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

Molecular Genetics and Metabolism Reports（2017）

Cited 21|Views26

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Abstract

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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Key words

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

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