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Nutrition and reproductive outcome in maternal phenylketonuria
K Michals1, P B Acosta, V Austin
1College of Health, Florida International University, Miami 33199, USA.
Insights
Maternal weight gain and nutrient intake significantly impact pregnancy outcomes in women with phenylketonuria (PKU). Optimal management requires considering these factors alongside phenylalanine (Phe) control.
Area of Science:
- Biochemistry
- Maternal-Fetal Medicine
- Nutritional Science
Background:
- Phenylketonuria (PKU) requires careful management during pregnancy.
- Elevated maternal plasma phenylalanine (Phe) concentrations can negatively affect fetal development.
- The Maternal PKU Collaborative Study investigated factors influencing reproductive outcomes.
Purpose of the Study:
- To correlate maternal weight gain and nutrient intake with plasma Phe levels and reproductive outcomes in pregnant women with PKU.
- To identify key nutritional and metabolic factors influencing fetal development and birth parameters.
Main Methods:
- Analysis of data from 150 and 142 subjects for plasma Phe concentrations and reproductive outcomes, respectively.
- Correlation of daily protein intake with plasma Phe levels.
- Assessment of maternal weight gain as a percentage of recommended gain.
- Evaluation of first-trimester nutrient intakes (fat, calcium, phosphorus, vitamin A, folate) in relation to reproductive success.
Main Results:
- Daily protein intake showed a negative correlation with maternal plasma Phe concentration.
- Longer time for maternal plasma Phe to normalize below 600 mumol/l negatively impacted offspring birth length, weight, and head circumference.
- Maternal weight gain percentage positively influenced offspring birth parameters.
- Higher maternal protein and energy intake correlated with increased birth weight and length.
- Women with good reproductive outcomes had significantly greater first-trimester intakes of fat, calcium, phosphorus, vitamin A, and folate compared to those with poor outcomes.
Conclusions:
- Maternal weight gain and specific nutrient intakes are crucial alongside plasma Phe control in managing pregnant women with PKU.
- Comprehensive management strategies should integrate dietary counseling and weight monitoring for optimal pregnancy outcomes in PKU.
- Nutritional interventions targeting protein, energy, fat, calcium, phosphorus, vitamin A, and folate are vital for successful PKU pregnancy management.
Abstract:
Maternal weight gain and intake of selected nutrients were correlated with plasma phenylalanine (Phe) concentrations and reproductive outcomes (in 150 and 142 subjects respectively) in the Maternal PKU Collaborative Study. Daily protein intake was negatively correlated with plasma Phe concentration. Birth length, weight and head circumference of offspring were negatively influenced by the length of time required for the maternal plasma Phe to decline below 600 mumol/l (10 mg/dl) and positively influenced by weight gain of mother as a percentage of recommended 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 significantly greater in women who had a good reproductive outcome than by women who had a poor outcome. In addition to plasma Phe control in maternal phenylketonuria (PKU), maternal weight gain and dietary intake of protein, energy and fat were correlated with outcome. Therefore, nutrient intake and maternal weight gain should be considered along with plasma Phe concentration when managing the therapy of a pregnant woman with PKU.