Article
The first cardiac transplant experience in a patient with mucopolysaccharidosis.
Cardiovascular pathology : the official journal of the Society for Cardiovascular Pathology - 1 Jan 2000
Grinberg Henrique, Quaio Caio Robledo D'Angioli Costa, Avila Monica Samuel, Ferreira Silvia Moreira Ayub, Vieira Marcelo Luiz Campos, Benvenuti Luiz Alberto, Kim Chong Ae, Bocchi Edimar Alcides
Abstract excerpt
Hunter syndrome (MPSII) is a rare X-linked lysosomal storage disorder that can affect multiple systems but primarily affects the heart. We report the case of a previously asymptomatic 23-year-old patient who had an attenuated form of MPSII and presented with refractory heart failure that required a heart transplant. The diagnosis was confirmed by detection of an increase in urinary excretion of...
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