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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.

Authors :
Bucciol G
Cassiman D
Roskams T
Renard M
Hoffman I
Witters P
Schrijvers R
Schaballie H
Bosch B
Putti MC
Gheysens O
Knops N
Gewillig M
Mekahli D
Pirenne J
Meyts I
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.

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