Kartagener syndrome is an autosomal recessive disorder characterized by primary ciliary dyskinesia accompanied by sinusitis, bronchiectasis, and situs inversus. Synchronous extrahepatic biliary atresia and Kartagener syndrome are very rare. During the preoperative preparation of patients with Kartagener syndrome, special attention is required for the respiratory and cardiovascular system. It is...

The phenomenon of patent biliary tree at birth and subsequent development of biliary atresia later in the neonatal or infantile period has never been documented till date. In that sense, the authors have tried to report this phenomenon for the first time in the world literature. However, there are several caveats in this hypothesis proposed by the authors [1]. Firstly, the diagnosis of patent b...

Background: The use of high-dose steroid therapy peri portoenterostomy may have a positive impact on the frequency of cholangitis and survival rate. Methods: A prospective study was conducted on two groups of patients (less than three months of age) suffering from biliary atresia from 1999 to 2005. The patients in group I (G I) were managed peri-operatively by high-dose methylprednisolone while...

NEED AND PURPOSE OF REVIEW Biliary atresia is a progressive obstructive cholangiopathy and is fatal if left untreated within 2 years of life. Delay in referral is because of difficulties in differentiating it from physiologic jaundice and identifying an abnormal stool color. This paper presents an overview on the diagnosis and discusses the current strategies in the management of this disease i...

BACKGROUND The purpose of the study was to compare the intraoperative blood glucose changes and the dosage of glucose infused between biliary atresia and glycogen storage disease (GSD) patients undergoing living donor liver transplantation (LDLT). PATIENTS AND METHODS The anesthesia records of biliary atresia and GSD patients undergoing LDLT were reviewed retrospectively. The levels of intrao...

OBJECTIVE To determine the prognostic factors and optimal approaches to the diagnosis and management of biliary atresia, the leading indication for liver transplantation in children. STUDY DESIGN A retrospective study was performed of all children who underwent hepatoportoenterostomy (HPE) for biliary atresia between 1997 and 2000 at 9 centers in the United States. Outcome at age 24 months wa...

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 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...

Eleven children, eight females and three males, ranging in age from 17 months to seven years, eight months, with surgically corrected biliary atresia, were examined for oral problems. Four children had green staining of both p~’mary teeth and gingival tissues. Five children had abnormally large pulp chambers, and four had delayed primary tooth eruption. A significant relationship between primar...

Secondary obstructive cholangiopathy is characterized by intra- or extrahepatic bile tract obstruction. Liver inflammation and structural alterations develop due to progressive bile stagnation. Most frequent etiologies are biliary atresia in children, and hepatolithiasis, postcholecystectomy bile duct injury, and biliary primary cirrhosis in adults, which causes chronic biliary cholangitis. Bil...