NUTRITION AND REPRODUCTIVE OUTCOME IN MATERNAL PHENYLKETONURIA

Maternal weight gain and intake of selected nutrients were correlated with plasma phenylalanine (Phe) concentrations and reproductive outcom es (in 150 and 142 subjects respectively) in the Maternal PKU Collabor ative Study. Daily protein intake was negatively correlated with plasm a Phe concentration. Birth length, weight and head circumference of of fspring were negatively influenced by the length of time required for the maternal plasma Phe to decline below 600 mu mol/l (10 mg/dl) and p ositively influenced by weight gain of mother as a percentage of recom mended weight gain. Birth weight and length were positively correlated with maternal protein and energy intakes. During the first trimester, intakes of fat, calcium, phosphorus, vitamin A and folate were signif icantly greater in women who had a good reproductive outcome than by w omen who had a poor outcome. In addition to plasma Phe control in mate rnal phenylketonuria (PKU), maternal weight gain and dietary intake of protein, energy and fat were correlated with outcome. Therefore, nutr ient intake and maternal weight gain should be considered along with p lasma Phe concentration when managing the therapy of a pregnant woman with PKU.