5C52.01

ICD-11 MMS 2026-01

Disorders of mitochondrial fatty acid oxidation

Index terms

  • Disorders of mitochondrial fatty acid oxidation
  • Acyl-CoA dehydrogenase deficiencies
  • Short chain acyl-CoA dehydrogenase deficiency
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  • Butyryl-CoA dehydrogenase deficiency
  • SCAD - [Short chain acyl-CoA dehydrogenase] deficiency
  • Medium chain acyl-CoA dehydrogenase deficiency
  • MCAD - [Medium chain acyl-CoA dehydrogenase] deficiency
  • Long chain acyl-CoA dehydrogenase deficiency
  • LCAD - [Long chain acyl-CoA dehydrogenase] deficiency
  • Very long chain acyl-CoA dehydrogenase deficiency
  • VLCAD - [Very long chain acyl-CoA dehydrogenase] deficiency
  • Multiple acyl-CoA dehydrogenase deficiency
  • ETFDH - [Electron transfer flavoprotein ubiquinone oxydoreductase] deficiency
  • Electron transfer flavoprotein deficiency
  • Electron transfer flavoprotein ubiquinone oxydoreductase deficiency
  • Ethylmalonic-adipicaciduria
  • Glutaric acidaemia type 2
  • Glutaric aciduria type 2
  • MADD - [Multiple acyl-CoA dehydrogenase deficiency]
  • Multiple FAD dehydrogenase deficiency
  • Electron transfer flavoprotein deficiency, alpha chain
  • ETF - [Electron transfer flavoprotein] alpha chain deficiency
  • Electron transfer flavoprotein deficiency, beta chain
  • ETF - [Electron transfer flavoprotein] beta chain deficiency
  • Electron transfer flavoprotein-ubiquinone oxidoreductase deficiency
  • ETF-QO - [Electron transfer flavoprotein-ubiquinone oxidoreductase] deficiency
  • Electron transfer flavoprotein dehydrogenase deficiency
  • 3-hydroxyacyl-CoA dehydrogenase deficiencies
  • 3-hydroxyacyl-CoA dehydrogenase deficiency, not otherwise specified
  • Short chain 3-hydroxyacyl-CoA dehydrogenase deficiency
  • 17b-hydroxysteroid dehydrogenase deficiency type 10
  • 2-methyl-3-hydroxybutyryl-CoA dehydrogenase deficiency
  • 3-hydroxy-2-methylbutyryl-CoA dehydrogenase deficiency
  • MHBD - [2-methyl-3-hydroxybutyryl-CoA dehydrogenase] deficiency
  • SCHAD - [Short chain 3-hydroxyacyl-CoA dehydrogenase] deficiency
  • Medium or short chain 3-hydroxyacyl-CoA dehydrogenase deficiency
  • M/SCHAD - [Medium and short chain 3-hydroxyacyl-CoA dehydrogenase] deficiency
  • Long chain 3-hydroxyacyl-CoA dehydrogenase deficiency
  • LCHAD - [Long chain 3-hydroxyacyl-CoA dehydrogenase] deficiency
  • Mitochondrial trifunctional protein deficiency
  • Mitochondrial trifunctional enzyme 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
  • 5C52.02 – Disorders of ketone body metabolism
  • 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 mitochondrial fatty acid oxidation – http://id.who.int/icd/entity/1861994414

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