Orphanet: Acute intermittent porphyria

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Acute intermittent porphyria

Disease definition

A rare, severe form of the acute hepatic porphyrias characterized by the occurrence of neuro-visceral attacks without cutaneous manifestations.


Classification level: Disorder
  • Synonym(s): -
  • Prevalence: 1-9 / 1 000 000
  • Inheritance: Autosomal dominant 
  • Age of onset: Adolescent, Adult
  • ICD-10: E80.2
  • OMIM: 176000
  • UMLS: C0162565
  • MeSH: D017118
  • GARD: 5732
  • MedDRA: -

Detailed information

Article for general public


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