Faculty, Staff and Student Publications

Language

English

Publication Date

9-25-2024

Journal

Nutrients

DOI

10.3390/nu16193244

PMID

39408211

PMCID

PMC11478432

PubMedCentral® Posted Date

9-25-2024

PubMedCentral® Full Text Version

Post-print

Abstract

Background: 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.

Methods: 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.

Results: 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.

Conclusion: 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.

Keywords

Humans, Infant, Child, Preschool, Glycogen Storage Disease Type I, Male, Female, Hypoglycemia, Blood Glucose, Infant Formula, Surveys and Questionnaires, Nutritional Status, Nutrition Therapy, Fasting, GSD I, nutrition management, children, cornstarch

Published Open-Access

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