E71.542

HCC Category Mapping

What This Code Means

A group of rare inherited metabolic disorders affecting peroxisome function, classified as group 3 peroxisomal disorders with various clinical presentations.

Coding Tips

• •Use this code only when the specific group 3 peroxisomal disorder is not further specified
• •Requires documentation of genetic testing or metabolic studies confirming peroxisomal dysfunction

Clinical Significance

Other group 3 peroxisomal disorders encompass rare conditions involving defects in both peroxisome biogenesis and single-enzyme function, representing a hybrid category of peroxisomal dysfunction. These conditions present with variable combinations of neurological, skeletal, and metabolic abnormalities depending on the specific genetic defect. Diagnosis requires specialized peroxisomal function studies and genetic testing.

Documentation Requirements

• ✓Document specific biochemical abnormalities identified, peroxisomal function testing results, genetic testing findings, clinical manifestations across affected organ systems, and management approach.
• ✓Record why the condition is classified as group 3 rather than group 1 or 2.

Commonly Confused Codes