5C52.02

ICD-11 MMS 2026-01

Disorders of ketone body metabolism

Index terms

  • Disorders of ketone body metabolism
  • 3-hydroxy 3-methylglutaryl-CoA lyase deficiency
  • 3-hydroxy 3-methylglutaryl-CoA synthase deficiency
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  • HMG-CoA lyase deficiency
  • HMG-CoA synthase deficiency
  • Succinyl-CoA acetoacetate transferase deficiency
  • Succinyl-CoA:3-ketoacid CoA transferase (SCOT) deficiency

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

  • 5C52.00 – Disorders of carnitine transport or the carnitine cycle
  • 5C52.01 – Disorders of mitochondrial fatty acid oxidation
  • 5C52.03 – Sjögren-Larsson syndrome
  • 5C52.0Y – Other specified inborn errors of fatty acid oxidation or ketone body metabolism
  • 5C52.0Z – Inborn errors of fatty acid oxidation or ketone body metabolism, unspecified

Foundation entity

Disorders of ketone body metabolism – http://id.who.int/icd/entity/1454722246

Part of: 5C52.0: Inborn errors of fatty acid oxidation or ketone body metabolism › 5C52: Inborn errors of lipid metabolism › 05: Endocrine, nutritional or metabolic diseases