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Case of the Month – October 2017

Penelope Rose1,2, Ronalda de Lacy1, Elizabeth Goddard1

1 Paediatric Gastroenterology, Department of Paediatrics and Child Health, Red Cross War Memorial Children’s Hospital, University of Cape Town

2 Tygerberg Children’s Hospital, Department of Paediatrics and Child Health, Stellenbosch University

Case history

A one year old girl known with biliary atresia and a well-functioning Kasai portoenterostomy (bile ducts removed and a loop of intestine brought up to replace them) presented with fever and worsening jaundice of a day’s duration. She had been diagnosed with biliary atresia and had a Kasai portoenterostomy performed at the age of 8 weeks. She was also HIV-exposed and on the PMTCT program, but was confirmed PCR negative on three separate occasions. On examination she was found to be pyrexial and jaundiced with firm hepatosplenomegaly but no ascites or peritonism. She was haemodynamically stable, alert and had no evidence of bleeding.

Question 1 - What is the most likely diagnosis and why? What would the appropriate initial investigations and further management be?

Continue to Answer 1