4例Ⅰ型糖原累积症患儿的饮食管理报告并文献复习

Dietary management of children with glycogen storage disease type Ⅰ: 4 cases report and literature review

总结临床中4例不同年龄阶段、不同疾病状态和营养状况的Ⅰ型糖原累积症患儿的饮食管理方法。饮食管理要点包括:建立多学科团队协作,全面评估患儿的饮食现状和饮食问题,制定个体化饮食计划,强化饮食健康教育,根据患儿的血糖情况适时调整饮食,以及坚持定期随访和记录饮食日记。4例患儿的饮食管理干预时间分别为11、6、8、4个月。比较饮食管理前后患儿的各项指标结果显示,4例患儿血糖控制情况、主要代谢指标、主要脏器功能指标以及生长发育情况均不同程度得到改善,说明科学的饮食管理能够有效帮助Ⅰ型糖原累积症患儿更好地维持血糖稳定、改善其代谢控制及生长发育状况,以及预防各种远期并发症的发生发展,值得在临床中进一步地推广实施。

To summarize the dietary management methods of 4 children with glycogen storage disease type I at different ages,different disease states and nutritional status.The key points of diet management include:establishing multidisciplinary team cooperation,comprehensively evaluating the dietary status and problems of children,developing individualized diet plan,strengthening diet health education,timely adjusting diet according to the blood glucose of children,and adhering to regular follow-up and keeping a food diary.The intervention time of dietary management was 11,6,8 and 4 months,respectively.The results of comparing the indexes of children before and after dietary management showed that blood glucose control,main metabolic indexes,main organ function indexes and growth and development of 4 children were improved to varying degrees.These results indicate that scientific dietary management can effectively help children with glycogen storage disease type I to better maintain the stability of blood sugar,improve metabolic control and growth and development,and prevent the occurrence and development of various long-term complications,which is worthy of further promotion and implementation in clinical practice.