欧洲苯丙酮尿症患儿的早期喂养方式
Early feeding practices in infants with phenylketonuria across Europe.
作者信息
Pinto A, Adams S, Ahring K, Allen H, Almeida M F, Garcia-Arenas D, Arslan N, Assoun M, Atik Altınok Y, Barrio-Carreras D, Belanger Quintana A, Bernabei S M, Bontemps C, Boyle F, Bruni G, Bueno-Delgado M, Caine G, Carvalho R, Chrobot A, Chyż K, Cochrane B, Correia C, Corthouts K, Daly A, De Leo S, Desloovere A, De Meyer A, De Theux A, Didycz B, Dijsselhof M E, Dokoupil K, Drabik J, Dunlop C, Eberle-Pelloth W, Eftring K, Ekengren J, Errekalde I, Evans S, Foucart A, Fokkema L, François L, French M, Forssell E, Gingell C, Gonçalves C, Gökmen Özel H, Grimsley A, Gugelmo G, Gyüre E, Heller C, Hensler R, Jardim I, Joost C, Jörg-Streller M, Jouault C, Jung A, Kanthe M, Koç N, Kok I L, Kozanoğlu T, Kumru B, Lang F, Lang K, Liegeois I, Liguori A, Lilje R, Ļubina O, Manta-Vogli P, Mayr D, Meneses C, Newby C, Meyer U, Mexia S, Nicol C, Och U, Olivas S M, Pedrón-Giner C, Pereira R, Plutowska-Hoffmann K, Purves J, Re Dionigi A, Reinson K, Robert M, Robertson L, Rocha J C, Rohde C, Rosenbaum-Fabian S, Rossi A, Ruiz M, Saligova J, Gutiérrez-Sánchez A, Schlune A, Schulpis K, Serrano-Nieto J, Skarpalezou A, Skeath R, Slabbert A, Straczek K, Giżewska M, Terry A, Thom R, Tooke A, Tuokkola J, van Dam E, van den Hurk T A M, van der Ploeg E M C, Vande Kerckhove K, Van Driessche M, van Wegberg A M J, van Wyk K, Vasconcelos C, Velez García V, Wildgoose J, Winkler T, Żółkowska J, Zuvadelli J, MacDonald A
机构信息
Birmingham Women's and Children's Hospital, Birmingham, UK.
Royal Victoria Infirmary, Newcastle, UK.
出版信息
Mol Genet Metab Rep. 2018 Aug 8;16:82-89. doi: 10.1016/j.ymgmr.2018.07.008. eCollection 2018 Sep.
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 ( = 35/95), whereas 44% ( = 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.
背景
对于苯丙酮尿症（PKU）婴儿，饮食管理的基础是降低并调整母乳或标准婴儿配方奶粉中的苯丙氨酸（Phe）摄入量，并结合无苯丙氨酸婴儿配方奶粉，以将血苯丙氨酸水平维持在目标范围内。专业人员采用不同方法喂养苯丙酮尿症婴儿，我们的调查旨在记录欧洲各地的实际做法。
方法
我们向从事先天性代谢缺陷疾病（IMD）工作的欧洲卫生专业人员发送了一份横断面SurveyMonkey®问卷。问卷包含31个开放式和多项选择题。根据不同地理区域对结果进行分析。
结果
来自21个国家的95个中心做出了回应。超过60%的中心在婴儿10日龄时开始饮食管理，58%的中心在出生后第3天进行新生儿筛查。在诊断时，61%的代谢中心会让婴儿住院，主要集中在东欧、西欧和南欧。诊断后母乳喂养率急剧下降，6个月时只有30%的女性仍在母乳喂养。53%的中心在每次母乳喂养前提供预先计量的无苯丙氨酸婴儿配方奶粉，23%的中心将母乳喂养与无苯丙氨酸婴儿配方奶粉交替进行。对于标准婴儿配方奶粉喂养，37%的中心（n = 35/95）在按计量喂养后再用无苯丙氨酸婴儿配方奶粉喂至饱腹，而44%（n = 42/95）建议将两种配方奶粉混合。85%的中心在17至26周开始断奶，12%的中心在≥26周开始断奶，3%的中心在<17周开始断奶。
讨论
这是关于苯丙酮尿症婴儿喂养实践完成的最大规模的欧洲调查。很明显，欧洲各地的做法差异很大，而苯丙酮尿症婴儿喂养的实际问题在《2017年苯丙酮尿症欧洲指南》中很少受到关注。很少有报告比较不同喂养技术对血苯丙氨酸控制、苯丙氨酸波动和生长的影响。有必要进行对照前瞻性研究，以评估不同的婴儿喂养实践可能如何影响长期喂养发展。
Zotero 插件