5C50.9

ICD-11 MMS 2026-01

Disorders of ornithine metabolism

Index terms

  • Disorders of ornithine metabolism
  • Hyperornithinaemia
  • Generalised gyrate choroid atrophy
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  • HOGA - [hyperornithinaemia - gyrate atrophy of choroid and retina]
  • OAT - [ornithine oxo-acid aminotransferase] deficiency
  • OKT - [ornithine ketoacid transaminase] deficiency
  • Ornithinaemia
  • Ornithine aminotransferase deficiency
  • Ornithine keto-acid transaminase deficiency
  • Ornithine oxo-acid aminotransferase deficiency
  • ornithine-oxo-acid amino acid transferase deficiency
  • Ornithinaemia Type 1
  • Ornithinaemia Type 2
  • Deficiency of citrulline phosphorylase
  • Ornithine hyperaminoaciduria
  • Ornithine-delta-aminotransferase 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

  • 5C50.0 – Phenylketonuria
  • 5C50.1 – Disorders of tyrosine metabolism
  • 5C50.2 – Disorders of histidine metabolism
  • 5C50.3 – Disorders of tryptophan metabolism
  • 5C50.4 – Disorders of lysine or hydroxylysine metabolism
  • 5C50.5 – Disorders of the gamma-glutamyl cycle
  • 5C50.6 – Disorders of serine metabolism
  • 5C50.7 – Disorders of glycine metabolism
  • 5C50.8 – Disorders of proline or hydroxyproline metabolism
  • 5C50.A – Disorders of urea cycle metabolism
  • 5C50.B – Disorders of methionine cycle or sulphur amino acid metabolism
  • 5C50.C – Disorders of beta or omega amino acid metabolism
  • 5C50.D – Disorders of branched-chain amino acid metabolism
  • 5C50.E – Organic aciduria
  • 5C50.F – Disorders of peptide metabolism
  • 5C50.G – Trimethylaminuria
  • 5C50.Y – Other specified inborn errors of amino acid or other organic acid metabolism
  • 5C50.Z – Inborn errors of amino acid or other organic acid metabolism, unspecified

Foundation entity

Disorders of ornithine metabolism – http://id.who.int/icd/entity/1711515805

Part of: 5C50: Inborn errors of amino acid or other organic acid metabolism › 05: Endocrine, nutritional or metabolic diseases