E71.111

Billable

3-methylglutaconic aciduria

3-methylglutaconic aciduria

Status

Billable / Specific

Block

E70-E88

Parent Code

E71.11

Coding Notes

Excludes 1

Codes that cannot be used together with this code (mutual exclusion)

Excludes 2

Conditions not included here, but the patient may have both

Related Codes(2)
E71.110

Isovaleric acidemia

BillableE71.118

Other branched-chain organic acidurias

Billable
Also Known As / Clinical Terms(56)

SNOMED CT

UMLS

Clinical Terms

  • 3-Methylglutaconic aciduria (disorder)
  • 3-methylglutaconic aciduria type VII
  • 3mga (3-Methylglutaconic Aciduria)
  • 3-methylglutaconic aciduria with hearing loss, encephalopathy, Leigh-like syndrome
  • CLBP (ClpB homolog, mitochondrial AAA ATPase chaperonin) deficiency
  • Unclassified 3-methylglutaconic aciduria
  • Dilated cardiomyopathy with ataxia syndrome
  • 3-methylglutaconic aciduria type 7
  • 3-Methylglutaconic aciduria type 4
  • MGA7 - 3-methylglutaconic aciduria type 7
  • AUH-gene related 3-methylglutaconic aciduria type 1
  • 3-methylglutaconic aciduria type 5
  • 3-Methylglutaconic aciduria with normal 3-methylglutaconyl-coenzyme A hydratase activity
  • 3-methylglutaconic aciduria type IV with sensorineural deafness, encephalopathy and Leigh-like syndrome
  • 3-Methylglutaconyl-CoA hydratase deficiency with 3-methylglutaconic acid
  • 3-Methylglutaconic aciduria type 1
  • 3-methylglutaconic aciduria, cataract, neurologic involvement, neutropenia syndrome
  • 3-Methylglutaconic aciduria with normal 3-methylglutaconyl-CoA hydratase activity
  • 3-methylglutaconic aciduria type 8
  • 3-methylglutaconic aciduria type 9
  • MEGDEL syndrome
  • 3-Methylglutaconic aciduria type 3
  • 3-Methylglutaconicaciduria
  • 3-methylglutaconic aciduria with deafness, encephalopathy, Leigh-like syndrome
  • Costeff syndrome
  • 3-methylglutaconic aciduria, epilepsy, spasticity, severe intellectual disability syndrome
Frequently Asked Questions
What is the ICD-10 code for 3-methylglutaconic aciduria?

The ICD-10-CM code for 3-methylglutaconic aciduria is E71.111. The full clinical description is "3-methylglutaconic aciduria". E71.111 is a billable/specific code that can be used on insurance claims and medical billing.

What does ICD-10 code E71.111 mean?

ICD-10-CM code E71.111 represents “3-methylglutaconic aciduria”. It is classified under Chapter 4: Endocrine, Nutritional and Metabolic Diseases and is a billable/specific code that can be used on a claim.

Is E71.111 a billable code?

Yes, E71.111 is a billable/specific ICD-10-CM code and can be used to indicate a diagnosis on a medical claim.

What chapter is E71.111 in?

E71.111 is in Chapter 4: Endocrine, Nutritional and Metabolic Diseases (codes E00-E89).

What codes cannot be used with E71.111?

E71.111 has Excludes1 notes indicating codes that cannot be used together with it, including: transitory endocrine and metabolic disorders specific to newborn (P70-P74); androgen insensitivity syndrome (E34.5-); congenital adrenal hyperplasia (E25.0); and 3 more.

What SNOMED CT codes does E71.111 map to?

E71.111 maps to 11 SNOMED CT concepts: 237950009, 237951008, 297232009, 297233004, 237952001, and 6 more. SNOMED CT is a clinical terminology used in electronic health records.

What are the UMLS CUIs for E71.111?

E71.111 is linked to 1 UMLS Concept Unique Identifier: C3696376. The UMLS (Unified Medical Language System) integrates multiple biomedical vocabularies maintained by the U.S. National Library of Medicine.

How does E71.111 relate to ICF functioning codes?

ICF (International Classification of Functioning, Disability and Health) codes describe how conditions like 3-methylglutaconic aciduria affect a person's functioning: body functions, activities, participation, and environmental factors. AutoICD provides ICF Core Sets for 12+ conditions and can map clinical text to ICF categories automatically. Browse the ICF directory to explore functioning codes.

What is the ICD-11 equivalent of E71.111?

There is no direct ICD-11 mapping available for E71.111 in the WHO crosswalk tables. This may mean the concept is classified differently in ICD-11. Use the ICD-10 to ICD-11 converter to search for related codes.

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Includes SNOMED Clinical Terms® (SNOMED CT®) used by permission of SNOMED International. Includes content from the UMLS Metathesaurus, courtesy of the U.S. National Library of Medicine.