Biliary atresia is a rare birth defect that affects 1 in 12,000 to 1 in 19,500 live births. We used data from the National Birth Defects Prevention Study, a multistate case-control study, to identify potential risk factors for isolated biliary atresia (no additional unrelated major birth defects diagnosed). Infants were identified from eight states from 1997 to 2002, with clinical information a...

Alagille syndrome may mimic biliary atresia in early infancy. Since mutations in JAG1 typical for Alagille syndrome type 1 have also been found in biliary atresia, we aimed to identify JAG1 mutations in newborns with proven biliary atresia (n = 72). Five biliary atresia patients with cholestasis, one additional characteristic feature of Alagille syndrome and ambiguous liver histology were singl...

Introduction Early diagnosis of biliary atresia is very important for better outcome of treatment. Ultrasonography is one of the diagnostic tools for early differentiation of biliary atresia from other causes of neonatal cholestasis. It has been reported that triangular cord sign (TACS) in sonography is a reliable sign for diagnosis of biliary atresia. The aim of this study was to re-assess the...

BACKGROUND/PURPOSE This report describes our experiences with 5 cases of biliary atresia associated with meconium peritonitis caused by perforation of small bowel atresia. METHODS A review of medical records was undertaken in an effort to recognize cases of biliary atresia associated with meconium peritonitis. RESULTS Five patients of 171 with biliary atresia (2.9%) were detected to have me...

Kobayashi, A., Utsunomiya, T., Ohbe, Y., and Nagashima, Y. (1974). Archives of Disease in Childhood, 49, 90. Intestinal absorption of calcium and magnesium in hepatobiliary disease in infancy. The intestinal absorption of calcium and magnesium was measured by metabolic balance studies in 6 normal infants, 13 infants with biliary atresia, 5 infants with successfully repaired biliary atresia, 7 i...

BACKGROUND Delayed diagnosis of biliary atresia is an important cause of pediatric end-stage liver failure and liver transplantation. We sought to determine whether direct bilirubin is underutilized by retrospectively reviewing patients with biliary atresia. Further, we aimed to determine the role of reflex testing for direct bilirubin in patients suspected for jaundice. METHODS The time inte...

intrahepatic bile ducts in the vicinity of the porta hepatis in biliarv atresia. Tohokuj Exp Med 1976;118: 199-207. 3 Gautier M, Eliot N. Extrahepatic biliary atresia. Morphological study of 98 biliary remnants. Arch Pathol Lab Med 1981;105:397-402. 4 Mowat AP, Psacharopoulos HT, Williams R. Extrahepatic biliary atresia versus neonatal hepatitis. A review of 136 prospectively investigated infan...

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 d...

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 v...

Multiple studies have been conducted to demonstrate the role of viruses in causing biliary atresia. Although cytomegalovirus (CMV) is known to cause intrahepatic bile duct destruction, its role in biliary atresia is not proven. We report two cases of CMV infection, initially presenting with intrahepatic cholestasis, who subsequently developed biliary atresia.