Dietary practices in propionic acidemia: A European survey

A. Daly; A. Pinto; S. Evans; M. F. Almeida; M. Assoun; A. Belanger-Quintana; S. M. Bernabei; S. Bollhalder; D. Cassiman; H. Champion; H. Chan; J. Dalmau; F. de Boer; C. de Laet; A. de Meyer; A. Desloovere; A. Dianin; M. Dixon; K. Dokoupil; S. Dubois; F. Eyskens; A. Faria; I. Fasan; E. Favre; F. Feillet; A. Fekete; G. Gallo; C. Gingell; J. Gribben; K. Kaalund Hansen; N. M. Ter Horst; C. Jankowski; R. Janssen-Regelink; I. Jones; C. Jouault; G. E. Kahrs; I. L. Kok; A. Kowalik; C. Laguerre; S. Le Verge; R. Lilje; C. Maddalon; D. Mayr; U. Meyer; A. Micciche; U. Och; M. Robert; J. C. Rocha; H. Rogozinski; C. Rohde; K. Ross; I. Saruggia; A. Schlune; K. Singleton; E. Sjoqvist; R. Skeath; L. H. Stolen; A. Terry; C. Timmer; L. Tomlinson; A. Tooke; K. Vande Kerckhove; E. van Dam; T. van den Hurk; L. van der Ploeg; M. van Driessche; M. van Rijn; A. van Wegberg; C. Vasconcelos; H. Vestergaard; I. Vitoria; D. Webster; F. J. White; L. White; H. Zweers; A. MacDonald

doi:10.1016/j.ymgmr.2017.09.002

Dietary practices in propionic acidemia: A European survey

A. Daly, A. Pinto, S. Evans, M. F. Almeida, M. Assoun, A. Belanger-Quintana, S. M. Bernabei, S. Bollhalder, D. Cassiman, H. Champion, H. Chan, J. Dalmau, F. de Boer, C. de Laet, A. de Meyer, A. Desloovere, A. Dianin, M. Dixon, K. Dokoupil, S. DuboisF. Eyskens, A. Faria, I. Fasan, E. Favre, F. Feillet, A. Fekete, G. Gallo, C. Gingell, J. Gribben, K. Kaalund Hansen, N. M. Ter Horst, C. Jankowski, R. Janssen-Regelink, I. Jones, C. Jouault, G. E. Kahrs, I. L. Kok, A. Kowalik, C. Laguerre, S. Le Verge, R. Lilje, C. Maddalon, D. Mayr, U. Meyer, A. Micciche, U. Och, M. Robert, J. C. Rocha, H. Rogozinski, C. Rohde, K. Ross, I. Saruggia, A. Schlune, K. Singleton, E. Sjoqvist, R. Skeath, L. H. Stolen, A. Terry, C. Timmer, L. Tomlinson, A. Tooke, K. Vande Kerckhove, E. van Dam, T. van den Hurk, L. van der Ploeg, M. van Driessche, M. van Rijn, A. van Wegberg, C. Vasconcelos, H. Vestergaard, I. Vitoria, D. Webster, F. J. White, L. White, H. Zweers, A. MacDonald^*

^*Corresponding author for this work

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

Abstract

Background: The definitive dietary management of propionic acidaemia (PA) is unknown although natural protein restriction with adequate energy provision is of key importance.

Aim: To describe European dietary practices in the management of patients with PA prior to the publication of the European PA guidelines.

Methods: This was a cross-sectional survey consisting of 27 questions about the dietary practices in PA patients circulated to European IMD dietitians and health professionals in 2014.

Results: Information on protein restricted diets of 186 PA patients from 47 centres, representing 14 European countries was collected. Total protein intake [PA precursor-free L-amino acid supplements (PFAA) and natural protein] met WHO/FAO/UNU (2007) safe protein requirements for age in 36 centres (77%). PFAA were used to supplement natural protein intake in 81% (n = 38) of centres, providing a median of 44% (14-83%) of total protein requirement. Seventy-four per cent of patients were prescribed natural protein intakes below WHO/ FAO/UNU (2007) safe levels in one or more of the following age groups: 0-6 m, 7-12 m, 1-10 y, 11-16 y and > 16 y. Sixty-three per cent (n = 117) of patients were tube fed (74% gastrostomy), but only 22% received nocturnal feeds.

