Division of Metabolics, Children's Hospital of Orange County, Orange, CA 92868, USA.
Division of Medical Genetics, Department of Pediatrics, Duke University Medical Center, Durham, NC 27710, USA.
Nutrients. 2024 Sep 25;16(19):3244. doi: 10.3390/nu16193244.
Nutrition management for GSD Type I (GSDI; OMIM #232200, 232220) is complex, with the goal being to maintain euglycemia while minimizing metabolic derangements. Management guidelines were published in 2002 and 2014. However, there is limited information on the nuances of nutrition management and the unique feeding challenges of children. A REDCap survey focusing on staffing and current practices in the nutrition management of children with GSD I who were <5 years of age was sent to the metabolic dietitian's listserv and GMDI membership in 8/2023. There were 21 North American respondents. In 17/21 clinics (81%), Prosobee was the primary choice for infant formula. Dietitians used different methods to determine hourly glucose needs. Fasting recommendations ranged from 1 to 3 h, and the use of nighttime continuous feeding was common. Cornstarch was started between 6 and 12 months of age. Most clinics did not use Glycosade for children <5 years of age. Oral motor dysfunction, gagging, and lack of interest in food were common. Continuous glucose monitoring (CGM) devices were recommended in 20 clinics (95%). Most clinics followed patients on an outpatient basis. All clinics provided a hypoglycemia management plan; however, there was wide variability in practice. This survey highlights the variability in the care of individuals <5 years of age with GSD I. Updated guidelines are needed to help address the unique nutrition challenges in this age group.
I 型糖原贮积症（GSDI；OMIM #232200，232220）的营养管理较为复杂，目标是在将代谢紊乱最小化的同时维持血糖正常。管理指南已于 2002 年和 2014 年发布。然而，关于营养管理的细节以及儿童特殊喂养挑战的信息有限。2023 年 8 月，我们向代谢营养师的列表服务和 GMDI 成员发送了一份针对 5 岁以下 I 型糖原贮积症患儿营养管理的人员配备和当前实践情况的 REDCap 调查。共有 21 名北美受访者。在 21 个诊所中的 17 个（81%），Prosobee 是婴儿配方奶的主要选择。营养师使用不同的方法来确定每小时的葡萄糖需求量。禁食建议的时间范围从 1 小时到 3 小时不等，夜间连续喂养较为常见。玉米淀粉从 6 到 12 个月开始使用。大多数诊所不使用 Glycosade 治疗 5 岁以下儿童。口腔运动功能障碍、呛咳和对食物缺乏兴趣较为常见。20 个诊所（95%）建议使用连续血糖监测（CGM）设备。大多数诊所对门诊患者进行随访。所有诊所都提供低血糖管理计划；然而，实践中存在很大差异。本调查强调了 5 岁以下 I 型糖原贮积症患者护理的差异性。需要更新指南来帮助解决该年龄段的特殊营养挑战。
