5C53

ICD-11 MMS 2026-01

Inborn errors of energy metabolism

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

Child codes

  • 5C53.0 – Disorders of pyruvate metabolism
  • 5C53.1 – Disorders of the citric acid cycle
  • 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

Sibling codes

  • 5C50 – Inborn errors of amino acid or other organic acid metabolism
  • 5C51 – Inborn errors of carbohydrate metabolism
  • 5C52 – Inborn errors of lipid metabolism
  • 5C54 – Inborn errors of glycosylation or other specified protein modification
  • 5C55 – Inborn errors of purine, pyrimidine or nucleotide metabolism
  • 5C56 – Lysosomal diseases
  • 5C57 – Peroxisomal diseases
  • 5C58 – Inborn errors of porphyrin or heme metabolism
  • 5C59 – Inborn errors of neurotransmitter metabolism
  • 5C5A – Alpha-1-antitrypsin deficiency
  • 5C5Y – Other specified inborn errors of metabolism
  • 5C5Z – Inborn errors of metabolism, unspecified

Foundation entity

Inborn errors of energy metabolism – http://id.who.int/icd/entity/266291267

Part of: 05: Endocrine, nutritional or metabolic diseases