5C53.30

ICD-11 MMS 2026-01

Mitochondrial substrate carrier disorders

Index terms

  • Mitochondrial substrate carrier disorders
  • Cardiomyopathy - hypotonia - lactic acidosis
  • Mitochondrial phosphate carrier deficiency
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  • Mitochondrial aspartate glutamate carrier 1 deficiency
  • Mitochondrial glutamate carrier 1 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

  • 5C53.31 – Mitochondrial protein import disorders
  • 5C53.3Y – Other specified disorders of mitochondrial membrane transport
  • 5C53.3Z – Disorders of mitochondrial membrane transport, unspecified

Foundation entity

Mitochondrial substrate carrier disorders – http://id.who.int/icd/entity/1118834100

Part of: 5C53.3: Disorders of mitochondrial membrane transport › 5C53: Inborn errors of energy metabolism › 05: Endocrine, nutritional or metabolic diseases