Orphanet Journal of Rare Diseases
Liver transplantation for very severe hepatopulmonary syndrome due to vitamin A-induced chronic liver disease in a patient with Shwachman-Diamond syndrome
Orphanet Journal of Rare Diseases201813:69
© The Author(s). 2018
Published: 2 May 2018
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.
Vitamin ALiver transplantationShwachman-Diamond syndromeHepatopulmonary syndrome