Results Sixteen patients (2 males) were identified with median age at diagnosis of 4.96 years (range 2.5-7.83). Presenting symptoms were hyperactivity, weight loss, poor sleep and diarrhoea. They had predominant non-Anglo-Saxon ethnicity. Significant co-morbidities wereDown syndrome [1], juvenile idiopathic arthritis [1], situs inversus with extrahepatic biliary atresia ie EHBA [1].Two had fami...

Introduction: Early diagnosis of biliary atresia is very important for better outcome of treatment. Ultrasonography is one of the diagnostic tools for early differentiation of  biliary atresia from other causes of neonatal cholestasis. It has been reported that triangular cord sign  (TACS) in sonography is a reliable sign for diagnosis of biliary atresia. The aim of this study was to re-assess ...

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...

Introduction: Early differentiation of biliary atresia from neonatal hepatitis is of utmost importance, since on time surgery of biliary atresia significantly improves the outcome. Hepatobiliary scintigraphy is an integral part of diagnosis work-up of these patients; however its specificity for diagnosis of biliary atresia is suboptimal. In this study we evaluated the value of ursodeoxycholic a...

Recognition of neonatal liver disease has been heavily dependent on the occurrence of jaundice. In most instances the jaundice is related to specific disturbances in bilirubin transport and other tests of liver function are normal. In contrast, hepatitis and other liver diseases not specifically affecting bilirubin transport often go undetected unless jaundice occurs. The development of practic...

-Glutamyltransferase (GGT) is a microsomal enzyme that is widely distributed in human tissues involved in secretory and absorptive processes, particularly the bile canaliculi. Serum GGT is elevated in liver diseases affecting the biliary system, such as extrahepatic biliary atresia, sclerosing cholangitis and progressive familial intrahepatic cholestasis (PFIC) type 3. Conversely, two other sub...

Infants with idiopathic perinatal fibroinflammatory obliteration of the lumen of the extrahepatic biliary tree ("biliary atresia") invariably died of biliary cirrhosis before surgical techniques were devised to permit drainage of bile into the duodenum. Survival rates in operated patients now approach 75 percent at 10 years. While definitive diagnosis of biliary atresia without the use of chola...