Orphanet Journal of Rare Diseases
Liver transplantation for very severe hepatopulmonary syndrome due to vitamin A-induced chronic liver disease in a patient with Shwachman-Diamond syndrome
- Giorgia Bucciol,
- David Cassiman,
- Tania Roskams,
- Marleen Renard,
- Ilse Hoffman,
- Peter Witters,
- Rik Schrijvers,
- Heidi Schaballie,
- Barbara Bosch,
- Maria Caterina Putti,
- Olivier Gheysens,
- Noel Knops,
- Marc Gewillig,
- Djalila Mekahli,
- Jacques Pirenne† and
- Isabelle Meyts†
†Contributed equally
Orphanet Journal of Rare Diseases201813:69
© The Author(s). 2018
Received: 10 August 2017
Accepted: 27 April 2018
Published: 2 May 2018
Abstract
Vitamin A intoxication is a rare cause of liver disease, but the risk increases in patients with underlying liver dysfunction. We present a patient with Shwachman-Diamond Syndrome who developed liver fibrosis, portal hypertension and very severe hepatopulmonary syndrome as a consequence of chronic vitamin A intoxication. She underwent successful liver transplantation with complete resolution of the pulmonary shunting.
Keywords
Vitamin ALiver transplantationShwachman-Diamond syndromeHepatopulmonary syndrome
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