Practices in prescribing protein substitutes for PKU in Europe: No uniformity of approach

A. Aguiar; K. Ahring; M. F. Almeida; M. Assoun; A. Belanger Quintana; S. Bigot; G. Bihet; K. Blom Malmberg; A. Burlina; T. Bushueva; A. Caris; H. Chan; A. Clark; S. Clark; B. Cochrane; K. Corthouts; J. Dalmau; M. Dassy; A. de Meyer; B. Didycz; M. Diels; K. Dokupil; S. Dubois; K. Eftring; J. Ekengren; C. Ellerton; S. Evans; A. Faria; A. Fischer; S. Ford; P. Freisinger; M. Giżewska; H. Gokmen-Ozel; J. Gribben; F. Gunden; M. Heddrich-Ellerbrok; S. Heiber; C. Heidenborg; C. Jankowski; R. Janssen-Regelink; I. Jones; C. Jonkers; M. Joerg-Streller; K. Kaalund-Hansen; E. Kiss; A. M. Lammardo; K. Lang; D. Lier; R. Lilje; S. Lowry; K. Luyten; A. Macdonald; U. Meyer; D. Moor; A. Pal; M. Robert; L. Robertson; J. C. Rocha; C. Rohde; K. Ross; S. Saruhan; E. Sjöqvist; R. Skeath; L. Stoelen; N. M. ter Horst; A. Terry; C. Timmer; N. Tuncer; K. Vande Kerckhove; L. van der Ploeg; M. van Rijn; F. J. van Spronsen; A. van Teeffelen-Heithoff; A. van Wegberg; K. van Wyk; C. Vasconcelos; I. Vitoria; J. Wildgoose; D. Webster; F. J. White; H. Zweers

doi:https://doi.org/10.1016/j.ymgme.2015.03.006

Practices in prescribing protein substitutes for PKU in Europe: No uniformity of approach

A. Aguiar, K. Ahring, M. F. Almeida, M. Assoun, A. Belanger Quintana, S. Bigot, G. Bihet, K. Blom Malmberg, A. Burlina, T. Bushueva, A. Caris, H. Chan, A. Clark, S. Clark, B. Cochrane, K. Corthouts, J. Dalmau, M. Dassy, A. de Meyer, B. DidyczM. Diels, K. Dokupil, S. Dubois, K. Eftring, J. Ekengren, C. Ellerton, S. Evans, A. Faria, A. Fischer, S. Ford, P. Freisinger, M. Giżewska, H. Gokmen-Ozel, J. Gribben, F. Gunden, M. Heddrich-Ellerbrok, S. Heiber, C. Heidenborg, C. Jankowski, R. Janssen-Regelink, I. Jones, C. Jonkers, M. Joerg-Streller, K. Kaalund-Hansen, E. Kiss, A. M. Lammardo, K. Lang, D. Lier, R. Lilje, S. Lowry, K. Luyten, A. Macdonald, U. Meyer, D. Moor, A. Pal, M. Robert, L. Robertson, J. C. Rocha, C. Rohde, K. Ross, S. Saruhan, E. Sjöqvist, R. Skeath, L. Stoelen, N. M. ter Horst, A. Terry, C. Timmer, N. Tuncer, K. Vande Kerckhove, L. van der Ploeg, M. van Rijn, F. J. van Spronsen, A. van Teeffelen-Heithoff, A. van Wegberg, K. van Wyk, C. Vasconcelos, I. Vitoria, J. Wildgoose, D. Webster, F. J. White, H. Zweers

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

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Abstract

There appears little consensus concerning protein requirements in phenylketonuria (PKU). A questionnaire completed by 63 European and Turkish IMD centres from 18 countries collected data on prescribed total protein intake (natural/intact protein and phenylalanine-free protein substitute [PS]) by age, administration frequency and method, monitoring, and type of protein substitute. Data were analysed by European region using descriptive statistics. The amount of total protein (from PS and natural/intact protein) varied according to the European region. Higher median amounts of total protein were prescribed in infants and children in Northern Europe (n=24 centres) (infants <1 year, >2-3g/kg/day; 1-3 years of age, >2-3 g/kg/day; 4-10 years of age, >1.5-2.5 g/kg/day) and Southern Europe (n=10 centres) (infants <1 year, 2.5 g/kg/day, 1-3 years of age, 2 g/kg/day; 4-10 years of age, 1.5-2 g/kg/day), than by Eastern Europe (n=4 centres) (infants <1 year, 2.5 g/kg/day, 1-3 years of age, >2-2.5 g/kg/day; 4-10 years of age, >1.5-2 g/kg/day) and with Western Europe (n=25 centres) giving the least (infants <1 year, >2-2.5 g/kg/day, 1-3 years of age, 1.5-2 g/kg/day; 4-10 years of age, 1-1.5 g/kg/day). Total protein prescription was similar in patients aged >10 years (1-1.5 g/kg/day) and maternal patients (1-1.5 g/kg/day). The amounts of total protein prescribed varied between European countries and appeared to be influenced by geographical region. In PKU, all gave higher than the recommended 2007 WHO/FAO/UNU safe levels of protein intake for the general population

