ICD-10-CM 2027
ICD-10 code for carnitine deficiency
From the official ICD-10-CM alphabetic index entry “Deficiency, deficient › carnitine”. Page updated September 29, 2026.
About coding carnitine deficiency
The ICD-10-CM code for carnitine deficiency is E71.40 (Disorder of carnitine metabolism, unspecified). The official index lists 7 more specific codes, so check the documentation for details such as type, cause, site or severity before settling on E71.40.
Within E71.4, choose E71.40 (Disorder of carnitine metabolism, unspecified) only when documentation doesn't support a more specific option: primary carnitine deficiency (E71.41), carnitine deficiency due to inborn errors of metabolism (E71.42), iatrogenic carnitine deficiency (E71.43) and other secondary carnitine deficiency (E71.44).
It is not a CC or MCC, so as a secondary diagnosis it doesn't change the MS-DRG severity level.
In MS-DRG v44.0, it is part of the grouping logic for DRG 642 (Inborn and Other Disorders of Metabolism, relative weight 1.3219), in MDC 10 (Endocrine, Nutritional and Metabolic Diseases and Disorders), as a principal or secondary diagnosis. The DRG a claim lands in depends on the principal diagnosis, procedures and any CC/MCC secondary diagnoses.
More specific carnitine deficiency codes (official index)
- due to
- hemodialysis E71.43 — Iatrogenic carnitine deficiency
- inborn errors of metabolism E71.42 — Carnitine deficiency due to inborn errors of metabolism
- Valproic acid therapy E71.43 — Iatrogenic carnitine deficiency
- iatrogenic E71.43 — Iatrogenic carnitine deficiency
- muscle palmityltransferase E71.314 — Muscle carnitine palmitoyltransferase deficiency
- primary E71.41 — Primary carnitine deficiency
- secondary E71.448 — Other secondary carnitine deficiency
Common questions about carnitine deficiency ICD-10 codes
What is the ICD-10 code for carnitine deficiency?
- E71.40 — Disorder of carnitine metabolism, unspecified. More specific codes apply when the documentation supports them: E71.43, E71.42, E71.43, E71.43 and others below.
What is the ICD-10 code for iatrogenic carnitine deficiency?
- E71.43 — Iatrogenic carnitine deficiency.
What is the ICD-10 code for muscle palmityltransferase carnitine deficiency?
- E71.314 — Muscle carnitine palmitoyltransferase deficiency.
What is the ICD-10 code for primary carnitine deficiency?
- E71.41 — Primary carnitine deficiency.
What is the ICD-10 code for secondary carnitine deficiency?
- E71.448 — Other secondary carnitine deficiency.
Is E71.40 billable?
- Yes. E71.40 is a billable/specific ICD-10-CM code valid for FY2027 (October 1, 2026 – September 30, 2027).
Can E71.40 be used as a principal diagnosis?
- Yes. The Medicare Code Editor doesn't restrict E71.40 as a principal diagnosis.
Is E71.40 a CC or MCC?
- No. E71.40 is neither a CC nor an MCC under MS-DRG v44.0.