Conclusions: There was high use of PFAA with intakes of natural protein commonly below WHO/FAO/UNU (2007) safe levels. Optimal dietary management can only be determined by longitudinal, multi-centre, prospective case controlled studies. The metabolic instability of PA and small patient cohorts in each centre ensure that this is a challenging undertaking.

Original language	English
Pages (from-to)	83-89
Number of pages	7
Journal	Molecular Genetics and Metabolism Reports
Volume	13
DOIs	https://doi.org/10.1016/j.ymgmr.2017.09.002
Publication status	Published - Dec 2017

Keywords

Propionic acidemia
Protein restricted diet
Precursor-free amino acids
Natural protein
ENTEROPATHICA-LIKE SYNDROME
ORGANIC ACIDURIAS
MANAGEMENT
METABOLISM
DISORDERS
DEFICIENCY
TERM

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Daly, A., Pinto, A., Evans, S., Almeida, M. F., Assoun, M., Belanger-Quintana, A., Bernabei, S. M., Bollhalder, S., Cassiman, D., Champion, H., Chan, H., Dalmau, J., de Boer, F., de Laet, C., de Meyer, A., Desloovere, A., Dianin, A., Dixon, M., Dokoupil, K., ... MacDonald, A. (2017). Dietary practices in propionic acidemia: A European survey. Molecular Genetics and Metabolism Reports, 13, 83-89. https://doi.org/10.1016/j.ymgmr.2017.09.002

@article{636c15466feb458aafd5ffbd02ae8011,

title = "Dietary practices in propionic acidemia: A European survey",

abstract = "Background: The definitive dietary management of propionic acidaemia (PA) is unknown although natural protein restriction with adequate energy provision is of key importance.Aim: To describe European dietary practices in the management of patients with PA prior to the publication of the European PA guidelines.Methods: This was a cross-sectional survey consisting of 27 questions about the dietary practices in PA patients circulated to European IMD dietitians and health professionals in 2014.Results: Information on protein restricted diets of 186 PA patients from 47 centres, representing 14 European countries was collected. Total protein intake [PA precursor-free L-amino acid supplements (PFAA) and natural protein] met WHO/FAO/UNU (2007) safe protein requirements for age in 36 centres (77%). PFAA were used to supplement natural protein intake in 81% (n = 38) of centres, providing a median of 44% (14-83%) of total protein requirement. Seventy-four per cent of patients were prescribed natural protein intakes below WHO/ FAO/UNU (2007) safe levels in one or more of the following age groups: 0-6 m, 7-12 m, 1-10 y, 11-16 y and > 16 y. Sixty-three per cent (n = 117) of patients were tube fed (74% gastrostomy), but only 22% received nocturnal feeds.Conclusions: There was high use of PFAA with intakes of natural protein commonly below WHO/FAO/UNU (2007) safe levels. Optimal dietary management can only be determined by longitudinal, multi-centre, prospective case controlled studies. The metabolic instability of PA and small patient cohorts in each centre ensure that this is a challenging undertaking.",

keywords = "Propionic acidemia, Protein restricted diet, Precursor-free amino acids, Natural protein, ENTEROPATHICA-LIKE SYNDROME, ORGANIC ACIDURIAS, MANAGEMENT, METABOLISM, DISORDERS, DEFICIENCY, TERM",

author = "A. Daly and A. Pinto and S. Evans and Almeida, {M. F.} and M. Assoun and A. Belanger-Quintana and Bernabei, {S. M.} and S. Bollhalder and D. Cassiman and H. Champion and H. Chan and J. Dalmau and {de Boer}, F. and {de Laet}, C. and {de Meyer}, A. and A. Desloovere and A. Dianin and M. Dixon and K. Dokoupil and S. Dubois and F. Eyskens and A. Faria and I. Fasan and E. Favre and F. Feillet and A. Fekete and G. Gallo and C. Gingell and J. Gribben and Hansen, {K. Kaalund} and {Ter Horst}, {N. M.} and C. Jankowski and R. Janssen-Regelink and I. Jones and C. Jouault and Kahrs, {G. E.} and Kok, {I. L.} and A. Kowalik and C. Laguerre and {Le Verge}, S. and R. Lilje and C. Maddalon and D. Mayr and U. Meyer and A. Micciche and U. Och and M. Robert and Rocha, {J. C.} and H. Rogozinski and C. Rohde and K. Ross and I. Saruggia and A. Schlune and K. Singleton and E. Sjoqvist and R. Skeath and Stolen, {L. H.} and A. Terry and C. Timmer and L. Tomlinson and A. Tooke and Kerckhove, {K. Vande} and {van Dam}, E. and {van den Hurk}, T. and {van der Ploeg}, L. and {van Driessche}, M. and {van Rijn}, M. and {van Wegberg}, A. and C. Vasconcelos and H. Vestergaard and I. Vitoria and D. Webster and White, {F. J.} and L. White and H. Zweers and A. MacDonald",

