Peters C, Krivit W
Department of Pediatrics, University of Minnesota, Minneapolis, MN 55455, USA.
Bone Marrow Transplant. 2000 May;25(10):1097-9. doi: 10.1038/sj.bmt.1702398.
Hunter syndrome is an X-linked metabolic storage disorder arising from deficiency of iduronate sulfatase enzyme activity. Despite the successful use of hematopoietic cell transplantation for a variety of lysosomal and peroxisomal storage diseases, limited benefit occurs following transplantation in either the severe or mild forms of Hunter syndrome. A brief ethical commentary is provided on the case of a boy with mucopolysaccharidosis IIB (ie the mild form) who received an unrelated umbilical cord blood transplant to improve his future quality of life. Bone Marrow Transplantation (2000).
亨特综合征是一种X连锁代谢性贮积病,由艾杜糖醛酸硫酸酯酶活性缺乏引起。尽管造血细胞移植已成功用于多种溶酶体和过氧化物酶体贮积病,但无论是重症还是轻症亨特综合征患者,移植后的获益都很有限。本文就一名患有IIB型黏多糖贮积症(即轻症)的男孩接受非亲属脐带血移植以改善其未来生活质量的病例进行了简短的伦理评论。《骨髓移植》(2000年)