5C56.33 Mucopolysaccharidosis type 6

International Classification of Diseases for Mortality and Morbidity Statistics, 11th Revision, v2026-01

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
• ARSB - [Arylsulfatase B] deficiency
• Arylsulfatase B deficiency
• Maroteaux-Lamy syndrome
• N-acetylgalactosamine 4-sulfatase deficiency

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