year = "2017",

month = dec,

doi = "10.1016/j.ymgmr.2017.09.002",

language = "English",

volume = "13",

pages = "83--89",

journal = "Molecular Genetics and Metabolism Reports",

issn = "2214-4269",

publisher = "Elsevier Science",

}

Daly, A, Pinto, A, Evans, S, Almeida, MF, Assoun, M, Belanger-Quintana, A, Bernabei, SM, Bollhalder, S, Cassiman, D, Champion, H, Chan, H, Dalmau, J, de Boer, F, de Laet, C, de Meyer, A, Desloovere, A, Dianin, A, Dixon, M, Dokoupil, K, Dubois, S, Eyskens, F, Faria, A, Fasan, I, Favre, E, Feillet, F, Fekete, A, Gallo, G, Gingell, C, Gribben, J, Hansen, KK, Ter Horst, NM, Jankowski, C, Janssen-Regelink, R, Jones, I, Jouault, C, Kahrs, GE, Kok, IL, Kowalik, A, Laguerre, C, Le Verge, S, Lilje, R, Maddalon, C, Mayr, D, Meyer, U, Micciche, A, Och, U, Robert, M, Rocha, JC, Rogozinski, H, Rohde, C, Ross, K, Saruggia, I, Schlune, A, Singleton, K, Sjoqvist, E, Skeath, R, Stolen, LH, Terry, A, Timmer, C, Tomlinson, L, Tooke, A, Kerckhove, KV, van Dam, E, van den Hurk, T, van der Ploeg, L, van Driessche, M, van Rijn, M, van Wegberg, A, Vasconcelos, C, Vestergaard, H, Vitoria, I, Webster, D, White, FJ, White, L, Zweers, H & MacDonald, A 2017, 'Dietary practices in propionic acidemia: A European survey', Molecular Genetics and Metabolism Reports, vol. 13, pp. 83-89. https://doi.org/10.1016/j.ymgmr.2017.09.002

TY - JOUR

T1 - Dietary practices in propionic acidemia

T2 - A European survey

AU - Daly, A.

AU - Pinto, A.

AU - Evans, S.

AU - Almeida, M. F.

AU - Assoun, M.

AU - Belanger-Quintana, A.

AU - Bernabei, S. M.

AU - Bollhalder, S.

AU - Cassiman, D.

AU - Champion, H.

AU - Chan, H.

AU - Dalmau, J.

AU - de Boer, F.

AU - de Laet, C.

AU - de Meyer, A.

AU - Desloovere, A.

AU - Dianin, A.

AU - Dixon, M.

AU - Dokoupil, K.

AU - Dubois, S.

AU - Eyskens, F.

AU - Faria, A.

AU - Fasan, I.

AU - Favre, E.

AU - Feillet, F.

AU - Fekete, A.

AU - Gallo, G.

AU - Gingell, C.

AU - Gribben, J.

AU - Hansen, K. Kaalund

AU - Ter Horst, N. M.

AU - Jankowski, C.

AU - Janssen-Regelink, R.

AU - Jones, I.

AU - Jouault, C.

AU - Kahrs, G. E.

AU - Kok, I. L.

AU - Kowalik, A.

AU - Laguerre, C.

AU - Le Verge, S.

AU - Lilje, R.

AU - Maddalon, C.

AU - Mayr, D.

AU - Meyer, U.

AU - Micciche, A.

AU - Och, U.

AU - Robert, M.

AU - Rocha, J. C.

AU - Rogozinski, H.

AU - Rohde, C.

AU - Ross, K.

AU - Saruggia, I.

AU - Schlune, A.

AU - Singleton, K.

