5C56.33

ICD-11 MMS 2026-01

Mucopolysaccharidosis type 6

Description

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.

Index terms

  • Mucopolysaccharidosis type 6
  • ARSB - [Arylsulfatase B] deficiency
  • Arylsulfatase B deficiency
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  • Maroteaux-Lamy syndrome
  • N-acetylgalactosamine 4-sulfatase deficiency

Exclusions from above levels

  • 5C51.3 – Glycogen storage disease due to LAMP-2 deficiency
  • http://id.who.int/icd/entity/1191191920 – Disorders of lipoprotein metabolism or certain specified lipidaemias
  • 3A10.Z – Hereditary haemolytic anaemia due to enzyme deficiency
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  • 5A71.01 – Congenital adrenal hyperplasia
  • 5A81.1 – 5-alpha-reductase deficiency
  • LD28.01 – Marfan syndrome
  • LD28.1 – Ehlers-Danlos syndrome
  • LD2A.4 – androgen resistance syndrome
  • 18 – Pregnancy, childbirth or the puerperium
  • http://id.who.int/icd/entity/1004987305 – Transitory endocrine or metabolic disorders specific to fetus or newborn

Sibling codes

  • 5C56.30 – Mucopolysaccharidosis type 1
  • 5C56.31 – Mucopolysaccharidosis type 2
  • 5C56.32 – Mucopolysaccharidosis type 4
  • 5C56.3Y – Other specified mucopolysaccharidosis
  • 5C56.3Z – Mucopolysaccharidosis, unspecified

Foundation entity

Mucopolysaccharidosis type 6 – http://id.who.int/icd/entity/1288379621

Part of: 5C56.3: Mucopolysaccharidosis › 5C56: Lysosomal diseases › 05: Endocrine, nutritional or metabolic diseases