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ORIGINAL RESEARCH article

Front. Nutr.
Sec. Clinical Nutrition
Volume 11 - 2024 | doi: 10.3389/fnut.2024.1507464
This article is part of the Research Topic Innovative Approaches to Nutrition Counseling in Pediatric Dietetics - Guidelines, Practices, and Future Directions View all 4 articles

Transitioning of Protein Substitutes in Patients with Phenylketonuria: A Pilot Study

Provisionally accepted
Ozlem Yilmaz Nas Ozlem Yilmaz Nas 1,2,3*Catherine Ashmore Catherine Ashmore 2Sharon Evans Sharon Evans 2Alex Pinto Alex Pinto 2Anne Daly Anne Daly 2Nurcan Yabancı Ayhan Nurcan Yabancı Ayhan 3Anita MacDonald Anita MacDonald 2
  • 1 Ankara Yıldırım Beyazıt University, Ankara, Türkiye
  • 2 Birmingham Children's Hospital, Birmingham, England, United Kingdom
  • 3 Ankara University, Ankara, Ankara, Türkiye

The final, formatted version of the article will be published soon.

    Introduction: In phenylketonuria (PKU), there is limited information about transitioning between protein substitutes (PS) and the influencing factors, particularly in young children. This pilot study assessed the stepwise transition from second to third-stage protein substitutes in children with phenylketonuria, aged 3-5 years. Methods: Demographics, child behaviour, maternal anxiety, and food neophobia scores were collected at baseline, mid-transition, and final assessment. Blood phenylalanine (Phe) was collected from 6 months pre-baseline to post-final assessment. Results: Twelve children (n=4 males, 33%, median age 3.2 years) participated. Sixty-seven percent (n=8) transitioned to liquid amino acid-based protein substitute and 33% (n=4) to glycomacropeptide (cGMP) powder. Forty-two percent (n=5/12) had a smooth transition (Group 1, median 3.5 months), while the remaining faced difficulty (n=3, 25%, Group 2), or failed full transition (n=4, 33%, Group 3). In Groups 2 and 3, caregivers failed to follow instructions, demonstrating inconsistencies and child resistance. Group 2 children had significantly higher blood Phe levels (above 360 μmol/L), that was significantly higher than Groups 1 and 3 (p<0.01), with Groups 1 and 3 maintaining blood Phe within target (p<0.01). Higher maternal education and nursery/school attendance significantly influenced transition success (p<0.05). No significant differences were found in child neophobia, maternal anxiety, or child behaviour (p>0.05). Mothers generally reported satisfaction with the stepwise transition process. Conclusion: A stepwise transition to third-stage protein substitutes in PKU is effective, but is dependent on child metabolic control, parental education and nursery/school support.

    Keywords: transition1, protein substitute2, liquid3, powder4, ready-to-drink5, stepwise6, guidance7

    Received: 07 Oct 2024; Accepted: 23 Dec 2024.

    Copyright: © 2024 Yilmaz Nas, Ashmore, Evans, Pinto, Daly, Yabancı Ayhan and MacDonald. This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY). The use, distribution or reproduction in other forums is permitted, provided the original author(s) or licensor are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with these terms.

    * Correspondence: Ozlem Yilmaz Nas, Ankara Yıldırım Beyazıt University, Ankara, Türkiye

    Disclaimer: All claims expressed in this article are solely those of the authors and do not necessarily represent those of their affiliated organizations, or those of the publisher, the editors and the reviewers. Any product that may be evaluated in this article or claim that may be made by its manufacturer is not guaranteed or endorsed by the publisher.