Interferon (IFN)-γ-driven and CD8+ T cell-dependent inflammatory injury to extrahepatic biliary epithelium (EHBE) is likely to be involved in the development of biliary atresia (BA). We previously showed that viral protein NSP4 is the pathogenic immunogen that causes biliary injury in BA. In this study, NSP4 or four synthetic NSP4 (NSP4(157-170), NSP4(144-152), NSP4(93-110), NSP4(24-32)) identi...

In 25% of cases of biliary atresia associated malformations are present; polysplenia constitute 10-50% of these associated anomalies. Biliary atresia found associated with polysplenia syndrome, heterotaxy, and reverse rotation of intestine, in isolation or in various combinations; however its association with polysplenia syndrome, situs inversus abdominus, and reverse rotation of gut in the sam...

In a patient with duodenal atresia, a "double bubble" is classically present on plain radiographs. When bowel gas exists distal to the duodenum, duodenal atresia often is excluded from the differential diagnosis. The authors present a case in which contrast can be seen in the small bowel and biliary system on upper gastrointestinal series in a patient with duodenal atresia and an anomalous comm...

UNLABELLED Biliary atresia is the most common cause of death from liver disease in children. Although the Kasai operation before 60 days of age can significantly improve prognosis, delay in referral and surgery remains a formidable problem worldwide because of difficulties in differentiating it from benign prolonged neonatal jaundice. We established a universal screening system using an infant ...

BACKGROUND Increased age at surgery has a negative impact on results of the Kasai operation for biliary atresia in infancy and early childhood. It remained unclear if an age threshold exists and if this effect persists with extended follow-up. In this study we examined the relationship between increased age at surgery and its results in adolescence. METHODS All patients with biliary atresia w...

Kobayashi, A., Kawai, S., Utsunomiya, T., and Ohbe, Y. (1974). Archives of Disease in Childhood, 49, 641. Bone disease in infants and children with hepatobiliary disease. Radiological studies of bone were performed in infants and children with hepatobiliary disease. Rickets was found in 23 out of 39 patients (59%) with surgically unrepaired biliary atresia, in 4 out of 15 (27%) with surgically ...

BACKGROUND Fulminant hepatitis and biliary atresia are serious problems and their causes have not been explained well. We investigated whether or not erythrovirus B19 is a candidate etiologic agent in such liver disease patients who had undergone liver transplantation. METHODS Liver tissues from 47 patients consisted of 28 fulminant hepatitis and 19 biliary atresia were examined to detect B19...

atresia associated with prenatal infection by Listeria monocytogenes. Recent disorganization of extrahepatic bile ducts was shown in a premature infant dying at 3 hours of age of an intrauterine pneumonia associated with an amniotic infection by Listeria monocytogenes. The alimentary canal was heavily infected with Listeria and listerial abscesses were located in the periportal zones of the liv...