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Growth and Nutritional Status of Phenylketonuric Children and Adolescents

Mina Ahmadzadeh1, Golbon Sohrab2, Mohammadreza Alaei3

  • 1Department of Clinical Nutrition and Dietetics, Faculty of Nutrition Sciences and Food Technology, National Nutrition and Food Technology Research Institute, Shahid Beheshti University of Medical Sciences, Tehran, Iran.

BMC Pediatrics
|November 17, 2022
PubMed

Insights

Children and adolescents with phenylketonuria (PKU) show high rates of overweight and obesity, with many experiencing vitamin D deficiency. Special medical foods are recommended to address nutritional gaps in PKU patients.

Area of Science:

  • Biochemistry
  • Pediatrics
  • Nutrition Science

Background:

  • Phenylketonuria (PKU) is a genetic disorder requiring lifelong management.
  • Assessing anthropometric and biochemical profiles is crucial for PKU patient care.

Purpose of the Study:

  • To evaluate the anthropometric and biochemical status of children and adolescents diagnosed with PKU.
  • To identify potential nutritional deficiencies and metabolic abnormalities in this population.

Main Methods:

  • Cross-sectional study involving 80 participants aged 4-18 years.
  • Collected data on demographics, anthropometrics, and biochemical markers (Vitamins B12, D3, folic acid, iron, ferritin, calcium, zinc, Phe, Tyr).
  • Utilized a three-day dietary recall questionnaire to assess nutritional intake.

Main Results:

  • High prevalence of overweight (43.8%) and obesity (37.5%) observed.
  • Significant rates of vitamin D deficiency (35%) and elevated phenylalanine (Phe) levels (70.3% in <12 years).
  • High Phe intake identified as a risk factor for overweight and obesity (OR=4.44).

Conclusions:

  • PKU patients exhibit a high incidence of overweight/obesity and vitamin D deficiency.
  • Dietary management, including specialized medical foods, is essential for addressing nutritional deficits and metabolic control in PKU.
  • Further research may explore the long-term impact of these findings on PKU patient health outcomes.
Abstract

Keywords:
Nutritional statusPhenylalaninePhenylketonuria

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