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

1. Venditti C et al. Propionic Acidemia. Gene Reviews (internet). May 2012, last update September 2024.