Organic Acid DisordersNewborn ScreeningRUSP

Propionic Acidemia

PA

Propionic acidemia blocks metabolism of certain amino acids and odd-chain fatty acids, causing organic acid accumulation and life-threatening acidosis. Episodes may be triggered by catabolic stress such as infection or fasting. Early dietary protein management, carnitine, and emergency protocols reduce morbidity and mortality.

Prevalence
1 in 100,000
Inheritance
Autosomal recessive
Screened at Birth
Yes
On RUSP
Yes (Recommended Uniform Screening Panel)

About This Data

This disorder is part of the Cureledger rare disease data corpus. We are building privacy-preserving natural history datasets and advocacy tools for rare disease communities. Data is maintained by clinical advisors and the patient community.