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

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Abstract Vitamin A intoxication is a rare cause of merlin wizard costume 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 sophie allport bee curtains transplantation with complete resolution of the pulmonary shunting.