Amino Acid DisordersNewborn ScreeningRUSP

Homocystinuria

HCY

Classic homocystinuria most often results from cystathionine beta-synthase deficiency and elevates homocysteine and methionine, predisposing to thromboembolism, ectopia lentis, and developmental issues. Treatment may include dietary methionine restriction, betaine, and vitamin B6 responsiveness in some genotypes. Newborn screening allows initiation of therapy in the first weeks of life.

Prevalence
1 in 200,000-300,000
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.