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Nutrients 2019, 11(3), 552; https://doi.org/10.3390/nu11030552

Growth, Protein and Energy Intake in Children with PKU Taking a Weaning Protein Substitute in the First Two Years of Life: A Case-Control Study

1
Birmingham Women’s and Children’s Hospital NHS Foundation Trust, Birmingham B4 6NH, UK
2
Bradford Teaching Hospitals NHS Trust, Bradford BD9 6RJ, UK
3
Royal Hospital for Children Glasgow G51 4TF, UK
4
Danone Early Life Nutrition, Macquarie Park, New South Wales, Australia
*
Author to whom correspondence should be addressed.
Received: 21 December 2018 / Revised: 20 February 2019 / Accepted: 27 February 2019 / Published: 5 March 2019
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Abstract

Growth issues have been observed in young children with phenylketonuria (PKU), but studies are conflicting. In infancy, there is an increasing trend to introduce a second-stage semi-solid weaning protein substitute (WPS) but there is concern that this may not meet energy requirements. In this longitudinal, prospective study, 20 children with PKU transitioning to a WPS, and 20 non-PKU controls were observed monthly from weaning commencement (4–6 months) to 12 m and at 15, 18 and 24 months of age for: weight, length, head circumference, body mass index (BMI), energy and macronutrient intake. Growth parameters were within normal range at all ages in both groups with no significant difference in mean z-scores except for accelerated length in the PKU group. No child with PKU had z-scores < −2 for any growth parameter at age 2 years. Total protein and energy intake in both groups were similar at all ages; however, from 12–24 months in the PKU group, the percentage of energy intake from carbohydrate increased (60%) but from fat decreased (25%) and inversely for controls (48% and 36%). In PKU, use of low volume WPS meets Phe-free protein requirements, facilitates transition to solid foods and supports normal growth. Further longitudinal study of growth, body composition and energy/nutrient intakes in early childhood are required to identify any changing trends. View Full-Text
Keywords: Phenylketonuria (PKU); growth; protein substitute; weaning Phenylketonuria (PKU); growth; protein substitute; weaning
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Evans, S.; Daly, A.; Wildgoose, J.; Cochrane, B.; Chahal, S.; Ashmore, C.; Loveridge, N.; MacDonald, A. Growth, Protein and Energy Intake in Children with PKU Taking a Weaning Protein Substitute in the First Two Years of Life: A Case-Control Study. Nutrients 2019, 11, 552.

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