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Published in: Orphanet Journal of Rare Diseases 1/2018

Open Access 01-12-2018 | Letter to the Editor

Liver transplantation for very severe hepatopulmonary syndrome due to vitamin A-induced chronic liver disease in a patient with Shwachman-Diamond syndrome

Authors: 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, Isabelle Meyts

Published in: Orphanet Journal of Rare Diseases | Issue 1/2018

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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.
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Metadata
Title
Liver transplantation for very severe hepatopulmonary syndrome due to vitamin A-induced chronic liver disease in a patient with Shwachman-Diamond syndrome
Authors
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
Isabelle Meyts
Publication date
01-12-2018
Publisher
BioMed Central
Published in
Orphanet Journal of Rare Diseases / Issue 1/2018
Electronic ISSN: 1750-1172
DOI
https://doi.org/10.1186/s13023-018-0818-2

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