Treatment
Maintenance:
- manipulation of protein intake to remove precursors decreases propionic acid production. Often affected children have chronically poor appetites and require an enteral feeding tube
- regular carnitine restores free coenzyme A levels and facilitates renal excretion of propionic acid
- intermittent administration of oral metronidazole decreases the production of propionic acid by gut flora
Acute metabolic decompensation:
- high carbohydrate, protein free feed - either a carbohydrate energy supplement such as Maxijul, or IV 10% Dextrose
- administration of sodium benzoate and/or sodium phenylbutyrate provides alternative pathways for excretion of nitrogen
Reference
- Venditti C et al. Propionic Acidemia. Gene Reviews (internet). May 2012, last update September 2024.
Related pages
Create an account to add page annotations
Annotations allow you to add information to this page that would be handy to have on hand during a consultation. E.g. a website or number. This information will always show when you visit this page.