A study on cholestasis in infants less than six months of age presenting to Lady Ridgeway Hospital for Children, Colombo

Mohamed Infaq Mohamed Luthufdeen,Samantha Waidyanatha

doi:10.4038/sljch.v45i1.8083

Abstract

Objectives: To determine the common clinical presentations, common aetiological factors, average age at presentation and possible means of early detection of cholestasis by primary health care workers. Method: This prospective, descriptive study analyzed 60 infants less than six months old who presented to Lady Ridgeway Hospital from October 2013 to January 2015 with evidence of cholestasis. Results: The common clinical features were jaundice (100%), hepatomegaly (83.3%), pale stools (76.6%), dark urine (61.6%) and splenomegaly (56.6%). The common causes were idiopathic neonatal hepatitis (INHS) (26.6%) and biliary atresia (26.6%). Nine cases of INHS were transient. Sepsis (15%), multifactorial (13.3%) and cytomegalovirus infection (6.6%) were the other frequent causes. The less frequent causes were Alagille syndrome (3.2%), birth asphyxia (1.6 %), progressive familial intrahepatic cholestasis (PFIC) (1.6%), biliary sludge due to haemolysis (1.6%), intestinal obstruction (1.6%) and multiple small gall bladder calculi (1.6%). The average age at presentation of infants with cholestasis was 64.2 days and the mean age at presentation of infants with cholestasis who were eventually diagnosed as biliary atresia was 81.8 days. Conclusions: Jaundice, hepatomegaly and pale stools were the common clinical features on presentation. INHS and biliary atresia were the common causes of cholestasis. There was a significant delay in the presentation of cases of cholestasis due to primary health care failure.

Highlights

As extrahepatic biliary obstruction and neonatal hepatitis syndrome carry high mortality and morbidity rates if not treated early in life, it is important to diagnose these conditions early in order to have a good outcome[1]
The average age at presentation of infants with cholestasis was 64.2 days and the mean age at presentation of infants with cholestasis who were eventually diagnosed as biliary atresia was 81.8 days. 1Specialty Doctor in Paediatrics,The Queen Elizabeth Queen Mother Hospital, Margate, Kent, United Kingdom, 2Consultant Paediatrician, Lady Ridgeway Hospital for Children, Colombo, Sri Lanka
The frequencies of common clinical features for cholestasis in study population are shown in table 1

Summary

Introduction

As extrahepatic biliary obstruction and neonatal hepatitis syndrome carry high mortality and morbidity rates if not treated early in life, it is important to diagnose these conditions early in order to have a good outcome[1]. Idiopathic neonatal hepatitis (INHS) and biliary atresia are the most frequent causes of cholestatic jaundice in the first months of life[2,3,4]. Syndrome of neonatal hepatitis has diverse causes. Idiopathic form, sepsis/urinary tract infection, genetic diseases of metabolism and congenital infections are relatively common causes compared to toxic causes, post-haemolytic states, neonatal acute hepatic necrosis, parenteral nutrition, chromosomal anomalies, familial syndromes etc[4]. Diagnosis of extra hepatic biliary atresia as early as possible (younger than 60 days of age) is the key determinant to successful hepatic portoenterostomy[6]

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