Abstract
Weaning is complex for children with phenylketonuria (PKU). Breastmilk/infant formula and phenylalanine (Phe)-free infant protein-substitute (PS) are gradually replaced with equivalent amounts of Phe-containing food, a semi-solid/spoonable weaning PS and special low-protein foods. In PKU, feeding patterns/practices during weaning in PKU have not been formally evaluated. In this longitudinal, prospective, case-control study (n = 20) infants with PKU transitioning to a second-stage PS, were recruited at weaning (4â6 months) for a comparison of feeding practices and development with non-PKU infants. Subjects were monitored monthly to 12 months and at age 15 months, 18 months and 24 months for: feeding progression; food textures; motor skill development and self-feeding; feeding environment; gastrointestinal symptoms; and negative feeding behaviours. Children with PKU had comparable weaning progression to non-PKU infants including texture acceptance, infant formula volume and self-feeding skills. However, children with PKU had more prolonged Phe-free infant formula bottle-feeding and parental spoon feeding than controls; fewer meals/snacks per day; and experienced more flatulence (p = 0.0005), burping (p = 0.001), retching (p = 0.03); and less regurgitation (p = 0.003). Negative behaviours associated with PS at age 10â18 months, coincided with the age of teething. Use of semi-solid PS in PKU supports normal weaning development/progression but parents require support to manage the complexity of feeding and to normalise the social inclusivity of their childâs family food environment. Further study regarding parental anxiety associated with mealtimes is required.
Highlights
The dietary management of the inherited disorder of protein metabolism, phenylketonuria (PKU), in infancy and childhood, is a critical component of care, and treatment is recommended to commence prior to 10 days of age [1]
Intake, self-feeding skills, the feeding environment and gastrointestinal symptoms such as diarrhoea, There may be a critical period between 6â7 months when infants are developmentally ready for vomiting, constipation and colic were similar across the two groups and where differences did occur, the introduction of more textured foods, and delaying this beyond 10 months of age may increase the most had resolved by 2refusal, yearsfussy of age
Weaning development and progression in children with PKU is comparable to that of control children, despite the additional changes required in terms of introducing protein exchange foods, and weaning protein substitute
Summary
The dietary management of the inherited disorder of protein metabolism, phenylketonuria (PKU), in infancy and childhood, is a critical component of care, and treatment is recommended to commence prior to 10 days of age [1]. The feeding of young children with PKU is complex, with most children requiring a severe to moderate natural protein restriction (commonly < 10 g/day), in order to control blood phenylalanine (Phe) levels within target ranges and prevent severe intellectual impairment [2]. The diet is supplemented with a Phe-free protein substitute formula to meet energy and non-Phe protein requirements. Nutrients 2019, 11, 529 equivalent, and natural protein to individual tolerance in PKU is important for the promotion of normal growth and development and maintaining acceptable Phe control. Weaning in PKU should reflect the weaning process of healthy non-PKU infants [3], aiming to transition from an exclusively liquid diet, to a mixed diet encompassing a wide variety of foods, tastes and textures; and encouraging development of independent self-feeding
Sign-in/Register to view highlights & summary Sign-in/Register to access full text options 
Talk to us
Join us for a 30 min session where you can share your feedback and ask us any queries you have
Schedule a callDisclaimer: All third-party content on this website/platform is and will remain the property of their respective owners and is provided on "as is" basis without any warranties, express or implied. Use of third-party content does not indicate any affiliation, sponsorship with or endorsement by them. Any references to third-party content is to identify the corresponding services and shall be considered fair use under The CopyrightLaw.
