E71.313

HCC Category Mapping

What This Code Means

A rare inherited metabolic disorder where the body cannot properly process certain amino acids and fatty acids, resulting in the accumulation of glutaric acid in the body.

Coding Tips

• •Glutaric aciduria type II is distinct from type I (which has a different code); confirm the type is documented as type II
• •Look for associated complications such as hypoglycemia, cardiomyopathy, or developmental delays that may require additional coding

Clinical Significance

Glutaric aciduria type II (multiple acyl-CoA dehydrogenase deficiency or MADD) is a severe inborn error of metabolism affecting multiple mitochondrial dehydrogenases simultaneously, impairing both fatty acid oxidation and amino acid metabolism. The neonatal-onset form presents with severe hypoglycemia, metabolic acidosis, and often congenital anomalies, while later-onset forms may respond to riboflavin therapy. This is distinct from glutaric aciduria type I, which involves a different enzyme and clinical presentation.

Documentation Requirements

• ✓Document the specific form (neonatal severe, neonatal mild, or late-onset), acylcarnitine profile showing multiple elevated species, urine organic acid results, genetic testing for ETFA/ETFB/ETFDH mutations, and riboflavin responsiveness status.
• ✓Record any congenital anomalies and current treatment regimen.

Commonly Confused Codes