Clinical Features Differentiating Biliary Atresia from Other Causes of Neonatal Cholestasis

Author:

Lee Way Seah1,Chai Pei Fan1

Affiliation:

1. University of Malaya Medical Centre, Malaysia

Abstract

Introduction: This study determined any clinical features which may help to differentiate biliary atresia (BA) from other causes of neonatal cholestasis (NC). Materials and Methods: A prospective and observational study was conducted on consecutive infants with NC referred to the University of Malaya Medical Centre, Malaysia, between November 1996 and May 2004. Results: The 3 most common causes of cholestasis among the 146 infants with NC studied were idiopathic neonatal hepatitis (n = 63, 43%), BA (n = 35, 24%) and congenital cytomegalovirus hepatitis (n = 13, 9%). Common clinical features at presentation were jaundice (100%), hepatomegaly (95%), splenomegaly (52%) and pale stools (47%). Three clinical features noted to be sensitive for BA were the presence of acholic or variably acholic stools on admission, a liver which was firm/hard in consistency and a palpable liver of ≥4 cm (sensitivity of 77%, 80% and 94%, respectively), but the corresponding specificity was poor (51%, 65% and 39%, respectively). The stools of 2 children with BA were pigmented initially but became acholic subsequently. Conclusions: We did not find any single clinical feature with sufficient sensitivity and specificity to differentiate BA from other causes of NC. Repeated inspection of stools colour is necessary as occasionally, patients with BA may have initial pigmented stools. Biochemical assessment and imaging studies are important in the assessment of any infant with NC. Keywords: Differentiating features, Idiopathic neonatal hepatitis, Hepatomegaly

Publisher

Academy of Medicine, Singapore

Subject

General Medicine

Reference20 articles.

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2. McLin VA, Balistreri WF. Approach to neonatal cholestasis. In: Walker WA, Goulet O, Kleinman RE, Sherman PM, Shneider BL, Sanderson IR, editors. Pediatric Gastrointestinal Disease: Pathophysiology, Diagnosis, Management. 4th edition. Hamilton, Ontario: BC Decker, 2004.

3. Hartley J, Davenport M, Kelly DA. Biliary atresia. Lancet2009;374:1704-13.

4. Mowat AP, Psacharopoulos HT, Williams R. Extrahepatic biliary atresia versus neonatal hepatitis: review of 137 prospectively investigated infants. Arch Dis Child 1976;51:763-70.

5. Alagille D. Cholestasis in the first three months of life. Prog Liver Dis 1979;6:471-85.

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