenodeoxycholate ratio was always higher in bile than in serum. 3beta-hydroxy-5-cholenoic acid found in serum was not detectable in bile. This finding suggests that impairment of biliary excretion rather than increased hepatic synthesis is responsible for elevation of this monohydroxy bile acid in serum.
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PMID:Bile acids in serum and bile of infants with cholestatic syndromes. 61 61

Metabolism of calcium and magnesium may be disturbed in hepatobiliary disease because of deficient or absent bile flow into the gut, since bile is important for the intestinal absorption of these elements. In the present paper the tubular reabsorption of phosphate (TRP), calcium (TRCa), and magnesium (TRMg) were determined in an attempt to evaluate the parathyroid function of infants and children with hepatobiliary disease. In unrepaired biliary atresia TRP was conspicuously reduced (mean 49.8%, SD 15.1). In successfully repaired biliary atresia the value was increased near the normal range (mean 80.7%, SD 8.1). In neonatal hepatitis the value was variable in individual cases, but significantly lower than the normal (mean 47.6%, SD 19.9). TRCa was reduced in one third of the patients with unrepaired biliary atresia and in one fifth of the cases of neonatal hepatitis. The value was within the normal range in repaired biliary atresia. TRMg was decreased in both unrepaired and repaired biliary atresia and in neonatal hepatitis. The effect of intravenous calcium infusion on TRP, TRCa and TRMg was evaluated in 3 patients with unrepaired biliary atresia. TRP was conspicuously enhanced after infusion. TRCa was decreased in 3 to a variable extent. TRMg was moderately increased in 2 and greatly decreased in 1. These results indicate that infants with hepatobiliary disease are in a state of secondary hyperparathyroidism because of deficient or absent bile flow into the intestines.
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PMID:Hyperparathyroidism in hepatobiliary disease in infancy. 81 5

Serum bile acid patterns were determined in 31 infants ranging in age from 4 days to 24 weeks. Fifteen infants with extrahepatic biliary atresia had a mean concentration of serum bile acids of 90 microng/ml +/- 47 SD;; 16 infants with neonatal hepatitis had a mean concentration of 60 +/- 35.5 SD. The chenodeoxycholate/cholate ratio was greater than one in 13 infants (87%) with atresia and in 10 infants (66%) with neonatal hepatitis. Except for the tendency of a higher total concentration of serum bile acids in infants with atresia, a single serum bile acid value does not differentiate neonatal hepatitis from extrahepatic biliary atresia. The high proportion of chenodeoxycholate in extrahepatic atresia is different from the pattern in other types of cholestatic disease and may reflect an underlying hepatitis.
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PMID:Serum bile acid patterns in neonatal hepatitis and extrahepatic biliary atresia. 85 62

The value of ERCP in children is demonstrated on 3 cases. By using a usual duodenofiberscope (JF-B2), in 2 cases under general anesthesia and in one case after premedication with 1 ml Buscopan i.m., a similar technique is used as in adults. In congenital choledochal cyst only ERCP allowed the exact judgement of the distal part of the common bile duct and its relation to the pancreatic duct. Therefore, an exact preoperative diagnosis can be established. When duodenofiberscope will be more improved ERCP will also be possible in the newborn infant. Than the differential diagnosis of congenital biliary atresia, other congenital failures in infants causing jaundice and hepatitis in infant will be established endoscopically.
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PMID:Endoscopic retrograde cholangiopancreatography (ERCP) performed in children. 89 84


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