ICD-10 Code E71.314 – Muscle carnitine palmitoyltransferase deficiency (2026): Diagnosis, Symptoms & Billing Guide
2026 ICD-10-CM Diagnosis Code E71.314 – Muscle carnitine palmitoyltransferase deficiency
What it is
This code identifies a hereditary disorder of fatty acid metabolism affecting skeletal muscle. It reflects deficiency of muscle carnitine palmitoyltransferase, which impairs energy use during prolonged exercise or fasting.
Clinical signs
Clinical features vary; refer to documentation. Typical findings include exercise-induced muscle pain, weakness, cramps, and episodes of myoglobinuria or rhabdomyolysis, especially after prolonged exertion, fasting, or illness.
When to use this code
Use E71.314 when the provider documents muscle carnitine palmitoyltransferase deficiency or CPT II deficiency affecting muscle. It is appropriate for confirmed inherited metabolic disease and for encounters focused on its evaluation or follow-up. Check documentation if the record only notes nonspecific myopathy.
Do not use for
Do not use this code for general muscle pain, cramps, or rhabdomyolysis without documented CPT deficiency. Also avoid it when the chart specifies a different fatty acid oxidation disorder.
Coding tip
Code the documented inherited disorder, not the acute muscle episode alone, and confirm whether the provider specifies muscle involvement.