5C56.4

ICD-11 MMS 2026-01

Disorders of sialic acid metabolism

Description

This refers to any disorders of the N- or O-substituted derivatives of neuraminic acid, a monosaccharide with a nine-carbon backbone.

Index terms

  • Disorders of sialic acid metabolism
  • Free sialic acid storage disease
  • Finnish type sialuria
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  • Salla disease
  • Sialuria
  • French type sialuria

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

  • 5C56.0 – Sphingolipidosis
  • 5C56.1 – Neuronal ceroid lipofuscinosis
  • 5C56.2 – Glycoproteinosis
  • 5C56.3 – Mucopolysaccharidosis
  • 5C56.Y – Other specified lysosomal diseases
  • 5C56.Z – Lysosomal diseases, unspecified

Foundation entity

Disorders of sialic acid metabolism – http://id.who.int/icd/entity/1709765980

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