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Breastfeeding in the treatment of children with phenylketonuria

Viviane C Kanufre1, Ana L P Starling, Ennio Leão

  • 1Universidade Federal de Minas Gerais, Belo Horizonte, MG, Brazil. kanufre@terra.com.br

Jornal De Pediatria
|August 7, 2007
PubMed

Insights

Breastfeeding is effective for managing phenylketonuria (PKU) in infants. It adequately controls phenylalanine levels and supports growth when combined with a special low-phenylalanine formula.

Area of Science:

  • Biochemistry
  • Pediatrics
  • Metabolic Disorders

Background:

  • Phenylketonuria (PKU) is a genetic disorder requiring strict dietary management of phenylalanine (Phe).
  • Breastfeeding is a natural source of nutrition, but its Phe content poses a challenge for PKU infants.

Purpose of the Study:

  • To assess the impact of breast milk as a phenylalanine source on amino acid levels and growth in infants with phenylketonuria.
  • To compare the efficacy of breastfeeding with standard formula feeding in PKU management.

Main Methods:

  • A cohort of 35 breastfed PKU infants was compared with 35 formula-fed PKU infants, matched for age and sex.
  • Blood phenylalanine levels were monitored weekly/fortnightly, and anthropometric data were collected.
  • Special phenylalanine-free formula was administered alongside breastfeeding or formula feeding.

Main Results:

  • Median time for phenylalanine normalization was 8 days (breastfed) vs. 7 days (formula-fed).
  • Normal phenylalanine levels were achieved in 87% of tests in the breastfed group versus 74.4% in the formula-fed group.
  • Most infants in both groups maintained appropriate growth (z score > -2).

Conclusions:

  • Breastfeeding can be safely continued during phenylketonuria treatment.
  • Breast milk, supplemented with a special Phe-free formula, supports adequate metabolic control and growth in PKU infants.
Abstract

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