Liver transplantation for very severe hepatopulmonary syndrome due to vitamin A-induced chronic liver disease in a patient with Shwachman-Diamond syndrome.
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ABSTRACT:
SUBMITTER: Bucciol G
PROVIDER: S-EPMC5930429 | biostudies-literature | 2018 May
REPOSITORIES: biostudies-literature
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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.
Bucciol Giorgia G Cassiman David D Roskams Tania T Renard Marleen M Hoffman Ilse I Witters Peter P Schrijvers Rik R Schaballie Heidi H Bosch Barbara B Putti Maria Caterina MC Gheysens Olivier O Knops Noel N Gewillig Marc M Mekahli Djalila D Pirenne Jacques J Meyts Isabelle I
Orphanet journal of rare diseases 20180502 1
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. ...[more]
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