Biliary atresia is a neonatal obstructive cholangiopathy that progresses to end-stage liver disease. Although the etiology is unknown, a neonatal adaptive immune signature has been mechanistically linked to obstruction of the extrahepatic bile ducts. Here, we investigated the role of the innate immune response in the pathogenesis of biliary atresia. Analysis of livers of infants at diagnosis re...

Biliary atresia (BA) is the most frequent identifiable cause of neonatal cholestasis and the majority of patients will need liver transplantation for survival. Despite surgical intervention with the Kasai portoenterostomy, significant fibrosis and cirrhosis develops early in life. An increased understanding of what causes this inflammatory fibrosing cholangiopathy will lead to therapies aimed a...

BACKGROUND Few studies on autoimmune hepatitis have enrolled non-Caucasian groups. AIMS To evaluate Brazilian children with type 1 and 2 autoimmune hepatitis regarding outcome and clinical and biochemical parameters. PATIENTS AND METHODS Thirty-six patients were submitted to a protocol that evaluated the clinical history, physical and biochemical data, and the course of the disease. Twenty-...

Biliary atresia is an obstructive cholangiopathy of infancy that progresses to end-stage cirrhosis. Although the pathogenesis of the disease is not completely understood, previous reports link TNFα to apoptosis of the bile duct epithelium in the presence of IFNγ. Here, we investigate if TNFα signaling regulates pathogenic mechanisms of biliary atresia. First, we quantified the expression of TNF...

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 ...

BACKGROUND Liver diseases are common in patients with HIV due to viral hepatitis B and C co-infections, opportunistic infections or malignancies, antiretroviral drugs and drugs for opportunistic infections. OBJECTIVE To describe the spectrum of liver diseases in HIV-infected patients attending an HIV clinic in Kampala, Uganda. METHOD Consecutive patients presenting with jaundice, right uppe...