Evaluation of food intake in patients with mucopolysaccharidosis

Autor: Vaneisse Cristina Lima Monteiro, Renata B. Oliveira, Rita de Cássia de Aquino, Agatha Nogueira Previdelli, Ana Maria Martins, Beatriz Jurkiewicz Frangipani, José Araújo de Oliveira Silva, Vânia D'Almeida, Patrícia Rossetti Dearo
Rok vydání: 2018
Předmět:
Zdroj: Nutrire. 43
ISSN: 2316-7874
Popis: Background Mucopolysaccharidosis (MPS) are inborn errors of metabolism (IEM) recognized by deficient enzymes enrolled in glycosaminoglycans catabolism. The resulting accumulation of glycosaminoglycans leads to clinical progressive and generalized manifestations. Considering the severity of MPS and the relevance of establishing dietetic strategies to these patients, the present study was tailored to evaluate the food intake in patients with MPS types I, II, and VI. Methods Food intake in patients with MPS I, II, and VI was assessed. A descriptive cross-sectional study was conducted. Energetic demand based on estimated necessity of energy equations and reported food intake was analyzed. Total energetic value and nutrients (vitamins B1, B2, C, calcium, iron, and phosphate) were analyzed in accordance with the standardized interval for macronutrient distribution and the method of apparent adequacy for nutrient intake. Results Food intake of 17 patients (6- to 30-year-olds) was considered adequate regarding macronutrients. Children and adolescents failed in presenting this parameter. Macronutrients were satisfactory in both groups. Children and adolescents displayed increased intake of iron and vitamins B1 (p p Conclusions The results obtained demonstrated that patients with MPS have nutrition deficiency in their diet, which may directly or indirectly influence the course of the disease.
Databáze: OpenAIRE
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