E77.1
BillableDefects in glycoprotein degradation
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
A genetic condition where the body cannot properly break down glycoproteins, which are proteins with attached sugar molecules found throughout the body.
Coding Tips
- •Document the specific glycoprotein involved if identified
- •May present with progressive neurological or systemic symptoms; ensure complete clinical picture is documented
Clinical Significance
Defects in glycoprotein degradation encompass conditions such as alpha-mannosidosis, beta-mannosidosis, fucosidosis, sialidosis, and aspartylglucosaminuria, where glycoprotein breakdown within lysosomes is impaired. These rare inherited disorders cause progressive neurological decline and systemic disease requiring lifelong specialist care.
Documentation Requirements
- ✓Specific glycoprotein degradation disorder documented (alpha-mannosidosis, fucosidosis, sialidosis, etc.)
- ✓Confirmatory enzyme assay or genetic testing results
- ✓Current neurological status and cognitive function assessment
- ✓Systemic involvement: hepatosplenomegaly, skeletal abnormalities, immune dysfunction
- ✓Treatment plan including any enzyme replacement therapy (if available) or supportive care
Commonly Confused Codes
E77.0 — Defects in post-translational modification of lysosomal enzymes: different mechanism (enzyme trafficking vs. degradation)E77.8 — Other disorders of glycoprotein metabolism: for conditions that do not fit E77.0 or E77.1E75.19 — Other gangliosidoses: gangliosidoses involve glycolipids, not glycoproteinsE76.01 — Hurler syndrome: similar clinical features but involves GAG storage, not glycoprotein degradation