1例糖原累积病Ⅳ型合并急性肝衰竭患儿急性期的护理  

Nursing care of a child with glycogen storage disease type IV complicated with acute liver failure in acute stage

作　　者：陈湘威[1] 张杨 梁秋菊 段孟会 邵梦烨[2] CHEN Xiangwei;ZHANG Yang;LIANG Qiuju;DUAN Menghui;SHAO Mengye(Pediatric Intensive Care Unite,Third Affiliated Hospital of Sun Yat-sen University,Guangzhou 510630,Guangdong,China;Department of General Pediatric,Third Affiliated Hospital of Sun Yat-sen University,Guangzhou 510630,Guangdong,China)

机构地区：[1]中山大学附属第三医院儿科重症监护室,广东广州510630 [2]中山大学附属第三医院普通儿科,广东广州510630

出　　处：《护士进修杂志》2024年第13期1445-1448,1453,共5页Journal of Nurses Training

摘　　要：总结2022年7月我科收治的1例糖原累积病Ⅳ型(glycogen storage disease typeⅣ,GSDⅣ)合并急性肝衰竭患儿急性期的护理经验。护理要点:监测血流动力学,积极抗休克;监测消化道出血量,实施止血治疗;开展人工肝治疗及预防相关并发症;连续监测血糖,根据病情与血糖动态调整营养治疗方案;开展远程探视和音乐治疗。经过6 d的救治和护理,患儿病情好转,顺利进行肝移植。术后18 d好转出院;随访1年,恢复良好。The nursing experience of a child with glycogen storage disease type IV(GSD IV)complicated by a-cute liver failure was summarized.The main points of nursing included that hemodynamics was monitoredfor active anti-shock,and the amount of gastrointestinal bleeding was supervised for the implement of hemostatic treatment,and artificial liver treatment was developed and related complications was prevented,blood glucose was continuously monitored and nutritional treatment plan was dynamically adjusted according to condition of illness and blood glu-cose,and remote visits and music therapy were conducted.After 6 days of multidisciplinary cooperation and careful nursing,the childs condition improved and the liver transplant was successfully carried out.The child was discharged 18 days after surgery,and recovered well after 1 year of follow-up.

关 键 词：糖原累积病Ⅳ型 急性肝衰竭 血糖管理 护理 

分 类 号：R473[医药卫生—护理学] R575.3[医药卫生—临床医学]