5C50.4

ICD-11 MMS 2026-01

Disorders of lysine or hydroxylysine metabolism

Index terms

  • Disorders of lysine or hydroxylysine metabolism
  • glutaric aciduria NOS
  • Hyperlysinaemia
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  • Alpha-aminoadipic semialdehyde deficiency
  • Lysine alpha-ketoglutarate reductase deficiency
  • Hyperlysinaemia type 1
  • oxoglutarate reductase deficiency
  • Hyperlysinaemia type 2
  • Saccharopine dehydrogenase deficiency
  • Saccharopinuria
  • 2-aminoadipic aciduria
  • 2-aminoadipic acidaemia
  • 2-ketoadipic aciduria
  • 2-ketoadipic acidaemia
  • 2-oxoadipic acidaemia
  • 2-oxoadipic aciduria
  • Seizures - intellectual deficit due to hydroxylysinuria
  • Hydroxylysine metabolic disorder
  • Hydroxylysinemia
  • Lysine metabolic disorder
  • Pipecolic acidaemia
  • Lysine hyperaminoaciduria
  • Lysine intolerance

Excludes

  • 5C50.E1 – Glutaryl-CoA dehydrogenase deficiency
  • 5C57.0 – Zellweger syndrome
  • 5C57.1 – Refsum disease

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
  • 5C50.5 – 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
  • 5C50.9 – Disorders of ornithine 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 lysine or hydroxylysine metabolism – http://id.who.int/icd/entity/367868681

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