https://www.ncbi.nlm.nih.gov/pubmed/30101073

Metabolic liver disease

Mol Genet Metab Rep. 2018 Aug 8;16:82-89. doi: 10.1016/j.ymgmr.2018.07.008. eCollection 2018 Sep.
Early feeding practices in infants with phenylketonuria across Europe.

Pinto A1, Adams S2, Ahring K3, Allen H4, Almeida MF5,6,7, Garcia-Arenas D8, Arslan N9, Assoun M10, Atik Altınok Y11, Barrio-Carreras D12, Belanger Quintana A13, Bernabei SM14, Bontemps C15, Boyle F16, Bruni G17, Bueno-Delgado M18, Caine G19, Carvalho R20, Chrobot A21, Chyż K22, Cochrane B23, Correia C24, Corthouts K25, Daly A1, De Leo S26, Desloovere A27, De Meyer A28, De Theux A29, Didycz B30, Dijsselhof ME31, Dokoupil K32, Drabik J33, Dunlop C34, Eberle-Pelloth W35, Eftring K36, Ekengren J36, Errekalde I37, Evans S1, Foucart A38, Fokkema L39, François L40, French M41, Forssell E42, Gingell C43, Gonçalves C44, Gökmen Özel H45, Grimsley A46, Gugelmo G47, Gyüre E48, Heller C49, Hensler R50, Jardim I51, Joost C52, Jörg-Streller M53, Jouault C54, Jung A55, Kanthe M56, Koç N57, Kok IL39, Kozanoğlu T58, Kumru B59, Lang F60, Lang K61, Liegeois I62, Liguori A14, Lilje R63, Ļubina O64, Manta-Vogli P65, Mayr D66, Meneses C67, Newby C68, Meyer U69, Mexia S51, Nicol C2, Och U70, Olivas SM8, Pedrón-Giner C71, Pereira R72, Plutowska-Hoffmann K73, Purves J34, Re Dionigi A74, Reinson K75, Robert M76, Robertson L34, Rocha JC5,7,77,78, Rohde C79, Rosenbaum-Fabian S80, Rossi A81, Ruiz M82, Saligova J83, Gutiérrez-Sánchez A8, Schlune A84, Schulpis K65, Serrano-Nieto J85, Skarpalezou A86, Skeath R87, Slabbert A88, Straczek K89, Giżewska M89, Terry A90, Thom R46, Tooke A43, Tuokkola J91, van Dam E92, van den Hurk TAM39, van der Ploeg EMC93, Vande Kerckhove K25, Van Driessche M27, van Wegberg AMJ94, van Wyk K95, Vasconcelos C96, Velez García V97, Wildgoose J98, Winkler T99, Żółkowska J22, Zuvadelli J74, MacDonald A1.

Abstract

BACKGROUND:
In infants with phenylketonuria (PKU), dietary management is based on lowering and titrating phenylalanine (Phe) intake from breast milk or standard infant formula in combination with a Phe-free infant formula in order to maintain blood Phe levels within target range. Professionals use different methods to feed infants with PKU and our survey aimed to document practices across Europe.

METHODS:
We sent a cross sectional, survey monkey® questionnaire to European health professionals working in IMD. It contained 31 open and multiple-choice questions. The results were analysed according to different geographical regions.

RESULTS:
Ninety-five centres from 21 countries responded. Over 60% of centres commenced diet in infants by age 10 days, with 58% of centres implementing newborn screening by day 3 post birth. At diagnosis, infant hospital admission occurred in 61% of metabolic centres, mainly in Eastern, Western and Southern Europe. Breastfeeding fell sharply following diagnosis with only 30% of women still breast feeding at 6 months.53% of centres gave pre-measured Phe-free infant formula before each breast feed and 23% alternated breast feeds with Phe-free infant formula. With standard infant formula feeds, measured amounts were followed by Phe-free infant formula to satiety in 37% of centres (n = 35/95), whereas 44% (n = 42/95) advised mixing both formulas together. Weaning commenced between 17 and 26 weeks in 85% centres, ≥26 weeks in 12% and < 17 weeks in 3%.

DISCUSSION:
This is the largest European survey completed on PKU infant feeding practices. It is evident that practices varied widely across Europe, and the practicalities of infant feeding in PKU received little focus in the PKU European Guidelines (2017). There are few reports comparing different feeding techniques with blood Phe control, Phe fluctuations and growth. Controlled prospective studies are necessary to assess how different infant feeding practices may influence longer term feeding development.

Published on: 
Aug-2018

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