Body composition in patients with hepatic glycogen storage diseases.
Nutrition
; 103-104: 111763, 2022.
Article
en En
| MEDLINE
| ID: mdl-35843041
ABSTRACT
OBJECTIVES:
The present study aimed to evaluate the body composition of hepatic glycogen storage disorders (GSDs) through dual energy x-ray absorptiometry.METHODS:
This was an exploratory, observational, cross-sectional study. Twenty-four patients with GSD (type Ia n = 13, Ib n = 5, III n = 2, and IX-α/ß/γ n = 4; female sex n = 13; age <8 y n = 3, 8-19 y n = 14, and >19 y n = 7) were included. Three-day dietary records were collected in the week preceding dual energy x-ray absorptiometry. Body composition findings were correlated with clinical parameters, uncooked cornstarch (UCCS) regimen, dietary intake, and markers of treatment adherence.RESULTS:
An elevated fat mass (FM) index was found in 16 of 21 patients (age 8-19 y n = 10 and >19 y n = 6; GSD type Ia n = 12, Ib n = 2, III n = 1, and IX-γ n = 1). A lean mass (LM) index evaluation showed no LM deficits in relation to corresponding reference populations. Relative skeletal muscle index values were decreased in 2 of 7 adult patients (type Ib n = 1 and IX-α n = 1). UCCS (g/d) correlated positively with the FM index (rs = 0.7; P ≤ 0.01). In contrast, relative UCCS intake (g/kg body weight) was negatively associated with LM/kg (rs = -0.8; P ≤ 0.01).CONCLUSIONS:
These findings suggest a high frequency of elevated FM in patients with hepatic GSDs. We also suggest that treatment with UCCS is associated with excess weight in these patients. Additionally, the treatment strategy can impair protein intake, and lead to a decrease in LM.Palabras clave
Texto completo:
1
Colección:
01-internacional
Banco de datos:
MEDLINE
Asunto principal:
Composición Corporal
/
Enfermedad del Almacenamiento de Glucógeno
Tipo de estudio:
Observational_studies
/
Prevalence_studies
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Risk_factors_studies
Límite:
Adolescent
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Adult
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Child
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Female
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Humans
Idioma:
En
Revista:
Nutrition
Asunto de la revista:
CIENCIAS DA NUTRICAO
Año:
2022
Tipo del documento:
Article
País de afiliación:
Brasil