5C58.01

ICD-11 MMS 2026-01

Gilbert syndrome

Description

Gilbert's syndrome is an inherited liver disorder characterised by jaundice due to unconjugated hyperbilirubinemia, resulting from a partial deficiency in hepatic bilirubin glucuronosyltransferase activity.

Index terms

  • Gilbert syndrome
  • Gilbert cholaemia
  • Gilbert disease
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  • Gilbert--Lereboullet syndrome
  • Hyperbilirubinaemia type 1
  • Meulengracht icterus
  • Meulengracht syndrome
  • benign unconjugated bilirubinaemia syndrome
  • cholaemia familiaris simplex
  • chronic intermittent juvenile jaundice
  • congenital familial cholaemia
  • constitutional hepatic dysfunction
  • constitutional hyperbilirubinaemia (in part)
  • familial cholaemia
  • familial nonhaemolytic bilirubinaemia
  • familial nonhaemolytic jaundice
  • hereditary nonhaemolytic bilirubinaemia
  • hereditary nonhaemolytic jaundice
  • icterus intermittens juvenilis
  • idiopathic hyperbilirubinaemia (in part)
  • idiopathic unconjugated hyperbilirubinaemia
  • low-grade chronic hyperbilirubinaemia syndrome
  • physiologic cholaemia

Exclusions from above levels

  • http://id.who.int/icd/entity/1191191920 – Disorders of lipoprotein metabolism or certain specified lipidaemias
  • 3A10.Z – Hereditary haemolytic anaemia due to enzyme deficiency
  • 5A71.01 – Congenital adrenal hyperplasia
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  • 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

  • 5C58.00 – Crigler-Najjar syndrome
  • 5C58.02 – Dubin-Johnson syndrome
  • 5C58.03 – Progressive familial intrahepatic cholestasis
  • 5C58.04 – Benign recurrent intrahepatic cholestasis
  • 5C58.0Y – Other specified disorders of bilirubin metabolism or excretion
  • 5C58.0Z – Disorders of bilirubin metabolism or excretion, unspecified

Foundation entity

Gilbert syndrome – http://id.who.int/icd/entity/1947520963

Part of: 5C58.0: Disorders of bilirubin metabolism or excretion › 5C58: Inborn errors of porphyrin or heme metabolism › 05: Endocrine, nutritional or metabolic diseases