1/6. Atypical morphologic presentation of biliary atresia and value of serial liver biopsies.BACKGROUND: Liver biopsy findings are important in diagnosing extrahepatic biliary atresia. Diffuse ductular proliferation is a characteristic finding. We describe four patients with conjugated hyperbilirubinemia in whom the initial liver biopsy findings showed a lack of ductular proliferation, despite subsequent development of biliary atresia. RESULTS: On initial biopsy, paucity of intrahepatic bile ducts was present in three of four patients, with a bile duct to portal space ratio of 0.3 to 0.4 (normal, 0.9-1.8). A normal bile duct to portal space ratio of 1.0 was observed in the fourth patient. Ductular proliferation became apparent in three subjects between 9 and 12 weeks of age, and biliary atresia was noted at the time of a Kasai portoenterostomy. The fourth child had well-developed biliary cirrhosis at liver transplantation. CONCLUSIONS: Changes characteristic of biliary atresia may appear even after 9 weeks of age. Bile duct paucity and normal bile duct to portal space ratio do not preclude the subsequent development of biliary atresia. Infants with unexplained conjugated hyperbilirubinemia and acholic stools should undergo sequential liver biopsies until clinical improvement occurs or until biliary atresia can be excluded from the differential diagnosis.- - - - - - - - - - ranking = 1keywords = atresia (Clic here for more details about this article) |
2/6. aspergillus cholangitis: A late complication after Kasai portoenterostomy.Ascending bacterial cholangitis can be a recurring complication in older children and adolescents who have undergone successful surgery for biliary atresia. The authors describe a 19-year-old girl with a history of recurrent late-onset, febrile cholangitis in whom persistent jaundice developed. aspergillus terreus was isolated from her biliary fluid obtained at percutaneous transhepatic cholangiography. Despite prolonged external biliary drainage and systemic amphotericin b therapy, the fungus was not eliminated from her biliary system. aspergillus cholangitis can occur in the portoenterostomy patient and may be highly resistant to conventional therapy.- - - - - - - - - - ranking = 0.1keywords = atresia (Clic here for more details about this article) |
3/6. Extrahepatic biliary atresia and caudal regression syndrome in an infant of a diabetic mother.Although most cases of extrahepatic biliary atresia are thought to result from perinatal obliterating inflammation, some are associated with a faulty morphogenesis. The authors report on a baby girl of a mother with ill-managed insulin-dependent diabetes mellitus. The baby presented with sacro-coccygeal agenesis, clubfoot, and ano-urinary incontinence. In addition, there was polysplenia, no inferior vena cava, and the portal vein was grossly distorted. Progressive conjugated hyperbilirubinemia prompted liver biopsy at 4 weeks of age, showing intracanalicular cholestasis without fibrosis. The diagnosis of extrahepatic biliary atresia was confirmed during laparotomy performed for hepatoportojejunostomy. Some cases of extrahepatic biliary atresia might be part of a spectrum of malformations associated with maternal diabetes mellitus.- - - - - - - - - - ranking = 0.7keywords = atresia (Clic here for more details about this article) |
4/6. A novel approach to the management of late-onset liver failure in biliary atresia.PURPOSE: Late-onset hyperbilirubinemia in patients who have undergone a successful portoenterostomy (PE) for biliary atresia (BA) is usually considered evidence of ongoing severe liver failure. The authors recently have treated 2 patients who had acute hyperbilirubinemia years and months after a successful PE and had dilated intrahepatic cysts. A combined operative and percutaneous approach reestablished drainage and a reduction in their bilirubin levels. methods: Data from 2 cases of BA and late-onset hyperbilirubinemia from obstruction were reviewed and analyzed. RESULTS: Two patients (15-year-old boy and a 2.5-year-old girl) presented with increasing serum bilirubin after a PE for BA in infancy. Both had extensive preoperative workup, which showed intrahepatic biliary dilatation in one and a large bile lake in the other. They underwent attempted percutaneous transhepatic cholangiography and stenting, followed by an intraoperative transhepatic approach in which the dilated ducts were connected to the PE. A rapid and sustained reduction in the serum bilirubin level was noted in these patients. CONCLUSIONS: When patients with BA after a successful PE present with sudden onset of hyperbilirubinemia, imaging for biliary obstruction should be carried out. If biliary dilatation is found, then a combined radiologic and operative approach may help improve the bile flow and delay the need for liver transplant.- - - - - - - - - - ranking = 0.5keywords = atresia (Clic here for more details about this article) |
5/6. Dental findings of children with biliary atresia: report of seven cases.patients with congenital biliary atresia (CBA) showed green staining of the teeth and soft tissues. Current cases of biliary atresia show various degrees of green staining in the primary and/or permanent dentition, suggesting a correlation between the degree of staining and the severity of the disease. Rampant caries was found in five out of seven patients. The importance of meticulous oral hygiene should be emphasized.- - - - - - - - - - ranking = 0.6keywords = atresia (Clic here for more details about this article) |
6/6. False-positive rubella hemagglutination-inhibition (HAI) titers in neonates and children with conjugated hyperbilirubinemia.Two neonates, one with extrahepatic biliary atresia and one with cystic fibrosis, and a 9-year-old child with atresia of the common bile duct had conjugated hyperbilirubinemia and elevated rubella HAI titers when kaolin pretreatment of serum was used. A beta-lipoprotein fraction of the serum that is frequently found in association with biliary obstruction was shown to be the probable source of the rubella HAI inhibitor. This beta-lipoprotein was not removed by standard kaolin treatment of serum, but was removed almost completely by dextran sulfate--calcium chloride treatment. In the presence of conjugated hyperbilirubinemia, routine kaolin pretreatment of serum is an inadequate measure for the removal of interfering substances, as false-positive rubella HAI results are obtained consistently.- - - - - - - - - - ranking = 0.2keywords = atresia (Clic here for more details about this article) |