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Intake of major nutrients by women in the Maternal Phenylketonuria (MPKU) Study and effects on plasma phenylalanine concentrations^1,2,3

¹ From Medical and Regulatory Affairs, Ross Products Division, Abbott Laboratories, Columbus, OH; the Department of Human Development, the University of Houston; the Department of Pediatrics, the University of Texas Medical Branch, Galveston; the Division of Genetics, Boston Children's Hospital; the Division of Medical Genetics, Children's Hospital of Los Angeles; the Department of Pediatrics and Genetics, Hospital for Sick Children, Toronto; and the General Clinical Research Center, School of Medicine, University of Southern California, Los Angeles.

Background: Women with untreated phenylketonuria (PKU) often have poor reproductive outcomes.

Objective: We assessed the effects of intakes of major nutrients on plasma phenylalanine concentrations and we measured phenylalanine hydroxylase activity and phenylalanine intakes in pregnant women with PKU.

Design: Dietary intakes and plasma phenylalanine concentrations were compared in 4 subject groups defined on the basis of plasma phenylalanine concentrations: group 1 (n = 23), <360 µmol/L by 10 wk gestation and 120–360 µmol/L throughout the remainder of pregnancy; group 2 (n = 46), <600 µmol/L but not <360 µmol/L by 10 wk gestation and 120–600 µmol/L throughout the remainder of pregnancy; group 3 (n = 24), <600 µmol/L by 10 wk gestation but >600 µmol/L at least once thereafter; group 4 (n = 147), never <600 µmol/L.

Results: Except in the first trimester, mean intakes of phenylalanine, energy, and fat tended to be greater in group 1 than in the other groups. The mean protein intake of group 1 tended to be greater than that of the other groups. Intakes of protein (P < 0.0001), fat (P < 0.0001), and energy (P < 0.007) were negatively correlated with maternal plasma phenylalanine concentrations. It appeared that genotype did not affect phenylalanine tolerance.

Conclusions: Maternal genotype appeared to have little influence on phenylalanine requirements during the first trimester. Early decline and maintenance of maternal plasma phenylalanine concentrations at <360 µmol/L and mean protein intake greater than the recommended dietary allowance (RDA) with mean energy intake near the RDA resulted in the best reproductive outcomes. Inadequate intakes of protein, fat, and energy may result in elevated plasma phenylalanine concentrations and may contribute to poor reproductive outcomes.

Key Words: Maternal Phenylketonuria Study • phenylketonuria • PKU • genotype • plasma phenylalanine • protein • fat • energy • reproductive outcome • phenylalanine hydroxylase • pregnancy • pregnant women • birth outcome

This article has been cited by other articles:

				K. M. Matalon, P. B. Acosta, and C. Azen Role of Nutrition in Pregnancy With Phenylketonuria and Birth Defects Pediatrics, December 1, 2003; 112(6): 1534 - 1536. [Abstract] [Full Text] [PDF]