AHA Coding Clinic® for ICD-10-CM and ICD-10-PCS - 2025 Issue 4; New/Revised ICD-10-CM Codes

Disorders of Pyrophosphate Metabolism

Subcategory E83.82, Disorders of pyrophosphate metabolism, was created with new codes to identify specific disorders of pyrophosphate metabolism as follows:E83.820 Generalized arterial calcification of infancy with unspecified genetic causalityE83.821 ENPP1 deficiency causing generalized arterial calcification of infancyE83.822 ENPP1 deficiency causing autosomal recessive hypophosphatemic rickets type 2E83.823 ABCC6 deficiency causing generalized arterial calcification of infancyE83.824 ABCC6 deficiency causing pseudoxanthoma elasticumE83.825 CD73 deficiency causing arterial calcification Ectonucleotide pyrophosphatase/phosphodiesterase 1 (ENPP1) is a protein involved in the production of pyrophosphate, which is important in preventing the accumulation of abnormal deposits of calcium and other minerals...

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