Assessment of dietary intake of patients with mucopolysaccharidosis

Abstract

AIMS: To assess the dietary intake of children and adolescents with mucopolysaccharidosis. METHODS: Series of cases of mucopolysaccharidosis regularly followed in a referral center in Salvador, Bahia state, Brazil, in January-April 2012. Patients of both sexes aged between 2 and 18 years with food intake by mouth and without clinical complications were considered to inclusion. Structured anamnesis was applied contemplating socioeconomic, clinical, and dietary intake information. The food consumption data were obtained from 24-hour dietary recall and food record of three days. Possible dietary inadequacies were evaluated facing the recommendations of the Dietary Reference Intakes, 2005. Data were tabulated in 3.1 Epidata and analyzed by the statistical package R. RESULTS: Eight boys and two girls, with a median age of 10 years (interquartile range 6.4 years, minimum 3, maximum 16 years) were studied. The most common type of the disease was Mucopolysaccharidosis VI (60 %) and less frequent mucopolysaccharidosis I (10%). Six patients needed assistance to feed, nine had low calorie intake, and six had low intake of lipids. All patients had insufficient intake of fiber and adequate intake of carbohydrates and proteins. The intake of all micronutrients had impairments. CONCLUSIONS: There was a high frequency of inadequate dietary intake in children and adolescents with mucopolysaccharidosis. Some osteoarticular limitations caused by the disease lead to problems in food intake, and many patients need assistance in the act of eating. A specialized nutritional intervention may assist in quality of life and prognosis of these individuals.