AHA Coding Clinic® for ICD-10-CM and ICD-10-PCS - 2021 Issue 4; ICD-10-CM New/Revised Codes

Niemann-Pick disease Type A/B

Subcategory E75.24 Niemann-Pick disease has been further expanded, and a unique code E75.244 Niemann-Pick disease type A/B, has been created. Niemann-Pick disease (NPD) types A and B (and A/B) are also known as acid sphingomyelinase deficiency (ASMD), and these are rare progressive genetic disorders that are potentially life threatening. ASMD results from a deficiency of an enzyme called acid sphingomyelinase, also known as ASM enzyme, which is required to metabolize a fatty substance called sphingomyelin. When the body is unable to make enough ASM enzymes, sphingomyelin cannot be broken down efficiently, which leads to a buildup...

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