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Dietary practices in propionic acidemia: A European survey.

Data de publicació:

Autors de IIS La Fe

Autors aliens a IIS La Fe

  • Daly, A
  • Pinto, A
  • Evans, S
  • Almeida, MF
  • Assoun, M
  • Belanger-Quintana, A
  • Bernabei, SM
  • Bollhalder, S
  • Cassiman, D
  • Champion, H
  • Chan, H
  • 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
  • Kaalund Hansen K
  • 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
  • Vande Kerckhove K
  • van Dam, E
  • van den Hurk, T
  • van der Ploeg, L
  • van Driessche, M
  • van Rijn, M
  • van Wegberg, A
  • Vasconcelos, C
  • Vestergaard, H
  • Webster, D
  • White, FJ
  • White, L
  • Zweers, H
  • MacDonald, A

Grups d'Investigació

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.

Dades de la publicació

ISSN/ISSNe:
2214-4269, 2214-4269

Molecular Genetics And Metabolism Reports  ELSEVIER SCIENCE BV

Tipus:
Article
Pàgines:
83-89
PubMed:
29021961
Factor d'Impacte:
0,523 SCImago ℠
Quartil:
Q3 SCImago ℠

Cites Rebudes en Web of Science: 17

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Filiacions mostrar / ocultar

Keywords

  • Natural protein; Precursor-free amino acids; Propionic acidemia; Protein restricted diet

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