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JAMA. 2002;287:1258-1259.

	Since this article does not have an abstract, we have provided the first 150 words of the full text and any section headings.

MMWR. 2002;51:117-120

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Newborns in the United States are screened for phenylketonuria (PKU), a metabolic disorder that when left untreated is characterized by elevated blood phenylalanine (phe) levels and severe mental retardation (MR). An estimated 3,000-4,000 U.S.-born women of reproductive age with PKU have not gotten severe MR because as newborns their diets were severely restricted in the intake of protein-containing foods and were supplemented with medical foods (e.g., amino acid-modified formula and modified low-protein foods).^1-4 When women with PKU do not adhere to their diet before and during pregnancy, infants born to them have a 93% risk for MR and a 72% risk for microcephaly.^5-6 These risks result from the toxic effects of high maternal blood phe levels during pregnancy, not because the infant has PKU.^5-6 The restricted diet, which should be maintained for life, often is discontinued during adolescence.^5-10 This report describes the pregnancies of three . . . [Full Text of this Article]

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