Original language	English
Pages (from-to)	17-22
Journal	Molecular Genetics and Metabolism
Volume	115
Issue number	1
DOIs	https://doi.org/10.1016/j.ymgme.2015.03.006
Publication status	Published - 2015

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https://doi.org/10.1016/j.ymgme.2015.03.006

Cite this

Aguiar, A., Ahring, K., Almeida, M. F., Assoun, M., Belanger Quintana, A., Bigot, S., Bihet, G., Blom Malmberg, K., Burlina, A., Bushueva, T., Caris, A., Chan, H., Clark, A., Clark, S., Cochrane, B., Corthouts, K., Dalmau, J., Dassy, M., de Meyer, A., ... Zweers, H. (2015). Practices in prescribing protein substitutes for PKU in Europe: No uniformity of approach. Molecular Genetics and Metabolism, 115(1), 17-22. https://doi.org/10.1016/j.ymgme.2015.03.006

@article{4ea32d91c3f141bcb425a07c688e235b,

title = "Practices in prescribing protein substitutes for PKU in Europe: No uniformity of approach",

abstract = "There appears little consensus concerning protein requirements in phenylketonuria (PKU). A questionnaire completed by 63 European and Turkish IMD centres from 18 countries collected data on prescribed total protein intake (natural/intact protein and phenylalanine-free protein substitute [PS]) by age, administration frequency and method, monitoring, and type of protein substitute. Data were analysed by European region using descriptive statistics. The amount of total protein (from PS and natural/intact protein) varied according to the European region. Higher median amounts of total protein were prescribed in infants and children in Northern Europe (n=24 centres) (infants <1 year, >2-3g/kg/day; 1-3 years of age, >2-3 g/kg/day; 4-10 years of age, >1.5-2.5 g/kg/day) and Southern Europe (n=10 centres) (infants <1 year, 2.5 g/kg/day, 1-3 years of age, 2 g/kg/day; 4-10 years of age, 1.5-2 g/kg/day), than by Eastern Europe (n=4 centres) (infants <1 year, 2.5 g/kg/day, 1-3 years of age, >2-2.5 g/kg/day; 4-10 years of age, >1.5-2 g/kg/day) and with Western Europe (n=25 centres) giving the least (infants <1 year, >2-2.5 g/kg/day, 1-3 years of age, 1.5-2 g/kg/day; 4-10 years of age, 1-1.5 g/kg/day). Total protein prescription was similar in patients aged >10 years (1-1.5 g/kg/day) and maternal patients (1-1.5 g/kg/day). The amounts of total protein prescribed varied between European countries and appeared to be influenced by geographical region. In PKU, all gave higher than the recommended 2007 WHO/FAO/UNU safe levels of protein intake for the general population",

author = "A. Aguiar and K. Ahring and Almeida, {M. F.} and M. Assoun and {Belanger Quintana}, A. and S. Bigot and G. Bihet and {Blom Malmberg}, K. and A. Burlina and T. Bushueva and A. Caris and H. Chan and A. Clark and S. Clark and B. Cochrane and K. Corthouts and J. Dalmau and M. Dassy and {de Meyer}, A. and B. Didycz and M. Diels and K. Dokupil and S. Dubois and K. Eftring and J. Ekengren and C. Ellerton and S. Evans and A. Faria and A. Fischer and S. Ford and P. Freisinger and M. Gi{\.z}ewska and H. Gokmen-Ozel and J. Gribben and F. Gunden and M. Heddrich-Ellerbrok and S. Heiber and C. Heidenborg and C. Jankowski and R. Janssen-Regelink and I. Jones and C. Jonkers and M. Joerg-Streller and K. Kaalund-Hansen and E. Kiss and Lammardo, {A. M.} and K. Lang and D. Lier and R. Lilje and S. Lowry and K. Luyten and A. Macdonald and U. Meyer and D. Moor and A. Pal and M. Robert and L. Robertson and Rocha, {J. C.} and C. Rohde and K. Ross and S. Saruhan and E. Sj{\"o}qvist and R. Skeath and L. Stoelen and {ter Horst}, {N. M.} and A. Terry and C. Timmer and N. Tuncer and {Vande Kerckhove}, K. and {van der Ploeg}, L. and {van Rijn}, M. and {van Spronsen}, {F. J.} and {van Teeffelen-Heithoff}, A. and {van Wegberg}, A. and {van Wyk}, K. and C. Vasconcelos and I. Vitoria and J. Wildgoose and D. Webster and White, {F. J.} and H. Zweers",

