Unusual Clinical Course for Untreated Malformative Biliary Atresia Infant: Is Portal Hypertension an Important Driver of Liver Fibrosis?

Fabiola Di Dato, Giulia Ranucci, Jean de Ville de Goyet, Daniele Alberti, Raffaele Iorio

Research output: Contribution to journalArticlepeer-review

Abstract

ABSTRACT: In biliary atresia, infants left untreated, and in those with unsuccessful porto-enterostomy, hepatic condition and function worsen rapidly towards cirrhosis, malnutrition, portal hypertension with ascites, and variceal haemorrhage; many die within the first 3 years of life unless they benefit from liver replacement. We describe a girl with biliary atresia splenic malformation syndrome, who had portal vein cavernoma and microsplenia; she did not undergo porto-enterostomy. She survived with her native liver over the age of 3 years. Remarkably, she remained in satisfactory condition in absence of ascites or severe hepatic dysfunction, when 4 other similar patients-managed during the same period of time-all had the usual clinical deterioration and ascites, with the need for liver replacement. To our knowledge, there is no similar report in literature. Possible pathogenetic mechanisms and the role of portal hypertension as important factors are discussed.

Original languageEnglish
Pages (from-to)216-219
Number of pages4
JournalJournal of Pediatric Gastroenterology and Nutrition
Volume72
Issue number2
DOIs
Publication statusAccepted/In press - Aug 25 2020

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