NHD Issue 148 New study into weaning and PKU

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NEW STUDY INTO WEANING AND PKU

RESEARCH

Does the introduction of a second-stage weaning protein substitute affect growth in infants with PKU? A recent study by Sharon Evans, Anne Daly and Anita MacDonald et al1, looks into growth, energy and protein intake. In infants with PKU, introducing a second-stage semi-solid weaning protein substitute (WPS) is common, but there is concern that this may not meet energy requirements. A case-control study1 conducted earlier this year, has looked at growth in children with PKU who take a weaning protein substitute up to the age of two years. Protein and energy intake were also studied. Weaning in PKU is particularly complex and challenging, as solid foods can suppress the appetite for liquids, potentially lowering intake of infant protein substitute, thereby affecting the ability to achieve total protein requirements. As a consequence, it often becomes necessary to introduce a second stage, more concentrated protein substitute2 at around six months of age. Feeding problems are known to be more common in young children with PKU compared with those without PKU3 and so the weaning period may be a particularly vulnerable time for achieving optimal growth. Previous limited retrospective data has demonstrated that a weaning protein substitute can be introduced without adverse effects on appetite or growth.4 However, no studies until now have prospectively looked at growth, protein and energy intake of children during the weaning period and early years. There is concern that the low energy density of weaning protein substitute (8kcal/g protein) might not compensate for the energy content of infant protein substitute (33.5kcal/g protein) and this may impact on growth and weight gain. This longitudinal, prospective study1 looked at 20 children with PKU who were transitioning to a WPS, and 20 non-PKU controls, Subjects were recruited to assess their growth, energy and macronutrient (protein, fat and carbohydrate) intake from the introduction of a second stage, low volume, Phe-free protein substitute (three to six months of age) to the age of two years (total of 17 to 20 months follow-up), observing them monthly from weaning commencement (four to six months) to 12 months and at 15, 18 and 24 months of age for: weight, length, head circumference, BMI, energy and macronutrient intake.

The weaning protein substitute (PKU Anamix First Spoon) was a powdered Phefree protein substitute supplemented with long-chain polyunsaturated fatty acids (LCPs), containing essential and non-essential amino acids, carbohydrate, fat, vitamins, minerals and trace elements. The product was mixed with water to produce a semi-solid spoonable protein substitute and 5g of protein substitute powder provided 2g of protein and 16 kcal. RESULTS

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 two years. Total protein and energy intake in both groups were similar at all ages; however, from 12 to 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. CONCLUSION

Normal growth was observed in the group of children with PKU, who were weaned onto a second-stage semi-solid protein substitute, compared with their non-PKU peers. The study concluded that further studies into longitudinal growth, body composition, energy/nutrient intakes in early childhood and adolescence would be beneficial in establishing any changing trends that may affect long-term health outcomes. For references please visit the Subscriber zone at NHDmag.com/references.

www.NHDmag.com October 2019 - Issue 148

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PKU (Phenylketonuria). GMP (Glycomacropeptide). PE (protein equivalent). Phe (Phenylalanine). # Minimum 6 months in children aged between 5 and 16 years. 1 Data on file / SSIEM abstract 2016. PKU sphere™ is a food for special medical purposes and must be used under medical supervision. For the dietary management of PKU. *PKU sphere chocolate contains 2.8g / 20g PE and 2.2g / 15g PE. Contains: milk, soya, fish.


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