{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,3]],"date-time":"2026-10-03T07:40:10Z","timestamp":1791013210155,"version":"4.1.0"},"reference-count":16,"publisher":"MDPI AG","issue":"19","license":[{"start":{"date-parts":[[2024,9,25]],"date-time":"2024-09-25T00:00:00Z","timestamp":1727222400000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Nutrients"],"abstract":"<jats:p>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 &lt;5 years of age was sent to the metabolic dietitian\u2019s listserv and GMDI membership in 8\/2023. Results: There were 21 North American respondents. In 17\/21 clinics (81%), Prosobee\u00ae 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\u00ae for children &lt;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 &lt;5 years of age with GSD I. Updated guidelines are needed to help address the unique nutrition challenges in this age group.<\/jats:p>","DOI":"10.3390\/nu16193244","type":"journal-article","created":{"date-parts":[[2024,9,26]],"date-time":"2024-09-26T04:05:46Z","timestamp":1727323546000},"page":"3244","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":3,"title":["Nutrition Management in Children Less than 5 Years of Age with Glycogen Storage Disease Type I: Survey Results"],"prefix":"10.3390","volume":"16","author":[{"given":"Mary","family":"Sowa","sequence":"first","affiliation":[{"name":"Division of Metabolics, Children\u2019s Hospital of Orange County, Orange, CA 92868, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-1710-5285","authenticated-orcid":false,"given":"Monica","family":"Boyer","sequence":"additional","affiliation":[{"name":"Division of Metabolics, Children\u2019s Hospital of Orange County, Orange, CA 92868, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jessica","family":"Green","sequence":"additional","affiliation":[{"name":"Division of Medical Genetics, Department of Pediatrics, Duke University Medical Center, Durham, NC 27710, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Surekha","family":"Pendyal","sequence":"additional","affiliation":[{"name":"Division of Medical Genetics, Department of Pediatrics, Duke University Medical Center, Durham, NC 27710, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-1169-7072","authenticated-orcid":false,"given":"Heather","family":"Saavedra","sequence":"additional","affiliation":[{"name":"Division of Medical Genetics, Department of Pediatrics, McGovern Medical School at the University of Texas Health Science Center at Houston (UTHealth Houston), Children\u2019s Memorial Hermann Hospital, Houston, TX 77030, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2024,9,25]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"e1","DOI":"10.1038\/gim.2014.128","article-title":"Diagnosis and management of glycogen storage disease type I: A practice guideline of the American College of Medical Genetics and Genomics","volume":"16","author":"Kishnani","year":"2014","journal-title":"Genet. Med."},{"key":"ref_2","doi-asserted-by":"crossref","first-page":"439","DOI":"10.1093\/advances\/nmz092","article-title":"Dietary Management of the Glycogen Storage Diseases: Evolution of Treatment and Ongoing Controversies","volume":"11","author":"Ross","year":"2020","journal-title":"Adv. Nutr."},{"key":"ref_3","unstructured":"Bali, D.S., El-Gharbawy, A., Austin, S., Pendyal, S., and Kishnani, P.S. (2023, December 22). Glycogen Storage Disease Type I, Available online: https:\/\/www.ncbi.nlm.nih.gov\/books\/."},{"key":"ref_4","doi-asserted-by":"crossref","first-page":"1241","DOI":"10.1073\/pnas.1816143116","article-title":"Failure to eliminate a phosphorylated glucose analog leads to neutropenia in patients with G6PT and G6PC3 deficiency","volume":"116","author":"Chevalier","year":"2019","journal-title":"Proc. Natl. Acad. Sci. USA"},{"key":"ref_5","doi-asserted-by":"crossref","first-page":"1033","DOI":"10.1182\/blood.2019004465","article-title":"Treating neutropenia and neutrophil dysfunction in glycogen storage disease type Ib with an SGLT2 inhibitor","volume":"136","author":"Wortmann","year":"2020","journal-title":"Blood"},{"key":"ref_6","doi-asserted-by":"crossref","first-page":"S112","DOI":"10.1007\/BF02680007","article-title":"Guidelines for management of glycogen storage disease type I\u2014European Study on Glycogen Storage Disease Type I (ESGSD I)","volume":"161","author":"Rake","year":"2002","journal-title":"Eur. J. Pediatr."