E71.32

HCC Category Mapping

What This Code Means

A metabolic disorder affecting the body's ability to produce or use ketones, which are alternative fuel sources the body creates during fasting or low carbohydrate intake.

Coding Tips

• •Determine if the disorder involves ketone production (overproduction) or ketone utilization (deficiency) as this affects clinical management
• •Look for associated conditions like diabetic ketoacidosis or hypoketotic hypoglycemia that may require separate coding

Clinical Significance

Disorders of ketone metabolism affect the body's ability to produce or utilize ketone bodies as alternative fuel during fasting or metabolic stress, potentially causing life-threatening metabolic crises. Conditions include succinyl-CoA:3-oxoacid CoA transferase (SCOT) deficiency and mitochondrial 3-hydroxy-3-methylglutaryl-CoA synthase deficiency. These patients are at risk of severe metabolic acidosis during illness or prolonged fasting.

Documentation Requirements

• ✓Document whether the disorder involves ketone overproduction (ketolysis defect) or underproduction (ketogenesis defect), specific enzyme deficiency if identified, blood ketone and glucose levels during episodes, and management plan for illness and fasting protocols.
• ✓Record any history of metabolic crises.

Commonly Confused Codes