5C90.3

ICD-11 MMS 2026-01

Liver disease due to disorders of lysosomal storage

Description

This is liver disease due to a group of approximately 50 rare inherited metabolic disorders that result from defects in lysosomal function.

Index terms

  • Liver disease due to disorders of lysosomal storage

Exclusions from above levels

  • DB92 – Non-alcoholic fatty liver disease
  • DB94 – Alcoholic liver disease
  • DB95 – Drug-induced or toxic liver disease
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  • JA65.0 – Acute fatty liver of pregnancy
  • 3A10.Z – Hereditary haemolytic anaemia due to enzyme deficiency
  • 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

  • 5C90.0 – Liver diseases due to urea cycle defects
  • 5C90.1 – Liver diseases due to disorders of porphyrin or bilirubin metabolism or transport
  • 5C90.2 – Liver diseases due to disorders of amino acid metabolism
  • 5C90.4 – Liver diseases due to mitochondrial disorders
  • 5C90.5 – Liver diseases due to disorders of mineral metabolism
  • 5C90.Y – Other specified metabolic or transporter liver disease
  • 5C90.Z – Metabolic or transporter liver disease, unspecified

Foundation entity

Liver disease due to disorders of lysosomal storage – http://id.who.int/icd/entity/1600152465

Allowed postcoordination axes

Part of: 5C90: Metabolic or transporter liver disease › 05: Endocrine, nutritional or metabolic diseases