Breast-feeding success among infants with phenylketonuria

Sandra A Banta-Wright1, Kathleen C Shelton, Nancy D Lowe

  • 1School of Nursing at Oregon Health and Science University, Portland, OR, USA. bantawrs@ohsu.edu

Insights

Breast milk offers a lower phenylalanine (Phe) concentration ideal for infants with phenylketonuria (PKU). Breast-fed infants with PKU showed more Phe levels within the normal range compared to formula-fed infants.

Area of Science:

  • Pediatrics
  • Metabolic Disorders
  • Human Nutrition

Background:

  • Human breast milk is recommended for infants.
  • Breast milk has lower protein and phenylalanine (Phe) than formula.
  • These characteristics make breast milk suitable for infants with phenylketonuria (PKU).

Purpose of the Study:

  • To compare breast-feeding incidence and duration in infants with PKU.
  • To analyze Phe levels in breast-fed versus formula-fed infants with PKU.
  • To evaluate nutritional management strategies for PKU.

Main Methods:

  • Retrospective chart review of PKU infants (1980-2005).
  • Comparison of breast-fed and formula-fed infants.
  • Analysis of phenylalanine (Phe) levels during the first year of life.

Main Results:

  • Most infants had similar mean Phe levels regardless of feeding method.
  • Breast-fed infants with PKU were more likely to have normal Phe levels (120-360 micromol/L).
  • Breast-fed infants with PKU were less likely to have low Phe levels (<120 micromol/L).

Conclusions:

  • Breast milk can be a viable nutritional base for infants with PKU.
  • Breast-feeding may help maintain Phe levels within the normal range for PKU infants.
  • Further research is needed on managing breast-feeding for mothers with PKU infants.

Related Concept Videos

Inborn Errors of Metabolism01:20

Inborn Errors of Metabolism

Phenylketonuria (PKU) is a protein metabolism disorder characterized by high blood levels of the amino acid phenylalanine. This results from a mutation in the gene responsible for phenylalanine hydroxylase, an enzyme that converts phenylalanine into tyrosine. When this enzyme is deficient, phenylalanine builds up in the blood, leading to symptoms such as vomiting, rashes, seizures, growth deficiency, and severe mental retardation. An early diagnosis and a diet restricting phenylalanine intake...
Pharmacokinetics in Pediatric Patients: Drug Metabolism01:24

Pharmacokinetics in Pediatric Patients: Drug Metabolism

In pediatric care, understanding the nuances of hepatic drug metabolism is crucial, as it significantly differs from that of adults. This divergence is primarily due to the developmental stage of drug-metabolizing enzymes, which affects how medications are processed in the body. In neonates, for instance, the activity of Phase I enzymes—critical for the initial breakdown of drugs—is markedly reduced, functioning at just 20–40% of the levels seen in adults. This reduction poses a challenge in...
Pharmacokinetics in Pediatric Patients: Overview and Drug Absorption01:23

Pharmacokinetics in Pediatric Patients: Overview and Drug Absorption

Understanding the physiological differences in the pediatric population is crucial for effective pharmacotherapy. Neonates, infants, and children exhibit significant variations in gastric pH, gastric emptying time, intestinal transit time, and biliary function. These variations profoundly affect oral drug absorption, necessitating a nuanced approach to pediatric dosing.Neonates present with a unique physiological profile, having a gastric pH greater than 4 and faster and more irregular gastric...
Pharmacokinetics in Pediatric Patients: Drug Distribution01:17

Pharmacokinetics in Pediatric Patients: Drug Distribution

Drug distribution in the pediatric population exhibits unique challenges and considerations due to the physiological differences between children, particularly neonates and infants, and adults. A crucial aspect of pediatric pharmacology is understanding how these differences impact the pharmacokinetics of various drugs, necessitating age-specific dosing strategies to ensure efficacy and safety.Neonates and infants have a higher total body water content, ~75%–90% of their body weight, compared...
Pharmacokinetics in Pediatric Patients: Drug Excretion01:26

Pharmacokinetics in Pediatric Patients: Drug Excretion

In pediatric medicine, understanding the renal function and drug elimination nuances is crucial for administering safe and effective treatments. Newborns, in particular, display markedly slower renal functions than adults, profoundly affecting how drugs are cleared from their bodies. This slower drug clearance requires clinicians to extend the dosing intervals for many medications to prevent drug accumulation and toxicity while ensuring therapeutic efficacy.One key area where these adjustments...
Overview of Protein Metabolism01:21

Overview of Protein Metabolism

Proteins are broken down into amino acids during digestion. Unlike fats and carbohydrates, which are stored for later use, proteins are not. Instead, amino acids are either used to produce ATP through oxidation or contribute to the creation of new proteins for the growth and repair of the body. Any surplus amino acids from the diet are converted into glucose or triglycerides rather than excreted.
Amino acids play various roles in the body once they are absorbed into cells. They are restructured...