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Liver transplantation for very severe hepatopulmonary syndrome due to vitamin A-induced chronic liver disease in a patient with Shwachman-Diamond syndrome.
Liver transplantation for very severe hepatopulmonary syndrome due to vitamin A-induced chronic liver disease in a patient with Shwachman-Diamond syndrome.
- Source :
-
Orphanet journal of rare diseases [Orphanet J Rare Dis] 2018 May 02; Vol. 13 (1), pp. 69. Date of Electronic Publication: 2018 May 02. - Publication Year :
- 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.
- Subjects :
- Child
Child, Preschool
Female
Humans
Liver Diseases etiology
Shwachman-Diamond Syndrome
Bone Marrow Diseases surgery
Exocrine Pancreatic Insufficiency surgery
Hepatopulmonary Syndrome etiology
Hepatopulmonary Syndrome surgery
Lipomatosis surgery
Liver Diseases complications
Liver Transplantation methods
Vitamin A toxicity
Subjects
Details
- Language :
- English
- ISSN :
- 1750-1172
- Volume :
- 13
- Issue :
- 1
- Database :
- MEDLINE
- Journal :
- Orphanet journal of rare diseases
- Publication Type :
- Report
- Accession number :
- 29716638
- Full Text :
- https://doi.org/10.1186/s13023-018-0818-2