总结2022年7月我科收治的1例糖原累积病Ⅳ型(glycogen storage disease type Ⅳ，GSD Ⅳ)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6d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。
Nursing care of a child with glycogen storage disease type Ⅳ complicated with acute liver failure in acute stage
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 child's 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.
glycogen storage disease type Ⅳacute liver failureblood glucose managementnursing
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