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ICD10 code of Isovaleric acidemia and ICD9 code

What is the ICD10 code for Isovaleric acidemia? And the ICD9 code for Isovaleric acidemia?

ICD9 and ICD10 codes of Isovaleric acidemia

Isovaleric acidemia is a rare genetic disorder that affects the breakdown of proteins in the body. It is caused by a deficiency of the enzyme isovaleryl-CoA dehydrogenase. The ICD-10 code for isovaleric acidemia is E71.110. In the previous ICD-9 code system, isovaleric acidemia was classified under 277.85. It is important to consult with a healthcare professional for accurate diagnosis and appropriate management of this condition.


Isovaleric acidemia is a rare genetic disorder characterized by the body's inability to break down the amino acid called leucine. This leads to a buildup of isovaleric acid in the blood and tissues, which can be toxic. The International Classification of Diseases, Tenth Revision (ICD-10) provides a specific code for isovaleric acidemia, which is E71.322.

On the other hand, the Ninth Revision of ICD (ICD-9) does not have a specific code for isovaleric acidemia. However, it falls under the broader category of "Other specified disorders of carbohydrate metabolism" with the code 271.8. This category includes various metabolic disorders, including rare conditions like isovaleric acidemia.

It is essential for healthcare professionals to accurately code isovaleric acidemia to ensure proper diagnosis, treatment, and tracking of the condition. The ICD-10 code E71.322 allows for better specificity and identification of this specific disorder, aiding in research, statistical analysis, and healthcare management.

Please note that while I strive to provide accurate and up-to-date information, it is always recommended to consult a healthcare professional or coding specialist for the most current and precise coding guidelines.
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ICD9 and ICD10 codes of Isovaleric acidemia

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