Short answer · Medically reviewed summary · Last updated: 2026-04-07
The primary ICD-10-CM code for Niemann-Pick disease is E75.24, while the corresponding ICD-9-CM code is 272.7. As a physician working with patients affected by this condition, I understand that navigating the complexities of medical coding is often the first step in coordinating care for Niemann-Pick disease. While E75.24 is the standard billing code, it is important to recognize that Niemann-Pick disease is a heterogeneous group of lysosomal storage disorders characterized by the accumulation of lipids in various organs.
1 people with Niemann-Pick Disease have shared their first-person experience on this question at DiseaseMaps.
ICD10 code of Niemann-Pick Disease and ICD9 code
ICD-10 and ICD-9 codes for Niemann-Pick Disease, with classification details for clinicians, coders and patients.
The primary ICD-10-CM code for Niemann-Pick disease is E75.24, while the corresponding ICD-9-CM code is 272.7.
As a physician working with patients affected by this condition, I understand that navigating the complexities of medical coding is often the first step in coordinating care for Niemann-Pick disease. While E75.24 is the standard billing code, it is important to recognize that Niemann-Pick disease is a heterogeneous group of lysosomal storage disorders characterized by the accumulation of lipids in various organs. Because the clinical presentation varies significantly between Type A, Type B, and the Type C variants, your medical team may also utilize more specific documentation to reflect the systemic impact on the liver, spleen, lungs, and central nervous system.
Understanding Clinical Classification
In clinical practice, we distinguish between these types because they require vastly different management strategies:
- Type A and B: These are classified as acid sphingomyelinase deficiency (ASMD).
- Type C: This is a distinct neurovisceral disorder caused by mutations in the NPC1 or NPC2 genes, which impairs cholesterol trafficking.
Accurate coding for Niemann-Pick disease is essential not just for administrative purposes, but to ensure that insurance providers and healthcare systems recognize the high-intensity, multidisciplinary care required for patients. Whether you are managing the visceral symptoms of Niemann-Pick disease or the progressive neurological challenges associated with Type C, having precise coding ensures your medical records reflect the true nature of the diagnosis.
If you or a loved one are struggling with the emotional burden of navigating these diagnostic codes, please remember that you are not alone; our community at DiseaseMaps.org provides a space to share experiences with others who truly understand the journey of living with a rare metabolic condition. We are here to support you through the clinical, logistical, and emotional aspects of this diagnosis.
Medical Disclaimer: This information is for educational purposes only and does not constitute medical advice, diagnosis, or treatment. Always seek the advice of your physician or other qualified health provider with any questions you may have regarding a medical condition.
References
- NIH Genetic and Rare Diseases Information Center (GARD)
- Orphanet: The portal for rare diseases and orphan drugs
- Online Mendelian Inheritance in Man (OMIM)
- National Niemann-Pick Disease Foundation (NNPDF)
Posted Jan 23, 2019 by pamela carlin 1700
