Argininosuccinate Acidemia (ASA)


Argininosuccinic aciduria (ASA) is a condition that causes dangerous amounts of ammonia to build up in the body. ASA is considered an amino acid condition because people with ASA are unable to process a waste product, ammonia, that is produced when the body breaks down amino acids. ASA is also described as a urea cycle condition. This name is used to describe conditions in which the body's system for removing ammonia from the blood is not working correctly. Complications from ASA may include developmental delay and intellectual disability. Early detection and treatment can prevent many of the serious outcomes of ASA.

This condition is also known as: argininosuccinate lyase deficiency, argininosuccinic acid lyase deficiency, argininosuccinic acidemia, argininosuccinyl-CoA lyase deficiency, argininosuccinase deficiency, ASAuria, ASL deficiency.

To learn more about Argininosuccinic Aciduria visit Screening, Technology, and Research in Genetics (STAR-G): Argininosuccinic Acid Lyase Deficiency and Baby's First Test: Argininosuccinic Aciduria. Please see Medically Prescribed Formula for information about medically prescribed formula and pharmacologic interventions for inborn errors of metabolism. Visit Board Certified Metabolic Providers (PDF: 2 pages/204KB) for a listing of medical specialists and clinic contact information.