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ICD10 code of Histidinemia and ICD9 code

What is the ICD10 code for Histidinemia? And the ICD9 code for Histidinemia?

ICD9 and ICD10 codes of Histidinemia

Histidinemia is a rare metabolic disorder characterized by the inability to properly break down histidine, an amino acid. The ICD-10 code for Histidinemia is E70.8. Unfortunately, there is no specific ICD-9 code for Histidinemia as it was replaced by the ICD-10 coding system. It is important to consult with a healthcare professional for accurate diagnosis and appropriate treatment of this condition.


Histidinemia is a rare metabolic disorder characterized by the impaired breakdown of the amino acid histidine. This condition is caused by a deficiency of the enzyme histidase, which is responsible for breaking down histidine into other molecules. As a result, histidine and its byproducts accumulate in the body, leading to various symptoms and complications.

In the International Classification of Diseases, 10th Revision (ICD-10), histidinemia is classified under the E70.0 code. This code specifically denotes "disorders of histidine metabolism," which includes histidinemia. The E70 category encompasses a range of inherited metabolic disorders involving amino acid metabolism.

On the other hand, in the previous version of the classification system, ICD-9, histidinemia is represented by the code 270.3. This code falls under the broader category of "disorders of amino-acid transport and metabolism." It is worth noting that the ICD-9 system has been replaced by the ICD-10 system for medical coding purposes.

Histidinemia is typically diagnosed through newborn screening programs, as early detection allows for prompt management and treatment. Although histidinemia is considered a benign condition in most cases, some individuals may experience developmental delays, intellectual disability, or behavioral issues if left untreated.

Treatment for histidinemia usually involves dietary modifications, such as restricting histidine intake. This can be achieved by following a low-protein diet or by using medical formulas specifically designed for individuals with histidinemia. Regular monitoring and follow-up with healthcare professionals are essential to ensure optimal management and to address any potential complications that may arise.

In conclusion, histidinemia is classified under the ICD-10 code E70.0 and the ICD-9 code 270.3. It is a rare metabolic disorder characterized by the impaired breakdown of histidine, requiring dietary modifications and ongoing medical supervision.
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