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International Classification of Disease (ICD)

The International Classification of Diseases (ICD) is the standard diagnostic tool for epidemiology, health management and clinical purposes. This includes the analysis of the general health situation of population groups. It is used to monitor the incidence and prevalence of diseases and other health problems, proving a picture of the general health situation of countries and populations.

ICD is used by physicians, nurses, other providers, researchers, health information managers and coders, health information technology workers, policy-makers, insurers and patient organizations to classify diseases and other health problems recorded on many types of health and vital records, including death certificates and health records. In addition to enabling the storage and retrieval of diagnostic information for clinical, epidemiological and quality purposes, these records also provide the basis for the compilation of national mortality and morbidity statistics by WHO Member States. 


To Find the Barth syndrome ICD-10 Diagnostic Code:

Endocrine, nutritional and metabolic diseases (E00 – E89)

Metabolic disorders (E70 – E88)

Disorders of lipoprotein metabolism and other lipidemias (E78)

Disorders of bile acid and cholesterol metabolism (E78.7)

Barth syndrome = E78.71 (with dot)

Code Type: Diagnosis

Description: Barth syndrome


         Orphanet Classification of Rare Heart Disease

Barth Syndrome: Orpha111



 For information mapping ICD-9 to ICD-10 E7871/E78.71 (Barth Syndrome) or ICD-9 General Equivalence Mapping, please see BSF.

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