Weaning practices in phenylketonuria vary between health professionals in Europe.
Infant
Phe-free infant formula
Phenylalanine
Phenylketonuria
Weaning
Journal
Molecular genetics and metabolism reports
ISSN: 2214-4269
Titre abrégé: Mol Genet Metab Rep
Pays: United States
ID NLM: 101624422
Informations de publication
Date de publication:
Mar 2019
Mar 2019
Historique:
received:
12
10
2018
revised:
14
11
2018
accepted:
14
11
2018
entrez:
2
2
2019
pubmed:
2
2
2019
medline:
2
2
2019
Statut:
epublish
Résumé
In phenylketonuria (PKU), weaning is considered more challenging when compared to feeding healthy infants. The primary aim of weaning is to gradually replace natural protein from breast milk or standard infant formula with solids containing equivalent phenylalanine (Phe). In addition, a Phe-free second stage L-amino acid supplement is usually recommended from around 6 months to replace Phe-free infant formula. Our aim was to assess different weaning approaches used by health professionals across Europe. A cross sectional questionnaire (survey monkey®) composed of 31 multiple and single choice questions was sent to European colleagues caring for inherited metabolic disorders (IMD). Centres were grouped into geographical regions for analysis. Weaning started at 17-26 weeks in 85% ( Weaning strategies vary throughout European PKU centres. There is evidence to suggest that different infant weaning strategies may influence longer term adherence to the PKU diet or acceptance of Phe-free L-amino acid supplements; rendering prospective long-term studies important. It is essential to identify an effective weaning strategy that reduces caregiver burden but is associated with acceptable dietary adherence and optimal infant feeding development.
Sections du résumé
BACKGROUND
BACKGROUND
In phenylketonuria (PKU), weaning is considered more challenging when compared to feeding healthy infants. The primary aim of weaning is to gradually replace natural protein from breast milk or standard infant formula with solids containing equivalent phenylalanine (Phe). In addition, a Phe-free second stage L-amino acid supplement is usually recommended from around 6 months to replace Phe-free infant formula. Our aim was to assess different weaning approaches used by health professionals across Europe.
METHODS
METHODS
A cross sectional questionnaire (survey monkey®) composed of 31 multiple and single choice questions was sent to European colleagues caring for inherited metabolic disorders (IMD). Centres were grouped into geographical regions for analysis.
RESULTS
RESULTS
Weaning started at 17-26 weeks in 85% (
CONCLUSIONS
CONCLUSIONS
Weaning strategies vary throughout European PKU centres. There is evidence to suggest that different infant weaning strategies may influence longer term adherence to the PKU diet or acceptance of Phe-free L-amino acid supplements; rendering prospective long-term studies important. It is essential to identify an effective weaning strategy that reduces caregiver burden but is associated with acceptable dietary adherence and optimal infant feeding development.
Identifiants
pubmed: 30705824
doi: 10.1016/j.ymgmr.2018.11.003
pii: S2214-4269(18)30115-0
pmc: PMC6349955
doi:
Types de publication
Journal Article
Langues
eng
Pagination
39-44Références
J Inherit Metab Dis. 2003;26(4):327-38
pubmed: 12971420
J Hum Nutr Diet. 2006 Jun;19(3):229-36
pubmed: 16756538
Ann Nutr Metab. 2007;51(4):352-8
pubmed: 17726313
J Pediatr Gastroenterol Nutr. 2008 Jan;46(1):99-110
pubmed: 18162844
J Hum Nutr Diet. 2008 Aug;21(4):351-8
pubmed: 18721402
Mol Genet Metab. 2010 Aug;100(4):303-8
pubmed: 20466571
Eur J Clin Nutr. 2011 Feb;65(2):275-8
pubmed: 21119696
Ann Nutr Metab. 2011;58(2):94-100
pubmed: 21474926
J Hum Nutr Diet. 2012 Apr;25(2):103-10
pubmed: 21819461
Eur J Clin Nutr. 2012 May;66(5):633-8
pubmed: 22318648
Mol Genet Metab. 2012 Jul;106(3):264-8
pubmed: 22607939
Mol Genet Metab. 2013 Jul;109(3):237-42
pubmed: 23731533
Eur J Clin Nutr. 2014 Mar;68(3):401-3
pubmed: 24398645
Mol Genet Metab Rep. 2016 Aug 28;9:1-5
pubmed: 27622144
Mol Genet Metab Rep. 2015 Oct 22;5:36-41
pubmed: 28649540
J Hum Nutr Diet. 2018 Jun;31(3):349-356
pubmed: 28940742
Mol Genet Metab Rep. 2017 Oct 03;13:83-89
pubmed: 29021961
Orphanet J Rare Dis. 2017 Oct 12;12(1):162
pubmed: 29025426
J Nutr Metab. 2017;2017:4083293
pubmed: 29057118
Orphanet J Rare Dis. 2018 Jan 25;13(1):21
pubmed: 29370874
Pediatric Health Med Ther. 2016 Dec 01;7:155-163
pubmed: 29388626
Cad Saude Publica. 2018 Feb 19;34(2):e00202816
pubmed: 29489953
Acta Paediatr Suppl. 1994 Dec;407:73-4
pubmed: 7766964