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 of fatty substances in major organs such as the liver, lungs, and spleen. Over time, this can lead to complications, as these organs may not be able to function properly. This disease has a wide variety of symptoms that can affect the liver, lung, spleen, blood, as well as the digestive system.
There are three types of ASMD: type A, type A/B, and type B. Codes already exist for type A and type B at subcategory E75.24, Niemann-Pick disease. Therefore, a unique code has been created for type A/B. ASMD type A/B includes patients with disease manifestations in-between type A and type B; the diagnosis is sometimes called intermediate NPD or type A/B ASMD. There can be considerable overlap along the entire ASMD disease spectrum with symptoms ranging in onset, complexity and severity. The creation of code E75.244 will allow accurate reporting of ASMD type A/B and allow further specification of the clinical manifestations and the risks associated with this specific type of ASMD.
NPD types C and D have a different pathophysiology and are not caused by ASMD.
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
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