骨髓移植在黏多糖贮积症Ⅰ型中的效用。
Usefulness of bone marrow transplantation in the Hurler syndrome.
作者信息
Braunlin Elizabeth A, Stauffer Nanci R, Peters Charles H, Bass John L, Berry James M, Hopwood John J, Krivit William
机构信息
University of Minnesota Medical School, Minneapolis, Minnesota 55455, USA.
出版信息
Am J Cardiol. 2003 Oct 1;92(7):882-6. doi: 10.1016/s0002-9149(03)00909-3.
Abstract
The Hurler syndrome, an autosomal recessive storage disease of childhood, leads to death within the first decade of life from progressive deposition of glycosaminoglycans within the myointima of the coronary arteries and airways. Cardiac ultrasound findings of patients with this syndrome >10 years after successful bone marrow transplantation are described.
摘要
黏多糖贮积症I型（Hurler综合征）是一种常染色体隐性遗传的儿童期贮积病，由于糖胺聚糖在冠状动脉和气道肌内膜内进行性沉积，患者会在生命的第一个十年内死亡。本文描述了成功进行骨髓移植超过10年的该综合征患者的心脏超声检查结果。
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