Abstract:

To summarize the perioperative nursing experience of a child with erythropoietic protoporphyria who underwent liver transplantation and splenectomy. The key points of preoperative care include implementing diversified interventions to reduce the accumulation of porphyrin in the body； actively controlling pain and improving the comfort of children； early debridement and treatment，and good care of solar allergic dermatitis. The key points of postoperative care include protecting the transplanted liver and preventing the deposition of protoporphyrin； strengthening respiratory management to control lung infection； psychological counseling throughout the process to reduce negative emotions； individualized follow-up management to prepare for bone marrow transplantation. After careful treatment and nursing，the child recovered smoothly and was discharged from the hospital. A follow-up visit after 1 month discharge showed a good recovery.

Key words: Erythropoietic Protoporphyria, Liver Transplantation, Splenectomy, Perioperative Nursing, Pediatric Nursing

摘要：

总结1例红细胞生成性原卟啉病患儿行肝移植及脾切除的围手术期护理经验。术前护理要点：实施多元化干预措施，减少体内卟啉蓄积；积极控制疼痛，提高患儿舒适感；早期进行清创处理，做好日光过敏性皮炎的护理。术后护理要点：保护移植肝，预防排斥反应；加强呼吸道管理，控制肺部感染；全程进行心理疏导，减少负性情绪；开展个体化随访管理，为骨髓移植做准备。经过精心的治疗和护理，患儿术后康复出院。出院后1个月随访，恢复良好。

关键词: 红细胞生成性原卟啉病, 肝移植, 脾切除, 围手术期护理, 儿科护理学