5C53.1

ICD-11 MMS 2026-01

Disorders of the citric acid cycle

Index terms

  • Disorders of the citric acid cycle
  • Krebs cycle disorders
  • TCA - [Tricarboxylic acid] cycle disorders
Show allHide
  • Tricarboxylic acid cycle disorders
  • Fumarase deficiency
  • Fumaric aciduria
  • Alpha-ketoglutarate dehydrogenase deficiency
  • 2-oxoglutarate deficiency
  • Oxoglutaric aciduria
  • Aconitase 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
Show allHide
  • 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

  • 5C53.0 – Disorders of pyruvate metabolism
  • 5C53.2 – Disorders of mitochondrial oxidative phosphorylation
  • 5C53.3 – Disorders of mitochondrial membrane transport
  • 5C53.4 – Disorders of creatine metabolism
  • 5C53.Y – Other specified inborn errors of energy metabolism
  • 5C53.Z – Inborn errors of energy metabolism, unspecified

Foundation entity

Disorders of the citric acid cycle – http://id.who.int/icd/entity/658415697

Part of: 5C53: Inborn errors of energy metabolism › 05: Endocrine, nutritional or metabolic diseases