总结4例遗传性血小板无力症患儿的护理经验。针对该类患儿先天基因缺陷引起血小板功能低下,不易止血、合并皮肤黏膜损伤后容易导致严重失血的特点,采取相应的护理措施,对严重失血患儿进行急救护理及针对性治疗;消化道出血患儿胃镜检查围术期风险评估及干预;心理护理;出院前对患儿及家长进行自发性出血应急处理及预防指导等。4例患儿症状均好转,病情稳定出院。其中1例患儿9个月后成功实施非血缘异基因造血干细胞移植,经过精细化护理,未发生移植物抗宿主病。
To summarize the nursing experience of 4 children with hereditary Glanzmann thrombasthenia. In view of the characteristics of congenital genetic defects of these children leading to low platelet function and difficulty in stop bleeding,combined skin and mucous membrane injury easily leading to severe blood loss,nursing measures were provided for emergency care and targeted treatment for children with severe blood loss. Perioperative risk assessment and intervention of gastroscopy for gastrointestinal bleeding,psychological nursing,guidance on emergency treatment of spontaneous bleeding for children and their parents before discharge were provided. All the 4 children had improved symptoms and were discharged stably. One child underwent successfully unrelated allogeneic hematopoietic stem cell transplantation 9 months later. The transplantation process was smooth without graft versus host disease after careful nursing care.
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