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; Rachael A Clark; Sue Clark; B. Cochrane; K. Corthouts; Judith Dalmau; M. Dassy; A. 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. Gizewska; H. Gokmen-Ozel; J. Gribben; F. Gunden; M. Heddrich-Ellerbrok; S. Heiber; C. Heidenborg; C. Jankowski; R.G. Janssen-Regelink; C.I. Jones; C.F. 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; J. de Moor; A. Pal; C.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 den Ploeg; M. van Rijn; Francjan 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: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, Rachael A Clark, Sue Clark, B. Cochrane, K. Corthouts, Judith Dalmau, M. Dassy, A. 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. Gizewska, H. Gokmen-Ozel, J. Gribben, F. Gunden, M. Heddrich-Ellerbrok, S. Heiber, C. Heidenborg, C. Jankowski, R.G. Janssen-Regelink, C.I. Jones, C.F. 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, J. de Moor, A. Pal, C.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 den Ploeg, M. van Rijn, Francjan 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

Dietetics

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

Abstract

Background: There appears little consensus concerning protein requirements in phenylketonuria (PKU). Methods: 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. Results: 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 . 2-3. g/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 . 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 . 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). Conclusions: 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. © 2015 Elsevier Inc..

Original language	English
Pages (from-to)	17-22
Number of pages	6
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

Keywords

amino acid
casein
caseinomacropeptide
peptide fragment
phenylalanine
protein intake, age distribution
Article
child
childhood disease
clinical practice
consensus
cross-sectional study
Eastern Europe
Europe
human
infant disease
major clinical study
Northern European
patient monitoring
phenylketonuria
preschool child
prescription
priority journal
protein intake
questionnaire
school child
Southern Europe
Western Europe
administration and dosage
adult
diet therapy
dietary supplement
female
infant
male
newborn
Turkey
world health organization, Adult
Amino Acids
Caseins
Child
Child, Preschool
Dietary Proteins
Dietary Supplements
Female
Humans
Infant
Infant, Newborn
Male
Peptide Fragments
Phenylalanine
Phenylketonurias
Surveys and Questionnaires
World Health Organization

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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, R. A., Clark, S., Cochrane, B., Corthouts, K., Dalmau, J., Dassy, M., 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{0dc36e57a85b498c8dbc38320ff14d2a,

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

abstract = "Background: There appears little consensus concerning protein requirements in phenylketonuria (PKU). Methods: 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. Results: 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 . 2-3. g/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 . 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 . 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). Conclusions: 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. {\textcopyright} 2015 Elsevier Inc..",

keywords = "amino acid, casein, caseinomacropeptide, peptide fragment, phenylalanine, protein intake, age distribution, Article, child, childhood disease, clinical practice, consensus, cross-sectional study, Eastern Europe, Europe, human, infant disease, major clinical study, Northern European, patient monitoring, phenylketonuria, preschool child, prescription, priority journal, protein intake, questionnaire, school child, Southern Europe, Western Europe, administration and dosage, adult, diet therapy, dietary supplement, female, infant, male, newborn, Turkey, world health organization, Adult, Amino Acids, Caseins, Child, Child, Preschool, Dietary Proteins, Dietary Supplements, Female, Humans, Infant, Infant, Newborn, Male, Peptide Fragments, Phenylalanine, Phenylketonurias, Surveys and Questionnaires, World Health Organization",

author = "A. Aguiar and K. Ahring and M.F. Almeida 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 Clark, {Rachael A} and Sue Clark and B. Cochrane and K. Corthouts and Judith Dalmau and M. Dassy and A. Meyer 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. Gizewska 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.G. Janssen-Regelink and C.I. Jones and C.F. Jonkers and M. Joerg-Streller and K. Kaalund-Hansen and E. Kiss and A.M. Lammardo and K. Lang and D. Lier and R. Lilje and S. Lowry and K. Luyten and A. MacDonald and U. Meyer and {de Moor}, J. and A. Pal and C.M. Robert and L. Robertson and J.C. Rocha 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 den Ploeg}, L. and {van Rijn}, M. and {van Spronsen}, {Francjan 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 F.J. White and H. Zweers",

note = "cited By 5",

year = "2015",

doi = "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, RA, Clark, S, Cochrane, B, Corthouts, K, Dalmau, J, Dassy, M, 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, Gizewska, M, Gokmen-Ozel, H, Gribben, J, Gunden, F, Heddrich-Ellerbrok, M, Heiber, S, Heidenborg, C, Jankowski, C, Janssen-Regelink, RG, Jones, CI, Jonkers, CF, Joerg-Streller, M, Kaalund-Hansen, K, Kiss, E, Lammardo, AM, Lang, K, Lier, D, Lilje, R, Lowry, S, Luyten, K, MacDonald, A, Meyer, U, de Moor, J, Pal, A, Robert, CM, 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 den 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

T2 - 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, Rachael A

AU - Clark, Sue

AU - Cochrane, B.

AU - Corthouts, K.

AU - Dalmau, Judith

AU - Dassy, M.

AU - 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 - Gizewska, 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.G.

AU - Jones, C.I.

AU - Jonkers, C.F.

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 - de Moor, J.

AU - Pal, A.

AU - Robert, C.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 den Ploeg, L.

AU - van Rijn, M.

AU - van Spronsen, Francjan 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.

N1 - cited By 5

PY - 2015

Y1 - 2015

N2 - Background: There appears little consensus concerning protein requirements in phenylketonuria (PKU). Methods: 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. Results: 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 . 2-3. g/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 . 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 . 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). Conclusions: 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. © 2015 Elsevier Inc..

AB - Background: There appears little consensus concerning protein requirements in phenylketonuria (PKU). Methods: 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. Results: 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 . 2-3. g/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 . 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 . 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). Conclusions: 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. © 2015 Elsevier Inc..

KW - amino acid

KW - casein

KW - caseinomacropeptide

KW - peptide fragment

KW - phenylalanine

KW - protein intake, age distribution

KW - Article

KW - child

KW - childhood disease

KW - clinical practice

KW - consensus

KW - cross-sectional study

KW - Eastern Europe

KW - Europe

KW - human

KW - infant disease

KW - major clinical study

KW - Northern European

KW - patient monitoring

KW - phenylketonuria

KW - preschool child

KW - prescription

KW - priority journal

KW - protein intake

KW - questionnaire

KW - school child

KW - Southern Europe

KW - Western Europe

KW - administration and dosage

KW - adult

KW - diet therapy

KW - dietary supplement

KW - female

KW - infant

KW - male

KW - newborn

KW - Turkey

KW - world health organization, Adult

KW - Amino Acids

KW - Caseins

KW - Child

KW - Child, Preschool

KW - Dietary Proteins

KW - Dietary Supplements

KW - Female

KW - Humans

KW - Infant

KW - Infant, Newborn

KW - Male

KW - Peptide Fragments

KW - Phenylalanine

KW - Phenylketonurias

KW - Surveys and Questionnaires

KW - World Health Organization

U2 - 10.1016/j.ymgme.2015.03.006

DO - 10.1016/j.ymgme.2015.03.006

M3 - Article

SN - 1096-7192

VL - 115

SP - 17

EP - 22

JO - Molecular Genetics and Metabolism

JF - Molecular Genetics and Metabolism

IS - 1

ER -

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

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Keywords

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