E71.128

HCC Category Mapping

What This Code Means

A group of rare inherited metabolic disorders affecting how the body processes propionate, a byproduct of amino acid and fat metabolism.

Coding Tips

• •Use this code when a specific propionate metabolism disorder is not identified
• •Review documentation for any identified specific disorders that would warrant a more specific code

Clinical Significance

This code captures disorders of propionate metabolism not classified as propionic acidemia, including conditions like holocarboxylase synthetase deficiency and biotinidase deficiency affecting propionate pathway function. These conditions impair the body's ability to process propionate from protein and fatty acid metabolism, leading to organic acid accumulation. Clinical presentations vary but can include metabolic acidosis, neurological symptoms, and developmental concerns.

Documentation Requirements

• ✓Document the specific propionate metabolism disorder identified, enzyme activity levels or genetic testing results, urine organic acid profiles, and clinical manifestations.
• ✓Record treatment approach including dietary modifications and any vitamin or cofactor supplementation.

Commonly Confused Codes