+ Site Statistics
References:
54,258,434
Abstracts:
29,560,870
PMIDs:
28,072,757
+ Search Articles
+ PDF Full Text Service
How our service works
Request PDF Full Text
+ Follow Us
Follow on Facebook
Follow on Twitter
Follow on LinkedIn
+ Subscribe to Site Feeds
Most Shared
PDF Full Text
+ Translate
+ Recently Requested

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



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



American Journal of Clinical Nutrition 73(4): 792-796



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 mmol/L by 10 wk gestation and 120-360 mmol/L throughout the remainder of pregnancy; group 2 (n = 46), <600 mmol/L but not <360 mmol/L by 10 wk gestation and 120-600 mmol/L throughout the remainder of pregnancy; group 3 (n = 24), <600 mmol/L by 10 wk gestation but >600 mmol/L at least once thereafter; group 4 (n = 147), never <600 mmol/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 mmol/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. Am J Clin Nutr 2001;73:792-6. Reprinted by permission of the publisher.

Please choose payment method:






(PDF emailed within 1 workday: $29.90)

Accession: 003480130

Download citation: RISBibTeXText

PMID: 11273855


Related references

Intake of major nutrients by women in the Maternal Phenylketonuria (Mpku) Study and effects on plasma phenylalanine concentrations. The American Journal of Clinical Nutrition 73(4): 792-796, 2001

Fetal damage due to maternal phenylketonuria: effects of dietary treatment and maternal phenylalanine concentrations around the time of conception (an interim report from the UK Phenylketonuria Register). Journal of Inherited Metabolic Disease 13(4): 651-657, 1990

Tissue amino acids and organic acids in the maternal phenylketonuria mpku fetus implications for the pathogenesis of fetal damage in mpku. American Journal of Human Genetics 43(3 SUPPL): A12, 1988

Congenital heart disease in maternal phenylketonuria: effects of blood phenylalanine and nutrient intake. Mental Retardation and Developmental Disabilities Research Reviews 5(2): 122-124, 1999

Maternal Phenylketonuria (MPKU). Canadian Family Physician Medecin de Famille Canadien 32: 2253-2258, 1986

The regulation of phenylalanine hydroxylase in rat tissues in vivo. The maintenance of high plasma phenylalanine concentrations in suckling rats: a model for phenylketonuria. Biochemical Journal 154(3): 613-618, 1976

Experimental maternal phenylketonuria in the rat enduring behavioral effects from in utero exposure to l phenylalanine and p chloro dl phenylalanine. Society for Neuroscience Abstracts 10(2): 956, 1984

Maternal phenylketonuria in two Sicilian families identified by maternal blood phenylalanine level screening and identification of a new phenylalanine hydroxylase gene mutation (P407L). European Journal of Pediatrics 158(1): 83-84, 1999

Plasma phenylalanine is associated with decreased serum ubiquinone-10 concentrations in phenylketonuria. Journal of Inherited Metabolic Disease 24(3): 359-366, 2001

Biochemical and neuropsychological effects of elevated plasma phenylalanine in patients with treated phenylketonuria. A model for the study of phenylalanine and brain function in man. Journal of Clinical Investigation 75(1): 40-48, 1985

Improved model of maternal phenylketonuria in rats by use of lower dietary concentrations of a-methylphenylalanine and L-phenylalanine in a semipurified diet. Nutrition Research 18(6): 9-1002, 1998

Plasma phenylalanine concentrations are associated with hepatic iron content in a murine model for phenylketonuria. Molecular Genetics and Metabolism 82(1): 76-82, 2004

Improved model of maternal phenylketonuria in rats by use of lower dietary concentrations of alpha-methylphenylalanine and L-phenylalanine in a semipurified diet. Nutrition Research 18(6): 989-1002, 1998

Pregnancy in phenylketonuria: dietary treatment aimed at normalising maternal plasma phenylalanine concentration. Archives of Disease in Childhood 66(11): 1346-1349, 1991

Elevation of plasma phenylalanine levels during pregnancies of women heterozygous for phenylketonuria. Journal of Pediatrics 63(2): 283-289, 1963