E72.3

HCC Category Mapping

What This Code Means

Metabolic disorders affecting how the body processes lysine and hydroxylysine, two important amino acids.

Coding Tips

• •This category includes conditions like hyperlysinemia and saccharopinuria; document the specific disorder if known
• •These are rare disorders; ensure documentation supports the diagnosis and any associated symptoms

Clinical Significance

Disorders of lysine and hydroxylysine metabolism are rare inborn errors affecting amino acid catabolism that can lead to intellectual disability, seizures, and metabolic crises. These conditions, including hyperlysinemia and saccharopinuria, require ongoing metabolic management and carry significant morbidity risk. Accurate coding ensures appropriate risk adjustment for the complex care these patients require.

Documentation Requirements

• ✓Specific disorder of lysine/hydroxylysine metabolism identified (e.g., hyperlysinemia, saccharopinuria, glutaric aciduria type I)
• ✓Biochemical confirmation via plasma amino acid analysis or urine organic acid testing
• ✓Genetic testing results if available
• ✓Current treatment regimen including dietary restrictions and supplements
• ✓Documentation of associated complications such as developmental delay or seizures

Commonly Confused Codes