E76.3
BillableMucopolysaccharidosis, unspecified
HCC Category Mapping
V28HCC 49 — Lysosomal Storage Disorders
0.226V24HCC 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
Mucopolysaccharidosis, unspecified, is used when a patient has a mucopolysaccharidosis disorder but the specific type cannot be determined from available information.
Coding Tips
- •This is a non-specific code; always attempt to obtain more specific diagnosis information from the provider or medical records
- •Use only when the type of MPS is truly unknown or when diagnostic testing has not yet been completed
Clinical Significance
Mucopolysaccharidosis, unspecified is used when a provider confirms an MPS diagnosis but has not documented the specific type. This code should prompt a query for specificity since each MPS type has distinct treatment options, prognoses, and management requirements. Using the unspecified code may indicate incomplete diagnostic workup.
Documentation Requirements
- ✓Provider statement confirming mucopolysaccharidosis diagnosis
- ✓Clinical findings suggestive of MPS: coarse facial features, skeletal abnormalities, organomegaly, corneal clouding
- ✓Documentation of why specific MPS type is not identified (pending enzyme studies, atypical presentation)
- ✓Urine glycosaminoglycan levels or other screening results
- ✓Plan for further diagnostic evaluation to determine specific MPS type
Commonly Confused Codes
E76.01-E76.03 — MPS I subtypes: use when any MPS I variant is documentedE76.1 — MPS II (Hunter): use when Hunter syndrome is documentedE76.22 — MPS III (Sanfilippo): use when Sanfilippo is documentedE76.210-E76.219 — MPS IV (Morquio): use when Morquio is documentedE76.9 — Glucosaminoglycan metabolism disorder, unspecified: even broader, for GAG disorders not confirmed as MPS
Code Hierarchy
└E76Disorders of glycosaminoglycan metabolism└E76.3Mucopolysaccharidosis, unspecified
└E76.3Mucopolysaccharidosis, unspecified