Orphanet: Triple A syndrome

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Triple A syndrome

Disease definition

Triple A syndrome is a very rare multisystem disease characterized by adrenal insufficiency with isolated glucocorticoid deficiency, achalasia, alacrima, autonomic dysfunction and neurodegeneration.


Classification level: Disorder
  • Synonym(s):
    • 2A syndrome
    • 3A syndrome
    • 4A syndrome
    • AAA syndrome
    • Achalasia-addisonianism-alacrima syndrome
    • Adrenal insufficiency-achalasia-alacrima syndrome
    • Allgrove syndrome
    • Double A syndrome
    • Quaternary A syndrome
  • Prevalence: <1 / 1 000 000
  • Inheritance: Autosomal recessive 
  • Age of onset: All ages
  • ICD-10: E27.4
  • OMIM: 231550  615510
  • UMLS: C0271742  C2931084
  • MeSH: C536008  C536009
  • GARD: 457
  • MedDRA: -

Detailed information

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