E71.42

HCC Category Mapping

What This Code Means

A carnitine deficiency that develops as a secondary consequence of another inherited metabolic disorder that affects the body's ability to produce or use carnitine.

Coding Tips

• •Always code the underlying inborn error of metabolism first, then sequence this code as a secondary diagnosis
• •Common underlying conditions include organic acidemias and fatty acid oxidation disorders

Clinical Significance

Carnitine deficiency occurring as a secondary consequence of other inborn errors of metabolism, such as organic acidemias or fatty acid oxidation disorders, where accumulated metabolites conjugate with and deplete carnitine stores. This secondary depletion exacerbates the underlying metabolic disorder by further impairing fatty acid oxidation. Carnitine supplementation is an important adjunctive therapy for the primary condition.

Documentation Requirements

• ✓Document the underlying inborn error of metabolism causing the secondary carnitine deficiency, plasma carnitine levels showing depletion, acylcarnitine profile demonstrating conjugation pattern, and carnitine supplementation dosage.
• ✓The primary metabolic condition should be coded first.

Commonly Confused Codes