Nutritional status of children affected by X-linked adrenoleukodystrophy.
J Hum Nutr Diet
; 36(4): 1316-1326, 2023 08.
Article
em En
| MEDLINE
| ID: mdl-36991579
ABSTRACT
BACKGROUND:
Adrenoleukodystrophy (ALD) is a rare X-linked metabolic disorder that causes the accumulation of very-long-chain fatty acids (VLCFAs) (C260) and the subsequent variety of clinical and neurological symptoms. Little is known about nutritional status and dietary habits of children affected by ALD, and so the present study aimed to assess nutritional status and food intake in children with ALD, also exploring the relationship between food intake and the consumption of disease-specific dietary supplements to reduce blood C260 concentrations and increase monounsaturated fatty acids (C261).METHODS:
All patients underwent a clinical and neurological evaluation and a comprehensive nutritional assessment. The association of VLCFA concentrations with dietary lipids was assessed.RESULTS:
Nine boys (11.49 ± 3.61 years) were enrolled in a cross-sectional study. All patients were normal weight, with normal resting energy expenditure. Only six of nine patients followed the low-fat diet and dietary supplements. An inverse association was found between the food intake of polyunsaturated lipids and C260; conversely, the C260 was positively associated with the dietary saturated lipids. When consumed, dietary supplement consumption correlated positively with C261 (ρ = 0.917, p = 0.029) and no correlation was found with C260 (ρ = 0.410, p = 0,493).CONCLUSIONS:
No children were found to be malnourished or overweight or obese; however, half of the children reported excessive body fat, probably as a result of the pharmacotherapies. A low-fat diet could be adjuvant in the management of the accumulation of VLCFAs, but poor dietary compliance to disease-specific nutritional guidelines appears to be a major problem of this condition and underlines the need for a structured and personalised nutritional management in ALD disease.Palavras-chave
Texto completo:
1
Coleções:
01-internacional
Base de dados:
MEDLINE
Assunto principal:
Adrenoleucodistrofia
Tipo de estudo:
Guideline
/
Observational_studies
/
Prevalence_studies
/
Risk_factors_studies
Limite:
Humans
/
Male
Idioma:
En
Revista:
J Hum Nutr Diet
Assunto da revista:
CIENCIAS DA NUTRICAO
Ano de publicação:
2023
Tipo de documento:
Article
País de afiliação:
Itália