5C56.32

ICD-11 MMS 2026-01

Mucopolysaccharidosis type 4

Description

Mucopolysaccharidosis type IV (MPS IV) is a lysosomal storage disease belonging to the group of mucopolysaccharidoses, and characterised by spondylo-epiphyso-metaphyseal dysplasia. It exists in two clinically indistinguishable forms, A and B. A deficiency in one of the two enzymes required for the degradation of keratan sulfate (KS) is responsible for the MPS IV subtypes: N-acetylgalactosamine-6-sulfate sulfatase in MPS IVA, and beta-D-galactosidase in MPS IVB.

Index terms

  • Mucopolysaccharidosis type 4
  • Galactosamine-6-sulfatase deficiency
  • Morquio syndrome
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  • Morquio-Brailsford disease
  • Mucopolysaccharidosis type 4A
  • Galactosamide-6-sulfatase deficiency
  • Galactose-6-sulfatase deficiency
  • Morquio syndrome type A
  • Mucopolysaccharidosis type 4B
  • Beta-D-galactosidase deficiency
  • Morquio syndrome type B

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
  • 5C56.33 – Mucopolysaccharidosis type 6
  • 5C56.3Y – Other specified mucopolysaccharidosis
  • 5C56.3Z – Mucopolysaccharidosis, unspecified

Foundation entity

Mucopolysaccharidosis type 4 – http://id.who.int/icd/entity/2078241550

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