Early feeding practices in infants with phenylketonuria across Europe

A. Pinto; S. Adams; K. Ahring; H. Allen; M. F. Almeida; D. Garcia-Arenas; N. Arslan; M. Assoun; Y. Atik Altinok; D. Barrio-Carreras; A. Belanger Quintana; S. M. Bernabei; C. Bontemps; F. Boyle; G. Bruni; M. Bueno-Delgado; G. Caine; R. Carvalho; A. Chrobot; K. Chyz; B. Cochrane; C. Correia; K. Corthouts; A. Daly; S. De Leo; A. Desloovere; A. De Meyer; A. De Theux; B. Didycz; M. E. Dijsselhof; K. Dokoupil; J. Drabik; C. Dunlop; W. Eberle-Pelloth; K. Eftring; J. Ekengren; I. Errekalde; S. Evans; A. Foucart; L. Fokkema; L. Francois; M. French; E. Forssell; I. L. Kok; M. Robert; E. van Dam; T. A. M. van den Hurk; E. M. C. van der Ploeg; A. M. J. van Wegberg; A. MacDonald

doi:10.1016/j.ymgmr.2018.07.008

Early feeding practices in infants with phenylketonuria across Europe

A. Pinto^*, S. Adams, K. Ahring, H. Allen, M. F. Almeida, D. Garcia-Arenas, N. Arslan, M. Assoun, Y. Atik Altinok, D. Barrio-Carreras, A. Belanger Quintana, S. M. Bernabei, C. Bontemps, F. Boyle, G. Bruni, M. Bueno-Delgado, G. Caine, R. Carvalho, A. Chrobot, K. ChyzB. Cochrane, C. Correia, K. Corthouts, A. Daly, S. De Leo, A. Desloovere, A. De Meyer, A. De Theux, B. Didycz, M. E. Dijsselhof, K. Dokoupil, J. Drabik, C. Dunlop, W. Eberle-Pelloth, K. Eftring, J. Ekengren, I. Errekalde, S. Evans, A. Foucart, L. Fokkema, L. Francois, M. French, E. Forssell, I. L. Kok, M. Robert, E. van Dam, T. A. M. van den Hurk, E. M. C. van der Ploeg, A. M. J. van Wegberg, A. MacDonald

^*Corresponding author for this work

Research output: Contribution to journal › Article › Academic › peer-review

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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 (R) 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.

Original language	English
Pages (from-to)	82-89
Number of pages	8
Journal	Molecular genetics and metabolism reports
Volume	16
DOIs	https://doi.org/10.1016/j.ymgmr.2018.07.008
Publication status	Published - Sept-2018

Keywords

Breastfeeding
Infant practices
Phenylketonuria
Phenylalanine
Phe-free infant formula
DIETARY-MANAGEMENT
PROTEIN
GROWTH

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Pinto, A., Adams, S., Ahring, K., Allen, H., Almeida, M. F., Garcia-Arenas, D., Arslan, N., Assoun, M., Altinok, Y. A., Barrio-Carreras, D., Belanger Quintana, A., Bernabei, S. M., Bontemps, C., Boyle, F., Bruni, G., Bueno-Delgado, M., Caine, G., Carvalho, R., Chrobot, A., ... MacDonald, A. (2018). Early feeding practices in infants with phenylketonuria across Europe. Molecular genetics and metabolism reports, 16, 82-89. https://doi.org/10.1016/j.ymgmr.2018.07.008

@article{01ce1c455a1144e9a992e32b414d69b2,

title = "Early feeding practices in infants with phenylketonuria across Europe",

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 (R) 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.",

keywords = "Breastfeeding, Infant practices, Phenylketonuria, Phenylalanine, Phe-free infant formula, DIETARY-MANAGEMENT, PROTEIN, GROWTH",

author = "A. Pinto and S. Adams and K. Ahring and H. Allen and Almeida, {M. F.} and D. Garcia-Arenas and N. Arslan and M. Assoun and Altinok, {Y. Atik} and D. Barrio-Carreras and {Belanger Quintana}, A. and Bernabei, {S. M.} and C. Bontemps and F. Boyle and G. Bruni and M. Bueno-Delgado and G. Caine and R. Carvalho and A. Chrobot and K. Chyz and B. Cochrane and C. Correia and K. Corthouts and A. Daly and {De Leo}, S. and A. Desloovere and {De Meyer}, A. and {De Theux}, A. and B. Didycz and Dijsselhof, {M. E.} and K. Dokoupil and J. Drabik and C. Dunlop and W. Eberle-Pelloth and K. Eftring and J. Ekengren and I. Errekalde and S. Evans and A. Foucart and L. Fokkema and L. Francois and M. French and E. Forssell and Kok, {I. L.} and M. Robert and {van Dam}, E. and {van den Hurk}, {T. A. M.} and {van der Ploeg}, {E. M. C.} and {van Wegberg}, {A. M. J.} and A. MacDonald",

