The Impact of the Quality of Nutrition and Lifestyle in the Reproductive Years of Women with PKU on the Long-Term Health of Their Children

Maria Inês Gama, Alex Pinto, Anne Daly, Júlio César Rocha, Anita Macdonald

Research output: Contribution to journalReview articlepeer-review

1 Downloads (Pure)

Abstract

A woman’s nutritional status before and during pregnancy can affect the health of her progeny. Phenylketonuria (PKU), a rare disorder causing high blood and brain phenylalanine (Phe) concentrations, is associated with neurocognitive disability. Lifelong treatment is mainly dietetic with a Phe-restricted diet, supplemented with a low-Phe protein substitute. Treatment adherence commonly decreases in adolescence, with some adults ceasing dietary treatment. In maternal PKU, elevated blood Phe is harmful to the fetus so a strict Phe-restricted diet must be re-established preconception, and this is particularly difficult to achieve. A woman’s reproductive years introduces an opportunity to adopt healthier behaviours to prepare for successful pregnancies and positive health outcomes for both themselves and their children. Several factors can influence the health status of women with PKU. Political, socioeconomic, and individual food and lifestyle choices affect diet quality, metabolic control, and epigenetics, which then pre-condition the overall maternal health and long-term health of the child. Here, we reflect on a comprehensive approach to treatment and introduce practical recommendations to optimize the wellbeing of women with PKU and the resultant health of their children.

Original languageEnglish
Article number1021
JournalNutrients
Volume14
Issue number5
DOIs
Publication statusPublished - 1 Mar 2022

Keywords

  • Adherence
  • Epigenetics
  • Health
  • Phenylketonuria
  • Preconception
  • Women

Fingerprint

Dive into the research topics of 'The Impact of the Quality of Nutrition and Lifestyle in the Reproductive Years of Women with PKU on the Long-Term Health of Their Children'. Together they form a unique fingerprint.

Cite this