E76.211

HCC Category Mapping

What This Code Means

Morquio B mucopolysaccharidosis is a rare inherited metabolic disorder similar to Morquio A but caused by a different enzyme deficiency, resulting in skeletal abnormalities and progressive physical limitations.

Coding Tips

• •Morquio B is less common than Morquio A; verify the specific enzyme deficiency is documented to ensure correct code selection
• •Document the degree of skeletal involvement and any systemic complications for complete clinical picture

Clinical Significance

Morquio B mucopolysaccharidosis (MPS IVB) is caused by beta-galactosidase deficiency, leading to keratan sulfate accumulation with a clinical phenotype similar to Morquio A but generally milder. Distinguishing Morquio B is important because it shares an enzyme deficiency with GM1 gangliosidosis, and treatment approaches may differ from Morquio A.

Documentation Requirements

• ✓Specific diagnosis of Morquio B syndrome (MPS IVB) documented
• ✓Confirmatory enzyme assay showing beta-galactosidase deficiency or GLB1 gene mutation analysis
• ✓Documentation distinguishing this from GM1 gangliosidosis (which shares the same enzyme deficiency but has different clinical features)
• ✓Skeletal manifestations and severity assessment
• ✓Current treatment and management plan

Commonly Confused Codes