Petty E M, Yanik G A, Hutchinson R J, Alter B P, Schmalstieg F C, Levine J E, Ginsburg D, Robillard J E, Castle V P
Department of Internal Medicine, University of Michigan, Ann Arbor, 48109-0638, USA.
J Pediatr. 2000 Dec;137(6):882-6. doi: 10.1067/mpd.2000.109147.
Early death in Schimke immuno-osseous dysplasia often results from renal failure and/or cell-mediated immunodeficiency. Kidney transplants have improved renal function, but effective therapy for the immunodeficiency has not yet been reported. We describe markedly improved marrow function 2 years after bone marrow transplantation in a boy with Schimke immunoosseous dysplasia.
施姆克免疫性骨发育不良的早期死亡通常源于肾衰竭和/或细胞介导的免疫缺陷。肾移植改善了肾功能,但尚未有针对免疫缺陷的有效治疗方法的报道。我们描述了一名患有施姆克免疫性骨发育不良的男孩在骨髓移植两年后骨髓功能显著改善的情况。