2027 ICD-10-CM Diagnosis Code E71.313Glutaric aciduria type II
ICD-10-CM Codes›E00–E89›E70-E88›E71
- Billable — Valid for Submission
- CC — Complication or Comorbidity
- Chronic Condition
E71.313 is a billable ICD-10-CM diagnosis code for glutaric aciduria type II. It is valid on HIPAA claims for fiscal year 2027 (October 1, 2026 through September 30, 2027) and groups to MS-DRG 642. As a secondary diagnosis, it counts as a complication or comorbidity (CC) and moves an inpatient stay to a higher severity level within its MS-DRG family. It does not count, however, when the principal diagnosis is one of 39 closely related codes. Coders also document this condition as alpha chain electron transfer flavoprotein deficiency. In AHRQ's Clinical Classifications Software (CCSR), this diagnosis falls under Other specified and unspecified nutritional and metabolic disorders.
E71.313 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
E71.313 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.
- Alpha chain electron transfer flavoprotein deficiency
- Beta chain electron transfer flavoprotein deficiency
- Electron transfer flavoprotein-ubiquinone oxidoreductase deficiency
- Glutaric aciduria
- Glutaric aciduria, type 2
Instructional NotesGuidance
Instructions from the official ICD-10-CM Tabular List that apply to E71.313: its own notes plus those printed at E71.3, block E70-E88, and Chapter 4. A note printed at a category, block or chapter applies to every code under it.
Applicable To
Conditions this code is used for: synonyms of the title or, for "other specified" codes, the conditions assigned to it. The list is not exhaustive.
- Glutaric aciduria type II A
- Glutaric aciduria type II B
- Glutaric aciduria type II C
Excludes1
Not coded here: the excluded code is never reported together with this one, unless the two conditions are unrelated.
- glutaric aciduria (type 1) NOS (E72.3)
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.
- carnitine deficiency due to inborn error of metabolism (E71.42)
- 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 E71.313, its category, or a range that includes it.
Excludes1 5
These codes carry an Excludes1 note naming E71.313: they are not reported together with it, unless the two conditions are unrelated.
- E72.3 Disorders of lysine and hydroxylysine metabolismglutaric aciduria type II (E71.313)
- E72 Other disorders of amino-acid metabolismfatty-acid metabolism (E71.3) names E71.3, which includes this code
- 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.
type II E71.313
Clinical ClassificationClinical
AHRQ’s CCSR groups this code into broader clinical categories.
Clinical InformationClinical
Multiple Acyl Coenzyme A Dehydrogenase Deficiency
an autosomal recessive disorder of fatty acid oxidation, and branched chain amino acids (amino acids, branched-chain); lysine; and choline catabolism, that is due to defects in either subunit of electron transfer flavoprotein or its dehydrogenase, electron transfer flavoprotein-ubiquinone oxidoreductase (ec 1.5.5.1).
Patient EducationClinical
Lipid Metabolism Disorders
Metabolism is the process your body uses to make energy from the food you eat. Food is made up of proteins, carbohydrates, and fats. Chemicals in your digestive system (enzymes) break the food parts down into sugars and acids, your body's fuel. Your body can use this fuel right away, or it can store the energy in your body tissues.
Read the full article at MedlinePlus
Courtesy of MedlinePlus, a service of the U.S. National Library of Medicine.
Convert E71.313 to ICD-9-CMHistory
The closest ICD-9-CM equivalents under the General Equivalence Mappings.
Code HistoryHistory
Questions About E71.313Overview
What is the ICD-10 code for glutaric aciduria type II?
The ICD-10-CM code for glutaric aciduria type II is E71.313 (sometimes written as E71313). It is billable on HIPAA-covered claims from October 1, 2026 through September 30, 2027.
Is E71.313 (Disorders of fatty-acid oxidation) a billable code?
Yes. This is a billable ICD-10-CM code, specific enough to report glutaric aciduria type II on HIPAA-covered claims from October 1, 2026 through September 30, 2027.
What MS-DRG does E71.313 group to?
When glutaric aciduria type II 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.
Is E71.313 a CC or MCC?
CMS lists E71.313 as a CC (complication or comorbidity) for FY 2027. Reported as a secondary diagnosis, it moves the inpatient stay to a higher-weighted DRG within its severity family. It does not count when the principal diagnosis is one of the 39 closely related codes in its exclusion list.
Can E71.313 be reported with E72.3?
Not as a rule. The Excludes1 note on E71.313 lists "glutaric aciduria (type 1) NOS (E72.3)". 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).
Does E71.313 risk-adjust for Medicare Advantage payment?
Not for Medicare Advantage. E71.313 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).
