2027 ICD-10-CM Diagnosis Code E71.41Primary carnitine deficiency
ICD-10-CM Codes›E00–E89›E70-E88›E71
- Billable — Valid for Submission
- Chronic Condition
E71.41 is a billable ICD-10-CM diagnosis code for primary carnitine deficiency. It is valid on HIPAA claims for fiscal year 2027 (October 1, 2026 through September 30, 2027) and groups to MS-DRG 642. Coders also document this condition as amino acid deficiency. In AHRQ's Clinical Classifications Software (CCSR), this diagnosis falls under Other specified and unspecified nutritional and metabolic disorders.
E71.41 no longer risk-adjusts for Medicare Advantage: it mapped to HCC 23 under the retired CMS-HCC V24 model through payment year 2025 but maps to no category in the live V28 model. It still risk-adjusts in the PACE (CMS-HCC V22) category 23, ESRD (V21) category 23, ESRD (V24) category 23, and RxHCC Part D (V08) category 43 for payment year 2027.
Code Identity
Code Classification
Medicare Risk Adjustment (HCC)Billing
E71.41 no longer risk-adjusts for Medicare Advantage: it maps to no payment category in the live CMS-HCC V28 model, although it still risk-adjusts in the other CMS models shown below.
Source: CMS Payment Year 2027 risk adjustment mappings and model software. Weights are relative factors, not dollar amounts; a beneficiary's total RAF also includes demographics and interactions. Browse all CMS-HCC categories.
Approximate SynonymsGuidance
Alternate terms and clinical phrases that map to this code.
- Amino acid deficiency
- Carnitine deficiency
- Renal carnitine transport defect
Instructional NotesGuidance
Instructions from the official ICD-10-CM Tabular List that apply to E71.41: its own notes plus those printed at E71.4, block E70-E88, and Chapter 4. A note printed at a category, block or chapter applies to every code under it.
Excludes1
Not coded here: the excluded code is never reported together with this one, unless the two conditions are unrelated.
- Muscle carnitine palmitoyltransferase deficiency (E71.314)
Excludes2
Not included here: the excluded condition is not part of this code, but a patient may have both, so both codes may be reported.
- Ehlers-Danlos syndromes (Q79.6-)
Notes
General instructions on how these codes are used.
- All neoplasms, whether functionally active or not, are classified in Chapter 2. Appropriate codes in this chapter (i.e. E05.8, E07.0, E16-E31, E34.-) may be used as additional codes to indicate either functional activity by neoplasms and ectopic endocrine tissue or hyperfunction and hypofunction of endocrine glands associated with neoplasms and other conditions classified elsewhere.
Source: CMS ICD-10-CM Tabular List. How to read instructional notes.
Referenced in Other NotesGuidance
Instructional notes printed at other codes that name E71.41, its category, or a range that includes it.
Excludes1 3
These codes carry an Excludes1 note naming E71.41: they are not reported together with it, unless the two conditions are unrelated.
- R79 Other abnormal findings of blood chemistry
- R79.83 Abnormal findings of blood amino-acid level
- Block R80-R82 Abnormal findings on examination of urine, without diagnosis
Index to Diseases and InjuriesGuidance
Alphabetical index entries that point to this code.
Clinical ClassificationClinical
AHRQ’s CCSR groups this code into broader clinical categories.
Clinical InformationClinical
Primary Carnitine Deficiency
an autosomal recessive inherited disorder caused by mutations in the slc22a5 gene. it is characterized by the presence of a defective protein called octn2 which is involved in the transportation of carnitine into the cells. this abnormality results in reduced energy production and accumulation of fatty acids in the tissues. clinical manifestations of confusion, muscle weakness, hypoglycemia, encephalopathy and cardiomyopathy may be exacerbated during fasting.
Patient EducationClinical
Metabolic Disorders
Metabolism is the process your body uses to get or make energy from the food you eat. Food is made up of proteins, carbohydrates, and fats. Chemicals in your digestive system break the food parts down into sugars and acids, your body's fuel.
Read the full article at MedlinePlus
Courtesy of MedlinePlus, a service of the U.S. National Library of Medicine.
Convert E71.41 to ICD-9-CMHistory
The closest ICD-9-CM equivalents under the General Equivalence Mappings.
Code HistoryHistory
Questions About E71.41Overview
What is the ICD-10 code for primary carnitine deficiency?
The ICD-10-CM code for primary carnitine deficiency is E71.41 (sometimes written as E7141). It is billable on HIPAA-covered claims from October 1, 2026 through September 30, 2027.
Is E71.41 (Disorders of carnitine metabolism) a billable code?
Yes. This is a billable ICD-10-CM code, specific enough to report primary carnitine deficiency on HIPAA-covered claims from October 1, 2026 through September 30, 2027.
What MS-DRG does E71.41 group to?
When primary carnitine deficiency is the principal diagnosis on an inpatient stay, it groups to MS-DRG 642 (inborn and Other Disorders of Metabolism), which carries a relative weight of 1.3226. Higher weights mean higher Medicare reimbursement.
Can E71.41 be reported with E71.314?
Not as a rule. The Excludes1 note at E71.4, which applies to E71.41, lists "Muscle carnitine palmitoyltransferase deficiency (E71.314)". An Excludes1 note means the excluded code is never reported together with this one; the only exception is when the two conditions are unrelated to each other (Official Guidelines, Section I.A.12.a).
What is the ICD-9 equivalent of E71.41?
Under the General Equivalence Mappings, primary carnitine deficiency converts to ICD-9-CM 277.81 (primary carnitine defncy). The mapping is a direct match.
Does E71.41 risk-adjust for Medicare Advantage payment?
Not for Medicare Advantage. E71.41 mapped to HCC 23 in the retired CMS-HCC V24 model, which last determined payment in 2025, but it maps to no category in the live V28 model; see all codes that no longer risk-adjust. It still risk-adjusts in the PACE (CMS-HCC V22) category 23 (Other Significant Endocrine and Metabolic Disorders), ESRD (V21) category 23 (Other Significant Endocrine and Metabolic Disorders), ESRD (V24) category 23 (Other Significant Endocrine and Metabolic Disorders), and RxHCC Part D (V08) category 43 (Pituitary, Adrenal Gland, and Other Endocrine and Metabolic Disorders).
