Mucopolysaccharidosis III

**Definition:** Mucopolysaccharidosis characterized by HEPARAN SULFATE in the urine, progressive mental retardation, mild dwarfism, and other skeletal disorders. There are four clinically indistinguishable but biochemically distinct forms, each due to a deficiency of a different enzyme.

**Tree numbers:** - C16.320.565.202.715.650 - C16.320.565.595.600.650 - C17.300.550.575.650 - C18.452.648.202.715.650 - C18.452.648.595.600.650

**Synonyms:** - Mucopolysaccharidosis 3 - Polydystrophic Oligophrenia - San Filippo's Syndrome - Sanfilippo Syndrome - Sanfilippo's Syndrome