year = "2015",

doi = "https://doi.org/10.1016/j.ymgme.2015.03.006",

language = "English",

volume = "115",

pages = "17--22",

journal = "Molecular Genetics and Metabolism",

issn = "1096-7192",

publisher = "Academic Press Inc.",

number = "1",

}

Aguiar, A, Ahring, K, Almeida, MF, Assoun, M, Belanger Quintana, A, Bigot, S, Bihet, G, Blom Malmberg, K, Burlina, A, Bushueva, T, Caris, A, Chan, H, Clark, A, Clark, S, Cochrane, B, Corthouts, K, Dalmau, J, Dassy, M, de Meyer, A, Didycz, B, Diels, M, Dokupil, K, Dubois, S, Eftring, K, Ekengren, J, Ellerton, C, Evans, S, Faria, A, Fischer, A, Ford, S, Freisinger, P, Giżewska, M, Gokmen-Ozel, H, Gribben, J, Gunden, F, Heddrich-Ellerbrok, M, Heiber, S, Heidenborg, C, Jankowski, C, Janssen-Regelink, R, Jones, I, Jonkers, C, Joerg-Streller, M, Kaalund-Hansen, K, Kiss, E, Lammardo, AM, Lang, K, Lier, D, Lilje, R, Lowry, S, Luyten, K, Macdonald, A, Meyer, U, Moor, D, Pal, A, Robert, M, Robertson, L, Rocha, JC, Rohde, C, Ross, K, Saruhan, S, Sjöqvist, E, Skeath, R, Stoelen, L, ter Horst, NM, Terry, A, Timmer, C, Tuncer, N, Vande Kerckhove, K, van der Ploeg, L, van Rijn, M, van Spronsen, FJ, van Teeffelen-Heithoff, A, van Wegberg, A, van Wyk, K, Vasconcelos, C, Vitoria, I, Wildgoose, J, Webster, D, White, FJ & Zweers, H 2015, 'Practices in prescribing protein substitutes for PKU in Europe: No uniformity of approach', Molecular Genetics and Metabolism, vol. 115, no. 1, pp. 17-22. https://doi.org/10.1016/j.ymgme.2015.03.006

TY - JOUR

T1 - Practices in prescribing protein substitutes for PKU in Europe: No uniformity of approach

AU - Aguiar, A.

AU - Ahring, K.

AU - Almeida, M. F.

AU - Assoun, M.

AU - Belanger Quintana, A.

AU - Bigot, S.

AU - Bihet, G.

AU - Blom Malmberg, K.

AU - Burlina, A.

AU - Bushueva, T.

AU - Caris, A.

AU - Chan, H.

AU - Clark, A.

AU - Clark, S.

AU - Cochrane, B.

AU - Corthouts, K.

AU - Dalmau, J.

AU - Dassy, M.

AU - de Meyer, A.

AU - Didycz, B.

AU - Diels, M.

AU - Dokupil, K.

AU - Dubois, S.

AU - Eftring, K.

AU - Ekengren, J.

AU - Ellerton, C.

AU - Evans, S.

AU - Faria, A.

AU - Fischer, A.

AU - Ford, S.

AU - Freisinger, P.

AU - Giżewska, M.

AU - Gokmen-Ozel, H.

AU - Gribben, J.

AU - Gunden, F.

AU - Heddrich-Ellerbrok, M.

AU - Heiber, S.

AU - Heidenborg, C.

AU - Jankowski, C.

AU - Janssen-Regelink, R.

AU - Jones, I.

AU - Jonkers, C.

AU - Joerg-Streller, M.

AU - Kaalund-Hansen, K.

AU - Kiss, E.

AU - Lammardo, A. M.

AU - Lang, K.

AU - Lier, D.

AU - Lilje, R.

AU - Lowry, S.

AU - Luyten, K.

AU - Macdonald, A.

AU - Meyer, U.

AU - Moor, D.

