Nutrient intakes of adolescents with phenylketonuria and infants and children with maple syrup urine disease on semisynthetic diets

Sareen Stepnick Gropper, Mary C. Naglak, Maria Nardella, Amy Plyler, Sheah Rarback, Steven Yannicelli

Research output: Contribution to journalArticlepeer-review

13 Scopus citations

Abstract

Adequacy of nutrient intakes of adolescents with and without phenylketonuria (PKU) and infants and children with and without maple syrup urine disease (MSUD) were assessed using 3-day diet records sorted by disease and by age of the subject. Mean intakes of all nutrients were greater than two-thirds of the Recommended Dietary Allowances (RDA) or Estimated Safe and Adequate Daily Dietary Intakes (ESADDI) for all adolescents studied, with the exception of selenium (Se) in PKU adolescents, which averaged 27.8 μg. For adolescents with PKU, >50% of the RDA or ESADDI for all nutrients was provided by elemental or modified protein hydrolysate medical foods, except for vitamin A in children aged 11-15 years and Se in children 11-18 years. Mean nutrient intakes of all infants and children were greater than two- thirds of the RDA or ESADDI for all nutrients except Se in MSUD children aged 1-11 years, where intakes ranged from 6.4 to 13.2 μg (21-66% of the RDA). The medical foods provided for most of the RDA and ESADDI recommendations, with the exception of Se in MSUD children.

Original languageEnglish (US)
Pages (from-to)108-114
Number of pages7
JournalJournal of the American College of Nutrition
Volume12
Issue number2
DOIs
StatePublished - Apr 1 1993

ASJC Scopus subject areas

  • Medicine (miscellaneous)
  • Nutrition and Dietetics

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