Recommended dietary allowances for pregnant women affected with phenylketonuria.

Prevention of embryopathy due to maternal phenylketonuria is possible thank s to a maternal-specific low-phenylalanine diet, which has to be started be fore conception and followed during the whole gestation. The setup of this diet implies knowing the recommended dietary allowances for normal pregnant women as well as for women with nutritional deficiencies. Women with pheny lketonuria must be considered at risk for nutritional imbalance for two mai n reasons. First, most adult women with phenylketonuria have been on a vege tarian diet for many years without protein substitutes or medical control. Secondly, the strict diet for pregnant women with phenylketonuria may induc e anorexia or nutritional deficits if it is not well tolerated or understoo d. Protein, iron, calcium, selenium, vitamin B 12 and caloric intakes are t he most sensitive parameters. Close cooperation with an experienced medical and dietician team is required. (C) 2001 Editions scientifiques et medical es Elsevier SAS.