Amino Acid DisordersNewborn ScreeningRUSP

Citrullinemia Type I

CIT-I

Citrullinemia type I is a urea cycle disorder caused by argininosuccinate synthetase deficiency, leading to hyperammonemia that can present catastrophically in infancy or later in life. Acute hyperammonemia is a medical emergency requiring specialized metabolic treatment. Screening facilitates presymptomatic diet and medication strategies to reduce ammonia load.

Prevalence
1 in 57,000
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.