【摘要】 总结了2例罹患X-连锁肾上腺脑白质营养不良的同胞兄弟实施造血干细胞移植的个性化护理经验，为同类罕见病患儿的护理实践提供参考。造血干细胞移植前护理要点：应用童趣化护理缓解移植前焦虑；强化皮肤黏膜及营养管理，为成功移植奠定基础。造血干细胞移植后护理要点：严密监测与处置骨髓抑制相关出血并发症；实施多元化随访与遗传指导。经过精细化、个体化的护理，2例患儿分别于入院第61天和第91天造血功能重建成功，病情稳定出院。出院后随访显示骨髓功能正常，恢复良好。对于行造血干细胞移植的X-连锁肾上腺脑白质营养不良患儿，实施以童趣化护理减轻心理应激，并基于个体差异严密监测、早期识别和处理移植后出血等并发症，是护理的关键。本个案为护理X-连锁肾上腺脑白质营养不良这一罕见病及造血干细胞移植相关并发症提供了实践依据。更多还原

【Abstract】 The individualized nursing experience of hematopoietic stem cell transplantation(HSCT) in 2 siblings with X-adrenoleukodystrophy(X-ALD) were summarized, aiming to provide reference for nursing practice of children with similar rare diseases.The key nursing points before HSCT included: implementing child-friendly nursing to alleviate pre-transplant anxiety; strengthening skin, mucous membrane and nutritional management to lay a solid foundation for successful transplantation.The key nursing points after HSCT included: conducting close monitoring and timely intervention of bleeding complications associated with myelosuppression; carrying out diversified follow-up and genetic counseling.With refined and individualized nursing care, the two children achieved successful hematopoietic reconstruction on the 61st and 91st day after admission respectively, and were discharged from hospital in stable condition.Post-discharge follow-up showed normal bone marrow function and good recovery.For children with adrenoleukodystrophy undergoing HSCT,the core of nursing care lied in relieving psychological stress through child-friendly nursing, as well as conducting rigorous monitoring, early identification and management of post-transplant complications such as bleeding based on individual differences.This case report provides practical evidence for the nursing care of the rare disease adrenoleukodystrophy and its HSCT-related complications.更多还原