THIAMINE-DEFICIENCY AND PULMONARY-HYPERTENSION IN CROW-FUKASE SYNDROME

A 57-year-old woman with Crow-Fukase syndrome presented thiamine defic iency and pulmonary hypertension of unknown etiology, After oral admin istration of prednisolone and thiamine, echocardiogram showed marked i mprovement of the pulmonary hypertension, To our knowledge, this is th e first case of this syndrome associated with thiamine deficiency and precapillary pulmonary hypertension, which may play a role in the path ogenesis of polyneuropathy and heart failure of this syndrome.