Propionic acidemia (PROP)

 

Propionic acidemia (PROP) is an inherited condition in which the body is unable to break down certain proteins and fats. It is an organic acid condition because it can lead to a harmful amount of organic acids and toxins in the body. If left untreated, PROP can cause brain defects or even death. If PROP is identified early in life and proper treatment is begun, children with PROP can minimize some of the early complications of the condition.

PROP is also known as: PA, ketotic glycinemia. ketotic hyperglycinemia, PCC deficiency, propionic aciduria, propionyl-CoA carboxylase deficiency.

To learn more about Propionic acidemia, visit Baby's First Test: Propionic acidemia and Screening, Technology, and Research in Genetics (STAR-G): Propionic acidemia. Please see Medically Prescribed Formula for information about medically prescribed formula and pharmacologic interventions for inborn errors of metabolism. Visit Pediatric Metabolic Providers for a listing of medical specialists and clinic contact information.