Mucopolysaccharidosis IV

**Definition:** Genetic disorder of mucopolysaccharide metabolism characterized by skeletal abnormalities, joint instability, development of cervical myelopathy, and excessive urinary keratan sulfate. There are two biochemically distinct forms, each due to a deficiency of a different enzyme.

**Tree numbers:** - C16.320.565.202.715.655 - C16.320.565.595.600.655 - C17.300.550.575.655 - C18.452.648.202.715.655 - C18.452.648.595.600.655

**Synonyms:** - Eccentro-Osteochondrodysplasia - Eccentroosteochondrodysplasia - Morquio Disease - Morquio Syndrome - Morquio's Disease - Morquio's Syndrome - Mucopolysaccharidosis 4 - Mucopolysaccharidosis Type IV