Dietary practices in propionic acidemia: A European survey.
Molecular Genetics and Metabolism Reports(2017)
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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