ICD-10 Code E71.41 – Primary carnitine deficiency (2026): Diagnosis, Symptoms & Billing Guide
2026 ICD-10-CM Diagnosis Code E71.41 – Primary carnitine deficiency
What it is
E71.41 identifies primary carnitine deficiency, an inherited disorder that impairs carnitine transport. This can limit fatty acid use for energy, especially during fasting or illness.
Clinical signs
Clinical features vary; refer to documentation. Commonly reported findings include hypoketotic hypoglycemia, muscle weakness, cardiomyopathy, and fatigue, but presentation may differ by age and severity.
When to use this code
Use E71.41 when the record clearly documents primary carnitine deficiency or a confirmed carnitine transport defect. It may appear in newborn screening follow-up, metabolic genetics evaluations, or problem lists for known inherited disease. Check documentation if the note only says “low carnitine” without a confirmed diagnosis.
Do not use for
Do not use this code for secondary carnitine deficiency from another condition, poor intake, or medication-related depletion. Check documentation when the cause is not explicitly primary.
Coding tip
Confirm that the provider documents the disorder as primary or inherited before assigning E71.41.