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 |
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Pages (from-to) | 83-89 |
Number of pages | 7 |
Journal | Molecular Genetics and Metabolism Reports |
Volume | 13 |
DOIs | |
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