Feeding Difficulties in Children with Hepatic Glycogen Storage Diseases Identified by a Brazilian Portuguese Validated Screening Tool

<b>Background/Objectives</b>: 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. <b>Methods</b>: A total of 29 caregivers of children aged 6 months to <7 years participated. Feeding difficulties were evaluated using the Brazilian Infant Feeding Scale (<i>Escala Brasileira de Alimentação Infantil</i>-EBAI), while parental stress was measured using the Parental Stress Scale (<i>Escala de Estresse Parental</i>-EEPa). Data were collected in 2020, and the study was approved by the ethics committee. <b>Results</b>: 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. <b>Conclusions</b>: The findings confirm a high prevalence of feeding issues in children with GSDs, which significantly affects caregivers' 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.

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Publication Details

Journal
Nutrients
Published
2025-05-23
DOI
https://doi.org/10.3390/nu17111758
Primary Topic
Child Nutrition and Feeding Issues
Type
article
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article

Feeding Difficulties in Children with Hepatic Glycogen Storage Diseases Identified by a Brazilian Portuguese Validated Screening Tool

Carolina Fischinger Moura de Souza, Katia Irie Teruya, Mariana Lima Scortegagna, Lı́lia Farret Refosco et al.
Nutrients
Child Nutrition and Feeding Issues
article

Feeding Difficulties in Children with Hepatic Glycogen Storage Diseases Identified by a Brazilian Portuguese Validated Screening Tool

Carolina Fischinger Moura de Souza, Katia Irie Teruya, Mariana Lima Scortegagna, Lı́lia Farret Refosco, Patrícia Barcellos Diniz, Bibiana Mello de Oliveira, Belinda M. Sartor, Tássia Tonon
article en

Abstract

<b>Background/Objectives</b>: 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. <b>Methods</b>: A total of 29 caregivers of children aged 6 months to <7 years participated. Feeding difficulties were evaluated using the Brazilian Infant Feeding Scale (<i>Escala Brasileira de Alimentação Infantil</i>-EBAI), while parental stress was measured using the Parental Stress Scale (<i>Escala de Estresse Parental</i>-EEPa). Data were collected in 2020, and the study was approved by the ethics committee. <b>Results</b>: 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. <b>Conclusions</b>: The findings confirm a high prevalence of feeding issues in children with GSDs, which significantly affects caregivers' 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.

NutrientsVol. 17(11)
Universidade Federal do Rio Grande do Sul (BR), Hospital de Clínicas de Porto Alegre (BR)
Openalex Percentile: Top 86%
Child Nutrition and Feeding Issues
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