Disorders of ketone metabolism

This code represents a group of metabolic disorders characterized by the body's inability to properly process or utilize ketones, which are alternative fuel sources produced during fat breakdown. These conditions often involve enzymatic deficiencies in the ketone body synthesis or degradation pathways, leading to their accumulation or insufficient production.

Use this code for patients diagnosed with specific inborn errors of metabolism affecting ketone metabolism, such as HMG-CoA lyase deficiency, beta-ketothiolase deficiency, or succinyl-CoA:3-ketoacid CoA transferase deficiency. Documentation should clearly state the specific enzymatic defect or a definitive diagnosis of a disorder of ketone metabolism.

• Do not use this code for diabetic ketoacidosis (DKA), which is a complication of diabetes and has specific codes.
• Ensure the documentation specifies a primary metabolic disorder, not just the presence of ketosis due to other conditions like starvation or uncontrolled diabetes.
• Always review the medical record for any associated neurological, developmental, or systemic manifestations that may require additional coding.