5C56.21

ICD-11 MMS 2026-01

Oligosaccharidosis

Index terms

  • Oligosaccharidosis
  • Aspartylglucosaminuria
  • Aspartylglucosaminidase deficiency
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  • Alpha-mannosidosis
  • Lysosomal alpha-D-mannosidase deficiency
  • Infantile alpha-mannosidosis
  • Adult alpha-mannosidosis
  • Beta-mannosidosis
  • Lysosomal beta-mannosidase deficiency
  • Mannosidosis, not otherwise specified
  • mannosidase deficiency
  • Fucosidosis
  • Alpha-L-fucosidase deficiency
  • Sialidosis
  • Mucolipidosis type 1
  • Sialidosis type 1
  • Cherry-red-spot-myoclonus syndrome
  • Lipomucopolysaccharidosis
  • Normomorphic sialidosis
  • Sialidosis type 2
  • Infantile dysmorphic sialidosis
  • Congenital sialidosis type 2
  • Juvenile sialidosis type 2
  • Alpha-N-acetylgalactosaminidase deficiency
  • N-acetyl-alpha-D-galactosaminidase deficiency
  • NAGA - [Alpha-N-acetylgalactosaminidase] deficiency
  • Alpha-N-acetylgalactosaminidase deficiency type 1
  • NAGA deficiency type 1 - [Alpha-N-acetylgalactosaminidase deficiency type 1]
  • Schindler disease
  • Schindler disease type 1
  • Alpha-N-acetylgalactosaminidase deficiency type 2
  • Adult-onset Alpha-N-acetylgalactosaminidase deficiency
  • Kanzaki disease
  • NAGA deficiency type 2 - [Alpha-N-acetylgalactosaminidase deficiency type 2]
  • Schindler disease type 2
  • Alpha-N-acetylgalactosaminidase deficiency type 3
  • NAGA deficiency type 3 - [Alpha-N-acetylgalactosaminidase deficiency type 3]
  • Schindler disease type 3
  • Galactosialidosis
  • Combined deficiency of neuraminidase and beta-galactosidase
  • Goldberg syndrome
  • Neuraminidase deficiency with beta-galactosidase deficiency
  • Protective protein 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

  • 5C56.20 – Mucolipidosis
  • 5C56.2Y – Other specified glycoproteinosis
  • 5C56.2Z – Glycoproteinosis, unspecified

Foundation entity

Oligosaccharidosis – http://id.who.int/icd/entity/1805681916

Part of: 5C56.2: Glycoproteinosis › 5C56: Lysosomal diseases › 05: Endocrine, nutritional or metabolic diseases