小儿血液代谢性疾病脾切除的指征及围手术期治疗

Indication and perioperative management of pediatrichema-metabolic disease for splenectomy

目的总结21例小儿血液代谢性疾病脾切除的手术指征和围手术期治疗体会。方法对21例血液代谢性疾病脾切除患儿的临床资料进行回顾性分析。结果本组患儿无死亡,术中均无大出血,术后脾功能亢进症状得以缓解。1例尼曼匹克氏病患儿脾切除术后1a,因巨大肝脏影响呼吸及活动进行右半肝部分切除;1例自身免疫性溶血性贫血患儿术后半年死于肺部严重感染。结论脾切除是治疗血液性代谢性疾病的有效方法,保留副脾或部分脾切除有助于提高术后免疫力和减少凶险性感染(OPS I)的发生。

Objective.-To explort the indication and perioperative management of pediatric hema-metabolic disease for splenectomy. Methods ：Twenty one patients with hema metabolic disease performed splenectomy were analysed retrospectively. Results ：No patient died and had massive bleeding intraoperatively,all cases the curative effects for reducement of hypersplenism is in the affirmative. One with autoimmune hemolytic anemia （AIHA） died of severe infection of lung 6 months after opration. One cases with Niemann-Pick disease NPD performed partial right hemi-hepalectorny because of magehepar one year postoperatively. Cnclusion： Splenectomy is effective in a selected group of patient with hemo-metabolic diseases. To keep paraspleen or partial splenectomy may improve the function of immune and prevent the overwhelming postsplenectomy infection.