2021 ICD-10-CM Diagnosis Code E75.249

Niemann-Pick disease, unspecified

    2016 2017 2018 2019 2020 2021 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 2021 edition of ICD-10-CM E75.249 became effective on October 1, 2020.
  • This is the American ICD-10-CM version of E75.249 - other international versions of ICD-10 E75.249 may differ.
The following code(s) above E75.249 contain annotation back-references
Annotation Back-References
In this context, annotation back-references refer to codes that contain:
  • Applicable To annotations, or
  • Code Also annotations, or
  • Code First annotations, or
  • Excludes1 annotations, or
  • Excludes2 annotations, or
  • Includes annotations, or
  • Note annotations, or
  • Use Additional annotations
that may be applicable to E75.249:
  • E00-E89
    2021 ICD-10-CM Range E00-E89

    Endocrine, nutritional and metabolic diseases

    Note
    • All neoplasms, whether functionally active or not, are classified in Chapter 2. Appropriate codes in this chapter (i.e. E05.8, E07.0, E16-E31, E34.-) may be used as additional codes to indicate either functional activity by neoplasms and ectopic endocrine tissue or hyperfunction and hypofunction of endocrine glands associated with neoplasms and other conditions classified elsewhere.
    Type 1 Excludes
    • transitory endocrine and metabolic disorders specific to newborn (P70-P74)
    Endocrine, nutritional and metabolic diseases
  • E70-E88
    2021 ICD-10-CM Range E70-E88

    Metabolic disorders

    Type 1 Excludes
    • androgen insensitivity syndrome (E34.5-)
    • congenital adrenal hyperplasia (E25.0)
    • Ehlers-Danlos syndromes (Q79.6-)
    • hemolytic anemias attributable to enzyme disorders (D55.-)
    • Marfan's syndrome (Q87.4)
    • 5-alpha-reductase deficiency (E29.1)
    Metabolic disorders
  • E75
    ICD-10-CM Diagnosis Code E75

    Disorders of sphingolipid metabolism and other lipid storage disorders

      2016 2017 2018 2019 2020 2021 Non-Billable/Non-Specific Code
    Type 1 Excludes
    Disorders of sphingolipid metabolism and other lipid storage disorders
  • E75.2
    ICD-10-CM Diagnosis Code E75.2

    Other sphingolipidosis

      2016 2017 2018 2019 2020 2021 Non-Billable/Non-Specific Code
    Type 1 Excludes
    • adrenoleukodystrophy [Addison-Schilder] (E71.528)
    Other sphingolipidosis
Approximate Synonyms
  • Niemann pick disease
  • Sphingomyelin/cholesterol lipidosis
Clinical Information
  • 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.
ICD-10-CM E75.249 is grouped within Diagnostic Related Group(s) (MS-DRG v38.0):
  • 642 Inborn and other disorders of metabolism

Convert E75.249 to ICD-9-CM

Code History
  • 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

Diagnosis Index entries containing back-references to E75.249:

ICD-10-CM Codes Adjacent To E75.249
E75.2 Other sphingolipidosis
E75.21 Fabry (-Anderson) disease
E75.22 Gaucher disease
E75.23 Krabbe disease
E75.24 Niemann-Pick disease
E75.240 …… type A
E75.241 …… type B
E75.242 …… type C
E75.243 …… type D
E75.248 Other Niemann-Pick disease
E75.249 …… unspecified
E75.25 Metachromatic leukodystrophy
E75.26 Sulfatase deficiency
E75.29 Other sphingolipidosis
E75.3 Sphingolipidosis, unspecified
E75.4 Neuronal ceroid lipofuscinosis
E75.5 Other lipid storage disorders
E75.6 Lipid storage disorder, unspecified
E76 Disorders of glycosaminoglycan metabolism
E76.0 Mucopolysaccharidosis, type I
E76.01 Hurler's syndrome

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