Metabolic Response to Dietary Restriction in Some Inborn Errors of Amino Acids
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- Category: Vol. 77, December 2009
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Metabolic Response to Dietary Restriction in Some Inborn Errors of Amino Acids,DINA I. SHEHAB, AFAF H. SOBHY and MONA A. MOHAMAD
Abstract
Background: Genetic diseases, though individually rare, are cumulatively frequent enough to be responsible for up to 42% of deaths within the first year of life and to make a significant contribution to the 1% of children of school age with physical handicap and the 0.3% with severe learning difficulties.
Study Aim: Assessment of metabolic and nutritional status before and after nutrition education program (NEP).
Subjects and Methods: A total number of 30 child (17 girls and 13 boys), of those attending the outpatient clinic of "Dietary Intervention of Inborn errors of Metabolism" at the National Nutrition Institute, Cairo, were selected. They were mainly referred from Children Hospital, Kasr El-Ainy and they were suffering from one of the three amino acid disorders; hyperammonia, aminoacidopathy, and organic acidurias. Sociodemographic data, food frequency pattern, 24-hour recall, morbidity recall, medical history and examination and anthropometric measurements were taken using pre-structured questionnaires. Dietary management was carried out by giving children restricted diets in protein and specific aminoacids according to each disorder.
Results: A significant change in laboratory indicators, an increase in macronutrients' intake, and a slight change in protein intake were recorded after implementation of the program. The normal weight-for-age group was higher in their protein intake and those who showed significant increase in their head circumference were higher in their caloric intake. The organic aciduria group was the least to respond. Early detected cases showed the best nutritional, anthropometric and laboratory responses.
Conclusion: Early detection of inborn errors through neonatal screening and early nutritional intervention ameliorate complications and save mental and intellectual functions. Needs for low protein products and special formula are mandatory to fulfill requirements of growth of these children.