The etiology and pathogenesis of bile duct obstruction in children with biliary atresia are largely unknown. We have previously reported that, despite phenotypic heterogeneity, genomic signatures of livers from patients display a proinflammatory phenotype. Here, we address the hypothesis that production of IFN-gamma is a key pathogenic mechanism of disease using a mouse model of rotavirus-induc...

INTRODUCTION Hepatopulmonary syndrome is a clinical syndrome that can affect patients of all ages with liver disease and is more common in children with biliary atresia. Contrast echocardiography is the test of choice to diagnose the presence of intrapulmonary vascular dilatation. The established treatment for hepatopulmonary syndrome is liver transplantation. CASE PRESENTATION We present the...

OBJECTIVE To evaluate epidemiological, clinical and prognostic characteristics of children with biliary atresia. METHODS Data regarding portoenterostomy, liver transplantation (LTx), age at last follow-up and survival were collected from the records of patients followed up in six Brazilian centers (1982-2008) and compared regarding decades of surgery. RESULTS Of 513 patients, 76.4% underwen...

OBJECTIVE To emphasize the importance of precocious diagnosis of extrahepatic biliary atresia and its direct relationship with the surgical re-establishment of the biliary flow before the second month of life. To discuss several complementary methods with the aim of selecting the ones that present better evidence, and avoiding delays in diagnosis and worse prognostic. SOURCES OF DATA Bibliogr...

Background: Biliary Atresia (BA) also known as "progressive obliterative cholangiopathy" is one of the most common conditions requiring pediatric liver transplant and surgically treatable cause neonatal cholestasis. Associated malformations are present in 25% cases it East Asia, with incidence reported high 5,000. BA presents cholestatic Jaundice, initially indistinguishable from physiological ...

tried, but with little success, and many do not recommend it because even in adult cases where carcinoma is the underlying obstructive factor, the result obtained with the Longmire procedure is invariably poor and short-lived. The final cure will ultimately be in the hands of the geneticist, who must prophylactically reduce the number of congenital atresia cases that come before the surgeon. Fi...

Extra-hepatic biliary atresia occurs in approximately 1:15,000 live births leading to about 50 new cases/year in the UK. Presentation is with prolonged jaundice, usually in a term baby who develops signs of obstructive jaundice. Management has been improved by public and professional education to encourage early referral and diagnosis to facilitate initial surgery before 8 weeks of age. Surgica...

We carried out a retrospective investigation of the 89 patients with extrahepatic biliary atresia born in The Netherlands during a 10 year period. Of these 89 patients 10 had a diagnostic laparotomy only. Eight patients had an anastomosis between the proximal bile duct and the intestine, and the remaining 71 had hepatic portoenterostomies. Bile drainage was re-established in 46 (65%). After suc...