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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.; Boer, F. de; Laet, C. de; Meyer, A. de; 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, K. Kaalund; Ter Horst, N. M.; Jankowski, C.; Janssen-Regelink, R.; Jones, I.; Jouault, C.; Kahrs, G. E.; Kok, I. L.; Kowalik, A.; Laguerre, C.; Verge, S. le; Lilje, R.; Maddalon, C.; Mayr, D.; Meyer, U.; Micciche, A.; Och, U.; Robert, M.; Rocha, J. C.; Rogozinski, H.; Rohde, C.; Ross, K.; Saruggia, I.; Schlune, A.; Singleton, K.; Sjoqvist, E.; Skeath, R.; Stolen, L. H.; Terry, A.; Timmer, C.; Tomlinson, L.; Tooke, A.; Kerckhove, K. Vande; Dam, E. van; Hurk, T. van den; Ploeg, L. van der; Driessche, M. van; Rijn, M. van; Wegberg, A. van; Vasconcelos, C.; Vestergaard, H.; Vitoria, I.; Webster, D.; White, F. J.; White, L.; Zweers, H.; MacDonald, A. (2017): Dietary practices in propionic acidemia: A European survey. In: Molecular Genetics and Metabolism Reports, Vol. 13: pp. 83-89
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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.

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