5C56.33 Mucopolysaccharidosis type 6
International Classification of Diseases for Mortality and Morbidity Statistics, 11th Revision, v2024-01
Mucopolysaccharidosis type 6 (MPS VI) is a lysosomal storage disease with progressive multisystem involvement, associated with a deficiency of arylsulfatase B (ASB) leading to the accumulation of dermatan sulfate. The disorder shows a wide spectrum of symptoms from slowly to rapidly progressing forms.
synonyms
- Mucopolysaccharidosis type 6
- N-acetylgalactosamine 4-sulfatase deficiency
- Arylsulfatase B deficiency
- Maroteaux-Lamy syndrome
- ARSB - [Arylsulfatase B] deficiency
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