year = "2018",

month = sep,

doi = "10.1016/j.ymgmr.2018.07.008",

language = "English",

volume = "16",

pages = "82--89",

journal = "Molecular genetics and metabolism reports",

issn = "2214-4269",

publisher = "ELSEVIER SCIENCE BV",

}

Pinto, A, Adams, S, Ahring, K, Allen, H, Almeida, MF, Garcia-Arenas, D, Arslan, N, Assoun, M, Altinok, YA, Barrio-Carreras, D, Belanger Quintana, A, Bernabei, SM, Bontemps, C, Boyle, F, Bruni, G, Bueno-Delgado, M, Caine, G, Carvalho, R, Chrobot, A, Chyz, K, Cochrane, B, Correia, C, Corthouts, K, Daly, A, De Leo, S, Desloovere, A, De Meyer, A, De Theux, A, Didycz, B, Dijsselhof, ME, Dokoupil, K, Drabik, J, Dunlop, C, Eberle-Pelloth, W, Eftring, K, Ekengren, J, Errekalde, I, Evans, S, Foucart, A, Fokkema, L, Francois, L, French, M, Forssell, E, Kok, IL, Robert, M, van Dam, E, van den Hurk, TAM, van der Ploeg, EMC, van Wegberg, AMJ & MacDonald, A 2018, 'Early feeding practices in infants with phenylketonuria across Europe', Molecular genetics and metabolism reports, vol. 16, pp. 82-89. https://doi.org/10.1016/j.ymgmr.2018.07.008

TY - JOUR

T1 - Early feeding practices in infants with phenylketonuria across Europe

AU - Pinto, A.

AU - Adams, S.

AU - Ahring, K.

AU - Allen, H.

AU - Almeida, M. F.

AU - Garcia-Arenas, D.

AU - Arslan, N.

AU - Assoun, M.

AU - Altinok, Y. Atik

AU - Barrio-Carreras, D.

AU - Belanger Quintana, A.

AU - Bernabei, S. M.

AU - Bontemps, C.

AU - Boyle, F.

AU - Bruni, G.

AU - Bueno-Delgado, M.

AU - Caine, G.

AU - Carvalho, R.

AU - Chrobot, A.

AU - Chyz, K.

AU - Cochrane, B.

AU - Correia, C.

AU - Corthouts, K.

AU - Daly, A.

AU - De Leo, S.

AU - Desloovere, A.

AU - De Meyer, A.

AU - De Theux, A.

AU - Didycz, B.

AU - Dijsselhof, M. E.

AU - Dokoupil, K.

AU - Drabik, J.

AU - Dunlop, C.

AU - Eberle-Pelloth, W.

AU - Eftring, K.

AU - Ekengren, J.

AU - Errekalde, I.

AU - Evans, S.

AU - Foucart, A.

AU - Fokkema, L.

AU - Francois, L.

AU - French, M.

AU - Forssell, E.

AU - Kok, I. L.

AU - Robert, M.

AU - van Dam, E.

AU - van den Hurk, T. A. M.

AU - van der Ploeg, E. M. C.

AU - van Wegberg, A. M. J.

AU - MacDonald, A.

PY - 2018/9

Y1 - 2018/9

N2 - 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 (R) 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.

AB - 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 (R) 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.

KW - Breastfeeding

KW - Infant practices

KW - Phenylketonuria

KW - Phenylalanine

KW - Phe-free infant formula

KW - DIETARY-MANAGEMENT

KW - PROTEIN

KW - GROWTH

U2 - 10.1016/j.ymgmr.2018.07.008

DO - 10.1016/j.ymgmr.2018.07.008

M3 - Article

SN - 2214-4269

VL - 16

SP - 82

EP - 89

JO - Molecular genetics and metabolism reports

JF - Molecular genetics and metabolism reports

ER -

Early feeding practices in infants with phenylketonuria across Europe

Abstract

Keywords

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