E75.249
BillableNiemann-Pick disease, unspecified
HCC Category Mapping
V24HCC 23 — Other Significant Endocrine and Metabolic Disorders
0.230ESRDHCC 23 — Other Significant Endocrine and Metabolic Disorders
0.000RxHCCHCC 41 — Lysosomal Storage Disorders
0.000What This Code Means
Niemann-Pick disease, unspecified is used when a patient has been diagnosed with Niemann-Pick disease but the specific type (A, B, C, or other) has not been determined or documented.
Coding Tips
- •This is a default code; attempt to obtain clarification on the specific type of Niemann-Pick disease from the provider
- •Query the physician if type information becomes available during the episode of care to allow for more specific coding
Clinical Significance
Niemann-Pick disease, unspecified, is used when a patient has been diagnosed with Niemann-Pick disease but the specific type has not yet been determined through enzyme assays or genetic testing. This code should be temporary, with efforts made to classify the type once diagnostic results are available. The specific type dramatically affects prognosis and treatment options.
Documentation Requirements
- ✓Provider diagnosis of Niemann-Pick disease
- ✓Clinical findings supporting the diagnosis (hepatosplenomegaly, neurological symptoms)
- ✓Documentation of pending or planned enzyme assays and genetic testing
- ✓Reason the specific type is not yet known
- ✓Current symptom management plan while awaiting definitive classification
Commonly Confused Codes
E75.240-E75.244 — Specific Niemann-Pick types: use the appropriate code once the type is confirmedE75.248 — Other Niemann-Pick disease: for confirmed atypical presentations, not pending workupE75.22 — Gaucher disease: also causes hepatosplenomegaly but different enzymeE75.10 — Unspecified gangliosidosis: broader category, not specific to Niemann-PickE88.9 — Metabolic disorder, unspecified: too general when Niemann-Pick is diagnosed