Maternal PKU

For women with PKU who intend to become pregnant, following a strict phenylalanine-restricted diet and controlling blood phenylalanine to 120-360moll-1 is critical to offspring health. Women with PKU who have high blood phenyl-alanine levels are at high risk of having children with microcephaly, mental retardation, low birth weight, and congenital heart anomalies. In an International Study of Maternal PKU, women who had good metabolic control by 10 weeks' gestation had babies with good birth outcomes and development. In women with poor control, the degree of microcephaly and mental retardation was proportional to the level of blood phenylalanine. Congenital heart disease, on the other hand, was not directly related to the degree of metabolic control, suggesting that etiology is multifactorial, although in this study, no serious heart defects occurred when mothers were in good metabolic control by 10 weeks' gestation. The recommendation is for women to be on the diet for PKU and in good metabolic control before conceiving in order to prevent damage to the fetus. Nevertheless, many women come to medical attention during pregnancy, indicating the need for better strategies for keeping women on the diet for life or helping them return to the diet before pregnancy. While blood phenyl-alanine during pregnancy was the best predictor of outcome in maternal PKU in the Collaborative Study, other nutritional factors, including sufficient energy, protein, vitamin B12 and fat, also played an important role.

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