},{"key":"ref_7","doi-asserted-by":"crossref","first-page":"108364","DOI":"10.1016\/j.ymgme.2024.108364","article-title":"1 A survey: Nutrition management of children less than five years of age with glycogen storage disease type I","volume":"141","author":"Sowa","year":"2024","journal-title":"Mol. Genet. Metab."},{"key":"ref_8","doi-asserted-by":"crossref","first-page":"1016","DOI":"10.2337\/diab.26.11.1016","article-title":"Measurement of \u201ctrue\u201d glucose production rates in infancy and childhood with 6,6-dideuteroglucose","volume":"26","author":"Bier","year":"1977","journal-title":"Diabetes"},{"key":"ref_9","doi-asserted-by":"crossref","first-page":"434","DOI":"10.1016\/j.ymgme.2009.12.012","article-title":"Hypovitaminosis D in glycogen storage disease type I","volume":"99","author":"Banugaria","year":"2010","journal-title":"Mol. Genet. Metab."},{"key":"ref_10","doi-asserted-by":"crossref","first-page":"621","DOI":"10.1007\/s10545-011-9322-8","article-title":"Dietary dilemmas in the management of glycogen storage disease type I","volume":"34","author":"Bhattacharya","year":"2011","journal-title":"J. Inherit. Metab. Dis."},{"key":"ref_11","doi-asserted-by":"crossref","first-page":"917","DOI":"10.1007\/s10545-018-0200-5","article-title":"Role of continuous glucose monitoring in the management of glycogen storage disorders","volume":"41","author":"Herbert","year":"2018","journal-title":"J. Inherit. Metab. Dis."},{"key":"ref_12","doi-asserted-by":"crossref","first-page":"301","DOI":"10.3345\/cep.2020.00059","article-title":"Components of human breast milk: From macronutrient to microbiome and microRNA","volume":"63","author":"Kim","year":"2020","journal-title":"Clin. Exp. Pediatr."},{"key":"ref_13","first-page":"e46730","article-title":"The Psychological Benefits of Breastfeeding: Fostering Maternal Well-Being and Child Development","volume":"15","author":"Modak","year":"2023","journal-title":"Cureus"},{"key":"ref_14","doi-asserted-by":"crossref","unstructured":"Lyons, K.E., Ryan, C.A., Dempsey, E.M., Ross, R.P., and Stanton, C. (2020). Breast Milk, a Source of Beneficial Microbes and Associated Benefits for Infant Health. Nutrients, 12.","DOI":"10.3390\/nu12041039"},{"key":"ref_15","doi-asserted-by":"crossref","first-page":"389","DOI":"10.1210\/clinem\/dgad537","article-title":"Endogenous Glucose Production in Patients with Glycogen Storage Disease Type Ia Estimated by Oral D-6,6-2H2-glucose","volume":"109","author":"Rossi","year":"2024","journal-title":"J. Clin. Endocrinol. Metab."},{"key":"ref_16","doi-asserted-by":"crossref","unstructured":"Colonetti, K., Bento Dos Santos, B., Nalin, T., Moura de Souza, C.F., Triplett, E.W., Dobbler, P.T., Schwartz, I.V.D., and Roesch, L.F.W. (2019). Hepatic glycogen storage diseases are associated to microbial dysbiosis. PLoS ONE, 14.","DOI":"10.1371\/journal.pone.0218254"}],"container-title":["Nutrients"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/www.mdpi.com\/2072-6643\/16\/19\/3244\/pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,10,10]],"date-time":"2025-10-10T16:03:10Z","timestamp":1760112190000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.mdpi.com\/2072-6643\/16\/19\/3244"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2024,9,25]]},"references-count":16,"journal-issue":{"issue":"19","published-online":{"date-parts":[[2024,10]]}},"alternative-id":["nu16193244"],"URL":"https:\/\/doi.org\/10.3390\/nu16193244","relation":{},"ISSN":["2072-6643"],"issn-type":[{"value":"2072-6643","type":"electronic"}],"subject":[],"published":{"date-parts":[[2024,9,25]]}}}