ICD-10 Code E71.4 – Disorders of carnitine metabolism (2026): Diagnosis, Symptoms & Billing Guide
2026 ICD-10-CM Diagnosis Code E71.4 – Disorders of carnitine metabolism
What it is
E71.4 identifies inherited or acquired disorders that disrupt carnitine handling in the body. These conditions can impair transport of fatty acids into cells and affect energy production, especially during fasting or illness.
Clinical signs
Clinical features vary; refer to documentation. Commonly reported findings include muscle weakness, fatigue, hypoglycemia, cardiomyopathy, or episodes of metabolic decompensation. Some patients are identified through newborn screening or family history.
When to use this code
Use E71.4 when the record documents a disorder of carnitine metabolism, such as primary carnitine deficiency or another specified carnitine-related metabolic defect. Apply it when the diagnosis is confirmed or clearly stated by the clinician. Check documentation if the note only mentions low carnitine without a diagnosis.
Do not use for
Do not use this code for nonspecific weakness, malnutrition, or isolated abnormal laboratory results without a documented metabolic disorder. Do not assume carnitine supplementation alone establishes the diagnosis.
Coding tip
Code the condition only when the provider names a carnitine metabolism disorder; otherwise, query for clarification.