{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,12]],"date-time":"2026-10-12T18:09:50Z","timestamp":1791828590956,"version":"4.3.4"},"reference-count":50,"publisher":"MDPI AG","issue":"11","license":[{"start":{"date-parts":[[2025,5,23]],"date-time":"2025-05-23T00:00:00Z","timestamp":1747958400000},"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\/Objectives: Hepatic glycogen storage diseases (GSDs) are inherited metabolic disorders that affect glycogen synthesis or breakdown, primarily involving the liver and muscles. Treatment typically consists of strict dietary management, including the consumption of uncooked cornstarch. However, there is limited research on feeding challenges and the associated stress experienced by parents of children with GSDs. This study aims to assess feeding difficulties in children with GSDs and the level of parental stress. Methods: A total of 29 caregivers of children aged 6 months to &lt;7 years participated. Feeding difficulties were evaluated using the Brazilian Infant Feeding Scale (Escala Brasileira de Alimenta\u00e7\u00e3o Infantil\u2014EBAI), while parental stress was measured using the Parental Stress Scale (Escala de Estresse Parental\u2014EEPa). Data were collected in 2020, and the study was approved by the ethics committee. Results: The majority of the children were male (19\/10), with a mean age of 47.75 months and an average age of diagnosis of 8.39 months. GSD type Ia (n = 15) and type Ib (n = 5) were the most prevalent, followed by types III and IX (n = 2). Among the participants, 22 out of 29 (76%) reported feeding difficulties, categorized as mild (n = 7, 24%), moderate (n = 7, 24%), and severe (n = 8, 28%). EBAI scores were higher in female patients and in those who did not eat meals with their family. Only one caregiver exhibited high levels of parental stress, as measured by the EEPA scale. No significant correlation was found between feeding difficulties and parental stress. Conclusions: The findings confirm a high prevalence of feeding issues in children with GSDs, which significantly affects caregivers\u2019 quality of life. Although no significant link between feeding difficulties and parental stress was identified, further research is needed to improve GSD management and provide better support for caregivers.<\/jats:p>","DOI":"10.3390\/nu17111758","type":"journal-article","created":{"date-parts":[[2025,5,23]],"date-time":"2025-05-23T06:16:03Z","timestamp":1747980963000},"page":"1758","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":1,"title":["Feeding Difficulties in Children with Hepatic Glycogen Storage Diseases Identified by a Brazilian Portuguese Validated Screening Tool"],"prefix":"10.3390","volume":"17","author":[{"given":"B\u00e1rbara Cristina Pezzi","family":"Sartor","sequence":"first","affiliation":[{"name":"Hospital Materno Infantil Presidente Vargas, Porto Alegre 90035-074, Brazil"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-2679-6858","authenticated-orcid":false,"given":"Bibiana Mello","family":"de Oliveira","sequence":"additional","affiliation":[{"name":"Postgraduate Program in Genetics and Molecular Biology, Universidade Federal do Rio Grande do Sul, Porto Alegre 91509-900, Brazil"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Katia Irie","family":"Teruya","sequence":"additional","affiliation":[{"name":"Postgraduate Program Psychology, Universidade Federal do Rio Grande do Sul, Porto Alegre 90035-002, Brazil"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Lilia Ramos","family":"Farret","sequence":"additional","affiliation":[{"name":"Medical Genetics Service, Hospital de Cl\u00ednicas de Porto Alegre, Porto Alegre 90035-903, Brazil"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"T\u00e1ssia","family":"Tonon","sequence":"additional","affiliation":[{"name":"Postgraduate Program in Medical Sciences Medicine, Universidade Federal do Rio Grande do Sul, Porto Alegre 90035-003, Brazil"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-9050-3343","authenticated-orcid":false,"given":"Mariana Lima","family":"Scortegagna","sequence":"additional","affiliation":[{"name":"Postgraduate Program in Medical Sciences Medicine, Universidade Federal do Rio Grande do Sul, Porto Alegre 90035-003, Brazil"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Patr\u00edcia Barcellos","family":"Diniz","sequence":"additional","affiliation":[{"name":"Hospital Materno Infantil Presidente Vargas, Porto Alegre 90035-074, Brazil"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-7280-6260","authenticated-orcid":false,"given":"Carolina Fischinger Moura","family":"de Souza","sequence":"additional","affiliation":[{"name":"Postgraduate Program in Child and Adolescent Health, Universidade Federal do Rio Grande do Sul, Porto Alegre 90035-003, Brazil"},{"name":"Clinical Research Service, Hospital de Cl\u00ednicas de Porto Alegre, Porto Alegre 90035-003, Brazil"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2025,5,23]]},"reference":[{"key":"ref_1","first-page":"2326409817733014","article-title":"Nutritional status and body composition in patients with hepatic glycogen storage diseases treated with uncooked cornstarch\u2014A controlled study","volume":"5","author":"Nalin","year":"2017","journal-title":"J. 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