Biliary atresia is progressive fibro-inflammatory cholangiopathy of young children. Central to pathogenic mechanisms of injury is the tissue targeting by the innate and adaptive immune cells. Among these cells, neutrophils and the IL-8/Cxcl-8 signaling via its Cxcr2 receptor have been linked to bile duct injury. Here, we aimed to investigate whether the intestinal microbiome modulates Cxcr2-dep...

Biliary cystadenocarcinoma (BCAC) is a very rare malignant cystic neoplasm. Some authors think this particular neoplasm was conversion of biliary cystadenoma over several years of evolution. In the majority of the cases, it habitually occurs in the liver parenchyma (intrahepatic cystadenocarcinoma); sometimes can be observed extrahepatic biliary origin3,4,6,7,8,10,11 extrahepatic biliary cystad...

A 4-week-old infant presented with a coagulation disorder resulting from a vitamin K deficiency. The vitamin K deficiency was caused by neonatal cholestasis due to biliary atresia. Jaundice, hepatomegaly and pale stools are the predominant presenting symptoms of biliary atresia, none of which were recognized in our patient before admission. However, the patient presented with bleeding caused by...

UNLABELLED One hundred and seventy three children, including 93 with biliary atresia, received liver grafts at Addenbrooke's Hospital between 1983 and 1993. Of these, only seven developed cyanosis due to intrapulmonary shunting as a complication of their liver disease, and all seven of these had the biliary atresia/polysplenia syndrome. Intrapulmonary shunting was confirmed by a radioisotope sc...

BACKGROUND & AIMS Altered extrahepatic bile ducts, gut, and cardiovascular anomalies constitute the variable phenotype of biliary atresia (BA). METHODS To identify potential susceptibility loci, Caucasian children, normal (controls) and with BA (cases) at two US centers were compared at >550000 SNP loci. Systems biology analysis was carried out on the data. In order to validate a key gene ide...

Simultaneous non-traumatic perforation of the extrahepatic bile duct and the gallbladder is an uncommon occurrence that has been infrequently reported. We describe a patient with a spontaneous perforation of both the extrahepatic bile duct and the gallbladder. A contrast-enhanced computed tomography (CECT) scan of the abdomen and endoscopic retrograde cholangiopancreatography (ERCP) demonstrate...

We studied the pharmacokinetics of an i.v. bolus dose of propofol 2.5-3.0 mg kg-1 in eight children (age 4-24 months) with biliary atresia and in six control (ASA I) children (age 11-43 months). Blood samples were obtained for 4 h after administration of propofol. Blood concentrations of propofol were measured by high pressure liquid chromatography. Systemic clearance of propofol (CI) and volum...