糖原贮积病患儿行肝移植手术术中液体及内环境的管理  

Management of fluid and metabolic/electrolytic disorders in living donor with liver transplantation in children with glycogen storage disease

作　　者：洪方晓[1] 仇焕容[1] 薛照静 张梁[1] 岳郅劼 HONG Fang-xiao;QIU Huan-rong;XUE Zhao-jing(Department of Anesthesiology,Beijing Friendship Hospital,Capital Medical University,Beijing 100050,China)

机构地区：[1]首都医科大学附属北京友谊医院麻醉科

出　　处：《临床和实验医学杂志》2020年第1期91-95,共5页Journal of Clinical and Experimental Medicine

基　　金：首都医科大学附属北京友谊医院科研启动基金(编号:yyqdkt-12)

摘　　要：目的观察糖原贮积病(GSD)患儿活体肝移植手术术中液体及内环境方面的管理特点。方法回顾性分析2015~2017年5例在首都医科大学附属北京友谊医院因糖原贮积病而行亲体肝移植手术患儿的基本资料、住院天数、发病的年龄、症状、术前合并症及检查、手术及麻醉记录、液体种类、血气监测以及重症监护室(ⅠCU)的血气监测等。结果5例GSD患儿手术时均存在严重的肝功能不全,其中3例GSD-Ⅰ型、1例GSD-Ⅳ型和1例GSD-Ⅵ型,3个月~2岁出现首发症状,手术年龄为1~9岁。所有患儿的晶体液以5%葡萄糖为主,后输注了琥珀酰明胶、红细胞、血浆等液体。入室后只有1例患儿血气值基本处于正常范围,3例GSD-Ⅰ型患儿均是代谢性酸中毒、高乳酸血症,其中患儿1入室后即为低钠、低钾血症,患儿3入室后为低钠血症;所有患儿在无肝前期间的最后1个血气值显示均处于代谢性酸中毒状态,GSD-Ⅰ型患儿的乳酸浓度仍继续升高,另外患儿1 K+浓度为2.2 mmol/L,患儿3、5为高钾血症,患儿1、3、4为低钠血症。患儿4在术中最后1个血气时提示处于代谢性酸中毒状态,所有患儿此时血气中K+浓度均不正常(患儿1、2、4、5为低钾血症,患儿3为高钾血症),患儿1、2、5为高钠血症。入ⅠCU的第1个血气中,所有患儿均有3种以上的酸碱平衡以及血糖、电解质等方面异常,包括低钾、高钠、高血糖、高乳酸、酸血症或碱血症。结论GSD患儿行肝移植手术术中内环境酸碱平衡以及电解质等方面出现剧烈的变化,围手术期风险非常大,这些给麻醉医师带来了新的挑战,应加强监测并根据结果及时调整管理方案,尽可能维持其内环境稳态。Objective To observe the characteristics of intraoperative management of fluid and metabolic/electrolytic disorder in living donors with liver transplantation in children with glycogen storage disease(GSD).Methods Retrospective audits were conducted in this hospital.Pediatrics with GSD were treated with surgery(liver transplantation n=5)during 2015~2017,they were recruited.The following data were collected from medical files:age,gender,weight,duration of hospitalization,symptoms,concurrent illness,preoperative biochemical examinations,operation,anesthesia,fluid prescribed,blood gas monitoring,etc.Results Five patients with GSD had severe hepatic failure during operation and 3 cases were GSD-Ⅰtype,1 case with GSD-Ⅳtype and 1 case with GSD-Ⅵtype.The onset of first symptom appeared in 3 months to 2 years,and the age for operation was 1-9 years old.The choice of fluid supply was mainly 5%glucose solution,and then succinylated gelatin,red blood cells,blood plasma and other kind of liquids.After entering the operating room,only the value of blood gas in child 4 was basically in normal range.Three children with GSD-Ⅰtype were all suffered with acidosis and hyperlactemia.Among them,it was showed that hyponatremia and hypokalemia in child 1 after entering the room and hyponatremia in Child 3.Ⅰn the pre-hepatic period,these patients were all in metabolic acidosis in light of the last blood gas value in this period.And the concentration of lactic acid in those GSD-Ⅰpatients continued to increase.Child 1 with K+2.2 mmol/L,Children 3 and 5 with hyperkalemia,Children 1,3 and 4 with hyponatremia,Child 4 was still in the state of metabolic acidosis in last intraoperative blood gas examination.All these children had abnormal concentration of K+in the blood gas analysis at this time-point(Children 1,2,4 and 5 with hypokalemia,and Child 3 with hyperkalemia)and Children 1,2 and 5 with abnormal concentrations of hypernatremia.According to the first blood gas analysis inⅠCU,all these patients had more than three kinds of

关 键 词：糖原贮积病 肝移植 液体管理 内环境平衡 

分 类 号：R726.1[医药卫生—儿科]