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The management of breast feeding among infants with phenylketonuria
L McCabe1, A E Ernest, M R Neifert
1Department of Pediatrics, University of Colorado Health Sciences Center, Denver.
Insights
Breastfeeding is safe for infants with phenylketonuria (PKU), offering adequate nutrition without adverse effects. This study found no significant differences in growth or key nutritional markers between breast-fed and formula-fed infants with PKU.
Area of Science:
- Metabolic Disorders
- Pediatric Nutrition
- Genetics
Background:
- Phenylketonuria (PKU) is a genetic disorder requiring strict dietary management.
- Treatment involves low-phenylalanine formulas and controlled phenylalanine intake for normal development.
- Breast milk is a natural source of phenylalanine, necessitating careful consideration in PKU management.
Purpose of the Study:
- To compare the nutritional status and growth of infants with PKU receiving breast milk versus infant formula.
- To assess the safety and efficacy of breastfeeding as a primary phenylalanine source in PKU management.
- To identify any potential adverse nutritional consequences of breastfeeding in infants with PKU.
Main Methods:
- Comparative study involving 18 breast-fed infants and 10 formula-fed infants with PKU.
- Monitoring of serum phenylalanine, tyrosine, and various nutritional markers (hematocrit, hemoglobin, iron, zinc, calories).
- Assessment of physical growth parameters including length, weight, and head circumference.
Main Results:
- No significant differences observed in serum phenylalanine, tyrosine, growth parameters, or most nutritional markers between the two groups.
- Breast-fed infants showed lower mean corpuscular volume at 3 and 6 months.
- Lower phenylalanine and protein intake were noted in breast-fed infants at specific time points.
Conclusions:
- Breastfeeding can be safely continued in newly diagnosed infants with phenylketonuria.
- Breast milk provides adequate nutrition for PKU infants without apparent adverse nutritional consequences.
- Further monitoring for specific parameters like mean corpuscular volume may be warranted in breast-fed PKU infants.
Abstract:
Treatment for phenylketonuria (PKU) involves using low phenylalanine-free or phenylalanine-free formulas and supplementation with sufficient phenylalanine for normal growth and development. Eighteen infants with phenylketonuria who received breast milk as their primary phenylalanine source were compared with ten other infants with PKU who received their phenylalanine primarily from infant formulas. There were no significant differences between breast-fed and formula-fed infants for serum phenylalanine, serum tyrosine, length, weight, head circumference, haematocrit, haemoglobin, serum iron, total iron binding capacity, percentage iron saturation, ferritin, plasma zinc and total calorie intake. Breast-fed infants did show lower mean corpuscular volume at 3 months and 6 months of age. Breast-fed infants had lower phenylalanine intake at 2, 4, 5 and 6 months of age. Breast-fed infants at 1, 2, 3, 4, 5 and 6 months of age had lower protein intake. Breast feeding may be continued in the newly diagnosed phenylketonuric infant without any apparent adverse nutritional consequences.