Neonatal Cholestasis And Its Various Presentations And Approach To Diagnosis - A Case Series

Abstract

Background:

Neonatal cholestasis or conjugated hyperbilirubinemia is defined biochemically as prolonged elevation of conjugated bilirubin beyond 14days of life. It is due failure to excrete bile. This may be caused by defects in intrahepatic bile production, defects in transmembrane transport of bile or mechanical obstruction of bile flow. The latest joint recommendation from the North American and European Societies for Pediatric Gastroenterology, Hepatology and Nutrition defines a direct serum bilirubin level >1mg/dl as abnormal[1]. A direct bilirubin level of >20% of the total serum bilirubin is no longer regarded as necessary for the diagnosis of cholestasis[1]. Any infant with jaundice beyond 2weeks needs to be evaluated for Neonatal cholestasis. Rapid diagnosis is important for initiation of essential treatment for the treatable disorders. The most commonly identifiable caues are biliary atresia (BA) (25%–35%), genetic disorders (25%), metabolic diseases (20%), and a₁-antitrypsin (A₁AT) deficiency (10%). Idiopathic neonatal hepatitis (INH) was the most common non surgical cause of neonatal cholestasis before but with the discovery of more advanced diagnostic methods, the incidence of INH has substantially reduced .

Case Presentation:

In this article we describe you a series of 6 cases of neonatal cholestasis presented to a tertiary care centre in Western Mahrashtra.Here we describe the most common causes of neonatal cholestasis, and its presentation and its treatment.

Conclusion:

Biliary atresia is found to be most common surgical cause of neonatal cholestasis and its early diagnosis and essential intervention improves the quality of life, Late diagnosis may lead to liver transplant and mortality later in life.