1. ICD-10-CM Codes
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2. E00-E89 Endocrine, nutritional and metabolic diseases
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3. E70-E88 Metabolic disorders
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4. E75- Disorders of sphingolipid metabolism and other lipid storage disorders
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5. 2022 ICD-10-CM Diagnosis Code E75.249

2022 ICD-10-CM Diagnosis Code E75.249

Niemann-Pick disease, unspecified

2016 2017 2018 2019 2020 2021 2022 Billable/Specific Code

• E75.249 is a billable/specific ICD-10-CM code that can be used to indicate a diagnosis for reimbursement purposes.
• The 2022 edition of ICD-10-CM E75.249 became effective on October 1, 2021.
• This is the American ICD-10-CM version of E75.249 - other international versions of ICD-10 E75.249 may differ.

• Niemann pick disease
• Sphingomyelin/cholesterol lipidosis

• A group of autosomal recessive disorders in which harmful quantities of lipids accumulate in the viscera and the central nervous system. They can be caused by deficiencies of enzyme activities (sphingomyelin phosphodiesterase) or defects in intracellular transport, resulting in the accumulation of sphingomyelins and cholesterol. There are various subtypes based on their clinical and genetic differences.
• An autosomal recessive inherited lysosomal storage disease characterized by deficiency of sphingomyelinase. It results in the accumulation of sphingomyelin in the liver, spleen, brain, lungs, and bone marrow. Signs and symptoms include hepatosplenomegaly, pancytopenia, ataxia, dystonia, and dementia.
• Caused by deficiency of sphingomyelinase, resulting in accumulation of sphingomyelin in the reticuloendothelial system, mental and physical retardation, enlarged liver and spleen, anemia, and blindness.

• 642 Inborn and other disorders of metabolism

Convert E75.249 to ICD-9-CM

• 2016 (effective 10/1/2015): New code (first year of non-draft ICD-10-CM)
• 2017 (effective 10/1/2016): No change
• 2018 (effective 10/1/2017): No change
• 2019 (effective 10/1/2018): No change
• 2020 (effective 10/1/2019): No change
• 2021 (effective 10/1/2020): No change
• 2022 (effective 10/1/2021): No change

• Deficiency, deficient
  • acid sphingomyelinase (ASMD) E75.249
• Degeneration, degenerative
  • brain (cortical) (progressive) G31.9
    ICD-10-CM Diagnosis Code G31.9

    Degenerative disease of nervous system, unspecified

    2016 2017 2018 2019 2020 2021 2022 Billable/Specific Code
    • in
      • Niemann-Pick disease E75.249
• Niemann-Pick disease or syndrome E75.249

Reimbursement claims with a date of service on or after October 1, 2015 require the use of ICD-10-CM codes.