treatment
Last reviewed 01/2018
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