AU - Pal, A.

AU - Robert, M.

AU - Robertson, L.

AU - Rocha, J. C.

AU - Rohde, C.

AU - Ross, K.

AU - Saruhan, S.

AU - Sjöqvist, E.

AU - Skeath, R.

AU - Stoelen, L.

AU - ter Horst, N. M.

AU - Terry, A.

AU - Timmer, C.

AU - Tuncer, N.

AU - Vande Kerckhove, K.

AU - van der Ploeg, L.

AU - van Rijn, M.

AU - van Spronsen, F. J.

AU - van Teeffelen-Heithoff, A.

AU - van Wegberg, A.

AU - van Wyk, K.

AU - Vasconcelos, C.

AU - Vitoria, I.

AU - Wildgoose, J.

AU - Webster, D.

AU - White, F. J.

AU - Zweers, H.

PY - 2015

Y1 - 2015

N2 - There appears little consensus concerning protein requirements in phenylketonuria (PKU). A questionnaire completed by 63 European and Turkish IMD centres from 18 countries collected data on prescribed total protein intake (natural/intact protein and phenylalanine-free protein substitute [PS]) by age, administration frequency and method, monitoring, and type of protein substitute. Data were analysed by European region using descriptive statistics. The amount of total protein (from PS and natural/intact protein) varied according to the European region. Higher median amounts of total protein were prescribed in infants and children in Northern Europe (n=24 centres) (infants <1 year, >2-3g/kg/day; 1-3 years of age, >2-3 g/kg/day; 4-10 years of age, >1.5-2.5 g/kg/day) and Southern Europe (n=10 centres) (infants <1 year, 2.5 g/kg/day, 1-3 years of age, 2 g/kg/day; 4-10 years of age, 1.5-2 g/kg/day), than by Eastern Europe (n=4 centres) (infants <1 year, 2.5 g/kg/day, 1-3 years of age, >2-2.5 g/kg/day; 4-10 years of age, >1.5-2 g/kg/day) and with Western Europe (n=25 centres) giving the least (infants <1 year, >2-2.5 g/kg/day, 1-3 years of age, 1.5-2 g/kg/day; 4-10 years of age, 1-1.5 g/kg/day). Total protein prescription was similar in patients aged >10 years (1-1.5 g/kg/day) and maternal patients (1-1.5 g/kg/day). The amounts of total protein prescribed varied between European countries and appeared to be influenced by geographical region. In PKU, all gave higher than the recommended 2007 WHO/FAO/UNU safe levels of protein intake for the general population

AB - There appears little consensus concerning protein requirements in phenylketonuria (PKU). A questionnaire completed by 63 European and Turkish IMD centres from 18 countries collected data on prescribed total protein intake (natural/intact protein and phenylalanine-free protein substitute [PS]) by age, administration frequency and method, monitoring, and type of protein substitute. Data were analysed by European region using descriptive statistics. The amount of total protein (from PS and natural/intact protein) varied according to the European region. Higher median amounts of total protein were prescribed in infants and children in Northern Europe (n=24 centres) (infants <1 year, >2-3g/kg/day; 1-3 years of age, >2-3 g/kg/day; 4-10 years of age, >1.5-2.5 g/kg/day) and Southern Europe (n=10 centres) (infants <1 year, 2.5 g/kg/day, 1-3 years of age, 2 g/kg/day; 4-10 years of age, 1.5-2 g/kg/day), than by Eastern Europe (n=4 centres) (infants <1 year, 2.5 g/kg/day, 1-3 years of age, >2-2.5 g/kg/day; 4-10 years of age, >1.5-2 g/kg/day) and with Western Europe (n=25 centres) giving the least (infants <1 year, >2-2.5 g/kg/day, 1-3 years of age, 1.5-2 g/kg/day; 4-10 years of age, 1-1.5 g/kg/day). Total protein prescription was similar in patients aged >10 years (1-1.5 g/kg/day) and maternal patients (1-1.5 g/kg/day). The amounts of total protein prescribed varied between European countries and appeared to be influenced by geographical region. In PKU, all gave higher than the recommended 2007 WHO/FAO/UNU safe levels of protein intake for the general population

U2 - https://doi.org/10.1016/j.ymgme.2015.03.006

DO - https://doi.org/10.1016/j.ymgme.2015.03.006

M3 - Article

C2 - 25862610

SN - 1096-7192

VL - 115

SP - 17

EP - 22

JO - Molecular Genetics and Metabolism

JF - Molecular Genetics and Metabolism

IS - 1

ER -