OBJECTIVE To know the magnitude, etiology and clinical profile, the efficacy of various investigations and the outcome in patients with neonatal cholestasis syndrome (NCS). DESIGN Prospective evaluation of 60 consecutive infants with NCS (mean age 3.9 +/- 1.9 months; 49 males) over a period of 3.5 years. SETTING Tertiary level referral gastroenterology center in North India. METHODS Liver...

Extrahepatic biliary atresia (EHBA) has long been defined as a progressive cholangiopathy of infancy of obscure aetiology. It is a grave disease with serious morbidity, mortality and economic burden. EHBA is currently treated surgically by Kasai portoenterostomy. In 10 years post-portoenterostomy the survival does not exceed 30%, and 60-80% of children with EHBA eventually develop liver cirrhos...

The diagnostic accuracy of laboratory investigations in the prelaparotomy differentiation between extrahepatic biliary atresia (EHBA) and intrahepatic disease (IHD) was assessed in 86 consecutive infants presenting with conjugated hyperbilirubinaemia. Forty five infants had EHBA and 41 IHD. The mean serum bilirubin concentration, gamma-glutamyltranspeptidase (GGT) activity, and the GGT/aspartat...

Background: Extrahepatic biliary atresia (EHBA) is a chronic progressive obstructive cholangiopathy of infancy of unknown aetiology. Pathology of bile duct damage involves unanimously neutrophil elastase, variable degrees of fibrosis, and variable CD14+ monocytes intensity staining in the presence of defective p53 and glutathione S transferases Pi class (GST Pi). GST is a super family responsib...

OBJECTIVE Extrahepatic biliary atresia (EHBA) is one of the main causes of neonatal cholestasis. Its early diagnosis could increase the survival of the infants with early surgery. We evaluated the diagnostic accuracy of procalcitonin and apolipoprotein E (Apo-E) levels in infants with and without EHBA. METHODS This prospective study included 18 infants with EHBA and 15 infants with other caus...

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 : most diagnostic tests for biliary atresia (ba) are invasive. this study evaluates the sensitivity and specificity of serum  tumor necrosis factor-α (tnf-α) for diagnosing extra hepatic biliary duct atresia (ehba) in infants. materials and methods : infants with cholestasis who were admitted to children′s medical center hospital were evaluated. a total of 50 infants (20 with ehba an...

Background: Neutrophils are part of our innate immune system. They are capable of accumulation in tissues, chemotaxis, phagocytosis and digests bacteria and fungi by their lysosymes. Yet, paradoxically neutrophils are mediators in acute liver injury. Extrahepatic biliary atresia (EHBA) is a progressive chronic cholangiopathy of infancy, even in those who undergo timely portoenterostomy. Objecti...

Mutations of human jagged 1 (JAG1) gene are responsible for Alagille Syndrome (AGS), whose 2 main symptoms are intrahepatic bile duct hypoplasia and pulmonary stenosis. We examined the JAG1 mutation in extrahepatic biliary atresia (EHBA), which is similar in phenotype to AGS, although a different pathogenesis is suggested. In 102 cases of EHBA, 9 missense mutations were detected, including 2 in...

The presentation of extra hepatic biliary-atresia (EHBA) as well as choledochal cyst (CDC) in the neonate may be similar. Since the surgical management and prognosis are entirely different, it is important to differentiate between the two entities. We present a case with co-existing EHBA and CDC which led to a diagnostic dilemma.