AU - Sjoqvist, E.

AU - Skeath, R.

AU - Stolen, L. H.

AU - Terry, A.

AU - Timmer, C.

AU - Tomlinson, L.

AU - Tooke, A.

AU - Kerckhove, K. Vande

AU - van Dam, E.

AU - van den Hurk, T.

AU - van der Ploeg, L.

AU - van Driessche, M.

AU - van Rijn, M.

AU - van Wegberg, A.

AU - Vasconcelos, C.

AU - Vestergaard, H.

AU - Vitoria, I.

AU - Webster, D.

AU - White, F. J.

AU - White, L.

AU - Zweers, H.

AU - MacDonald, A.

PY - 2017/12

Y1 - 2017/12

N2 - Background: The definitive dietary management of propionic acidaemia (PA) is unknown although natural protein restriction with adequate energy provision is of key importance.Aim: To describe European dietary practices in the management of patients with PA prior to the publication of the European PA guidelines.Methods: This was a cross-sectional survey consisting of 27 questions about the dietary practices in PA patients circulated to European IMD dietitians and health professionals in 2014.Results: Information on protein restricted diets of 186 PA patients from 47 centres, representing 14 European countries was collected. Total protein intake [PA precursor-free L-amino acid supplements (PFAA) and natural protein] met WHO/FAO/UNU (2007) safe protein requirements for age in 36 centres (77%). PFAA were used to supplement natural protein intake in 81% (n = 38) of centres, providing a median of 44% (14-83%) of total protein requirement. Seventy-four per cent of patients were prescribed natural protein intakes below WHO/ FAO/UNU (2007) safe levels in one or more of the following age groups: 0-6 m, 7-12 m, 1-10 y, 11-16 y and > 16 y. Sixty-three per cent (n = 117) of patients were tube fed (74% gastrostomy), but only 22% received nocturnal feeds.Conclusions: There was high use of PFAA with intakes of natural protein commonly below WHO/FAO/UNU (2007) safe levels. Optimal dietary management can only be determined by longitudinal, multi-centre, prospective case controlled studies. The metabolic instability of PA and small patient cohorts in each centre ensure that this is a challenging undertaking.

AB - Background: The definitive dietary management of propionic acidaemia (PA) is unknown although natural protein restriction with adequate energy provision is of key importance.Aim: To describe European dietary practices in the management of patients with PA prior to the publication of the European PA guidelines.Methods: This was a cross-sectional survey consisting of 27 questions about the dietary practices in PA patients circulated to European IMD dietitians and health professionals in 2014.Results: Information on protein restricted diets of 186 PA patients from 47 centres, representing 14 European countries was collected. Total protein intake [PA precursor-free L-amino acid supplements (PFAA) and natural protein] met WHO/FAO/UNU (2007) safe protein requirements for age in 36 centres (77%). PFAA were used to supplement natural protein intake in 81% (n = 38) of centres, providing a median of 44% (14-83%) of total protein requirement. Seventy-four per cent of patients were prescribed natural protein intakes below WHO/ FAO/UNU (2007) safe levels in one or more of the following age groups: 0-6 m, 7-12 m, 1-10 y, 11-16 y and > 16 y. Sixty-three per cent (n = 117) of patients were tube fed (74% gastrostomy), but only 22% received nocturnal feeds.Conclusions: There was high use of PFAA with intakes of natural protein commonly below WHO/FAO/UNU (2007) safe levels. Optimal dietary management can only be determined by longitudinal, multi-centre, prospective case controlled studies. The metabolic instability of PA and small patient cohorts in each centre ensure that this is a challenging undertaking.

KW - Propionic acidemia

KW - Protein restricted diet

KW - Precursor-free amino acids

KW - Natural protein

KW - ENTEROPATHICA-LIKE SYNDROME

KW - ORGANIC ACIDURIAS

KW - MANAGEMENT

KW - METABOLISM

KW - DISORDERS

KW - DEFICIENCY

KW - TERM

U2 - 10.1016/j.ymgmr.2017.09.002

DO - 10.1016/j.ymgmr.2017.09.002

M3 - Article

C2 - 29021961

SN - 2214-4269

VL - 13

SP - 83

EP - 89

JO - Molecular Genetics and Metabolism Reports

JF - Molecular Genetics and Metabolism Reports

ER -