Disorders of sphingolipid metabolism and other lipid storage disorders
In Metabolic disorders (E70-E88), chapter 4: Endocrine, Nutritional and Metabolic Diseases
26 billable codes under E75: 14 are a CC; 2 map to HCC 49 (Specified Lysosomal Storage Disorders).A CC or MCC (major CC) is a secondary diagnosis that raises the MS-DRG payment. An HCC counts toward a Medicare Advantage patient's risk score.
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Instructional notes
Excludes1: not coded here, use a different code
- mucolipidosis, types I-III (E77.0-E77.1)
- Refsum's disease (G60.1)
Also applies, from Metabolic disorders (E70-E88)
These notes sit on E70-E88 and apply to every code under it, including E75.
Excludes1: not coded here, use a different code
Excludes2: not included here, code separately if applicable
- Ehlers-Danlos syndromes (Q79.6-)
Official coding guidelines
From the ICD-10-CM Official Guidelines for Coding and Reporting, FY 2027 (CMS and NCHS). Links open the PDF at the right page.
Covers diabetes mellitus coding, including type, insulin and drug use, pump malfunction and secondary diabetes, plus obesity and obesity class codes.
- Diabetes mellitus (page 39)
- Obesity (page 42)
All codes under E75
E75 can't be used on a claim. Pick a billable code below; grey headings group them.
- E75.0GM2 gangliosidosis
- E75.00GM2 gangliosidosis, unspecified
- E75.01Sandhoff disease
- E75.02Tay-Sachs disease
- E75.09Other GM2 gangliosidosis
- E75.1Other and unspecified gangliosidosis
- E75.10Unspecified gangliosidosis
- E75.11Mucolipidosis IV
- E75.19Other gangliosidosis
- E75.2Other sphingolipidosis
- E75.21Fabry (-Anderson) disease
- E75.22Gaucher disease
- E75.23Krabbe disease
- E75.24Niemann-Pick disease
- E75.240Niemann-Pick disease type A
- E75.241Niemann-Pick disease type B
- E75.242Niemann-Pick disease type C
- E75.243Niemann-Pick disease type D
- E75.244Niemann-Pick disease type A/B
- E75.248Other Niemann-Pick disease
- E75.249Niemann-Pick disease, unspecified
- E75.25Metachromatic leukodystrophy
- E75.26Sulfatase deficiency
- E75.27Pelizaeus-Merzbacher disease
- E75.28Canavan disease
- E75.29Other sphingolipidosis
- E75.3Sphingolipidosis, unspecified
- E75.4Neuronal ceroid lipofuscinosis
- E75.5Other lipid storage disorders
- E75.6Lipid storage disorder, unspecified
E75 MS-DRGs
As the principal diagnosis, the billable codes under E75 group to MS-DRG 056, 057, or 642 (v44.0). Which one applies depends on procedures performed, secondary diagnoses (CC/MCC), and the patient's age and discharge status.
- DRG 056 Degenerative Nervous System Disorders with MCC (14 of 26 codes)
- DRG 057 Degenerative Nervous System Disorders without MCC (14 of 26 codes)
- DRG 642 Inborn and Other Disorders of Metabolism (12 of 26 codes)
Other codes that refer to E75
Notes on these codes point to E75.
About the condition
Plain-language background from MedlinePlus (National Library of Medicine), not coding guidance.
Genetic Brain Disorders
A genetic brain disorder is caused by a variation or a mutation in a gene. A variation is a different form of a gene. A mutation is a change in a gene. Genetic brain disorders affect the development and function of the brain.
Read more about genetic brain disorders on MedlinePlusCode history
Valid since FY2016, when ICD-10-CM took effect on October 1, 2015. No changes since.
