E72.22
BillableArginosuccinic aciduria
HCC Category Mapping
V24HCC 23 — Other Significant Endocrine and Metabolic Disorders
0.230ESRDHCC 23 — Other Significant Endocrine and Metabolic Disorders
0.000RxHCCHCC 43 — Other Significant Endocrine and Metabolic Disorders
0.000What This Code Means
A rare genetic disorder where the body cannot properly break down the amino acid arginine, causing accumulation of arginosuccinic acid.
Coding Tips
- •This is one of the more common urea cycle disorders; document any neurological complications or developmental delays
- •Monitor ammonia levels as part of ongoing management; document treatment with arginine or other therapies
Clinical Significance
Arginosuccinic aciduria (argininosuccinate lyase deficiency) is the second most common urea cycle disorder, characterized by accumulation of argininosuccinic acid in blood, urine, and cerebrospinal fluid. Patients present with neonatal-onset or late-onset hyperammonemia, but uniquely among urea cycle disorders, also frequently develop chronic hepatic disease with fibrosis and trichorrhexis nodosa (brittle, fragile hair). Long-term management requires dietary protein restriction and arginine supplementation.
Documentation Requirements
- ✓Document plasma argininosuccinic acid levels, ammonia levels, ASL gene mutation analysis, hepatic function including assessment for fibrosis, hair examination for trichorrhexis nodosa, developmental assessment, and dietary management plan.
- ✓Record arginine supplementation dosage and nitrogen scavenger therapy use.
Commonly Confused Codes
E72.21 (Argininemia) for arginase deficiencyE72.23 (Citrullinemia) for argininosuccinate synthetase deficiencyE72.20 (Disorder of urea cycle metabolism, unspecified) which lacks specificityK74.6 (Other and unspecified cirrhosis of liver) when hepatic disease is coded without the underlying metabolic cause.
Code Hierarchy
└E72Other disorders of amino-acid metabolism└E72.2Disorders of urea cycle metabolism└E72.22Arginosuccinic aciduria
└E72.22Arginosuccinic aciduria