关键词: 血小板无力症, 遗传性血小板功能障碍, 儿童, 急症护理

Abstract: 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.

Key words: Thrombasthenia, Hereditary Platelet Dysfunction, Child, Emergency Nursing