Grand Rounds

Neonatal cholestasis in an infant with Rh incompatibility


Ira Shah
Consultant in Pediatric Infectious Diseases and Pediatric Hepatology, Nanavati Hospital and Incharge Pediatric HIV, TB and Liver Clinics, B J Wadia Hospital for Children, Mumbai, India.

Address for Correspondence: Dr Ira Shah, 1, B Saguna, 271, B St Francis Road, Vile Parle {W}, Mumbai 400056.


Clinical Problem:
A 1 ½ months old boy was referred for jaundice with clay stools since birth. There was history of Rh incompatibility which led to jaundice in neonatal period that required phototherapy and exchange transfusion in the baby. Subsequently the child was noticed to have direct jaundice. On examination, weight was 4.4 kg, he had hepatomegaly. Other systems were normal. Investigations showed bilirubin 6.2 mg, dl {direct=3.2 mg, dl}, SGOT = 10 IU, L, SGPT = 125 IU, L, total proteins = 5.1 gm, dl, albumin= 3.1 gm, dl, alkaline phosphatase = 626 IU, L, GGTP = 250 IU, L. HIDA scan did not show excretion of tracer into small intestine even after 24 hours. An intraoperative cholangiogram {IOC} showed free flow of dye into intestines. Liver biopsy showed minimal intrahepatic and intra-canalicular cholestasis with no bile duct proliferation and mild to moderate cholangitis. After flushing the system after IOC, and supplementing with ursodeoxycholic acid the liver function test subsequently normalized. At last follow-up at 2 years of age, liver function tests were normal and there was no portal hypertension on doppler.

What is the likely cause of neonatal cholestasis_?


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