E78.71
BillableBarth syndrome
Barth syndrome
Coding Notes
Excludes 1
Codes that cannot be used together with this code (mutual exclusion)
- •transitory endocrine and metabolic disorders specific to newbornP70-P74
- •androgen insensitivity syndromeE34.5
- •congenital adrenal hyperplasiaE25.0
- •hemolytic anemias attributable to enzyme disordersD55
- •Marfan syndromeQ87.4
- •5-alpha-reductase deficiencyE29.1
- •sphingolipidosisE75.0-E75.3
- •Niemann-Pick disease type CE75.242
Related Codes(3)
Related Conditions
Also Known As / Clinical Terms(66)
UMLS
- 3 Methylglutaconic Aciduria, Type IIC0574083
- 3 Methylglutaconicaciduria Type 2C0574083
- 3 methylglutaconic aciduria, type IIC0574083
- 3-METHYLGLUTACONIC ACIDURIA, TYPE IIC0574083
- 3-Methylglutaconic Aciduria Type 2C0574083
- 3-Methylglutaconic Aciduria, Type IIC0574083
- 3-Methylglutaconic aciduria type 2C0574083
- 3-Methylglutaconic aciduria type 2 (disorder)C0574083
- 3-Methylglutaconicaciduria Type 2C0574083
- 3-Methylglutaconicaciduria Type 2sC0574083
- 3-Methylglutaconicaciduria Type IIC0574083
- 3-Methylglutaconicaciduria Type IIsC0574083
- 3-methylglutaconic aciduria type 2C0574083
- BARTH SYNDROMEC0574083
- BTHSC0574083
- Barth SyndromeC0574083
- Barth syndromeC0574083
- CARDIOSKELETAL MYOPATHY WITH NEUTROPENIA AND ABNORMAL MITOCHONDRIAC0574083
- Cardioskeletal Myopathy with Neutropenia and Abnormal MitochondriaC0574083
- Cardioskeletal myopathy with neutropenia and abnormal mitochondriaC0574083
- DNAJC19 defectC0574083
- MGA Type 2C0574083
- MGA Type 2sC0574083
- MGA Type IIC0574083
- MGA Type IIsC0574083
- MGA type 2C0574083
- MGA type IIC0574083
- MGA, TYPE IIC0574083
- MGA2C0574083
- MGCA2C0574083
- Syndrome, BarthC0574083
- Type 2, 3-MethylglutaconicaciduriaC0574083
- Type 2, MGAC0574083
- Type 2s, MGAC0574083
- Type II, MGAC0574083
- Type IIs, MGAC0574083
- barth syndromeC0574083
- barths syndromeC0574083
Clinical Terms
- MGA type 2
- Type 2s, MGA
- Type II, MGA
- barths syndrome
- 3-Methylglutaconic aciduria type 2 (disorder)
- 3-METHYLGLUTACONIC ACIDURIA, TYPE II
- Type 2, 3-Methylglutaconicaciduria
- 3 Methylglutaconicaciduria Type 2
- Cardioskeletal Myopathy with Neutropenia and Abnormal Mitochondria
- Type IIs, MGA
- 3 Methylglutaconic Aciduria, Type II
- 3-Methylglutaconicaciduria Type 2
- 3-Methylglutaconicaciduria Type IIs
- 3-Methylglutaconicaciduria Type 2s
- 3-Methylglutaconicaciduria Type II
- BTHS
- 3-methylglutaconic aciduria type 2
- MGA Type 2s
- MGA Type II
- MGA2
- DNAJC19 defect
- Syndrome, Barth
- MGCA2
- MGA, TYPE II
- Type 2, MGA
- MGA Type IIs
Frequently Asked Questions
What is the ICD-10 code for barth syndrome?
The ICD-10-CM code for barth syndrome is E78.71. The full clinical description is "Barth syndrome". E78.71 is a billable/specific code that can be used on insurance claims and medical billing.
What does ICD-10 code E78.71 mean?
ICD-10-CM code E78.71 represents “Barth syndrome”. 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 E78.71 a billable code?
Yes, E78.71 is a billable/specific ICD-10-CM code and can be used to indicate a diagnosis on a medical claim.
What chapter is E78.71 in?
E78.71 is in Chapter 4: Endocrine, Nutritional and Metabolic Diseases (codes E00-E89).
What codes cannot be used with E78.71?
E78.71 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 5 more.
What SNOMED CT codes does E78.71 map to?
E78.71 maps to 1 SNOMED CT concept: 297231002. SNOMED CT is a clinical terminology used in electronic health records.
What are the UMLS CUIs for E78.71?
E78.71 is linked to 1 UMLS Concept Unique Identifier: C0574083. The UMLS (Unified Medical Language System) integrates multiple biomedical vocabularies maintained by the U.S. National Library of Medicine.
How does E78.71 relate to ICF functioning codes?
ICF (International Classification of Functioning, Disability and Health) codes describe how conditions like barth syndrome 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 E78.71?
There is no direct ICD-11 mapping available for E78.71 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.