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- Title
Liver transplantation for very severe hepatopulmonary syndrome due to vitamin A-induced chronic liver disease in a patient with Shwachman-Diamond syndrome.
- Authors
Bucciol, Giorgia; Cassiman, David; Roskams, Tania; Renard, Marleen; Hoffman, Ilse; Witters, Peter; Schrijvers, Rik; Schaballie, Heidi; Bosch, Barbara; Putti, Maria Caterina; Gheysens, Olivier; Knops, Noel; Gewillig, Marc; Mekahli, Djalila; Pirenne, Jacques; Meyts, Isabelle
- 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.
- Subjects
HEPATOPULMONARY syndrome; LIVER transplantation; LIVER diseases; SHWACHMAN-Diamond Syndrome; VITAMIN A in the body; THERAPEUTICS; BONE marrow diseases; COMPARATIVE studies; RESEARCH methodology; MEDICAL cooperation; RESEARCH; VITAMIN A; EVALUATION research; EXOCRINE pancreatic insufficiency; LIPOMATOSIS; DISEASE complications
- Publication
Orphanet Journal of Rare Diseases, 2018, Vol 13, Issue 1, pN.PAG
- ISSN
1750-1172
- Publication type
case study
- DOI
10.1186/s13023-018-0818-2