Abstract:

To summarize the nursing experience of a pediatric patient who underwent hematopoietic stem cell transplantation following liver transplantation for erythropoietic protoporphyria，several key nursing points were identified. These included implementing plasma exchange to prevent liver failure，employing light-avoidance therapy to reduce photosensitivity，enhancing skin and mucosal care to minimize bacterial colonization，and strengthening caregiver management to prevent cross-infection. After hematopoietic stem cell transplantation，the main focus of nursing care shifted towards drug management to mitigate liver damage，closely monitoring the patient’s condition for potential nervous system lesions，implementing effective diarrhea management strategies to prevent perianal infection，prioritizing psychological support in order to alleviate negative emotions experienced by the patient，and providing individualized health education and follow-up management plans aimed at promoting rehabilitation. Because of meticulous nursing provided by a dedicated multidisciplinary team，the child showed significant improvement within 34 days post-transplantation and continued recovering well during outpatient follow-up visits conducted 4 months after hospital discharge.

Key words: Erythropoietic Protoporphyria, Hematopoietic Stem Cell Transplantation, Liver Transplantation, Pediatric Nursing

摘要：

总结1例因红细胞生成性原卟啉病行肝移植术后患儿再行造血干细胞移植的护理经验。造血干细胞移植前的护理要点：进行血浆置换，预防肝衰竭；采用避光治疗，减轻光敏反应；加强皮肤及黏膜护理，减少定植菌；加强陪护管理，避免交叉感染。造血干细胞移植后的护理要点：加强用药管理，减轻肝损伤；密切观察病情，警惕神经系统病变；加强腹泻管理，预防肛周感染；重视心理护理，减少负性情绪；开展个性化健康宣教及随访管理，促进康复。经过多学科团队的精心治疗与护理，患儿于造血干细胞移植后第34天好转出院，出院后门诊随访4个月，恢复良好。

关键词: 红细胞生成性原卟啉病, 造血干细胞移植, 肝移植, 儿科护理学