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Frasier syndrome

Disease definition

Frasier syndrome is characterised by the association of male pseudohermaphrodism and glomerular nephropathy. This syndrome is associated with a high risk of developing gonadoblastoma.


Classification level: Disorder
  • Synonym(s): -
  • Prevalence: <1 / 1 000 000
  • Inheritance: Autosomal dominant 
  • Age of onset: Childhood
  • ICD-10: N04.1
  • OMIM: 136680
  • UMLS: C0950122
  • MeSH: D052159
  • GARD: 2375
  • MedDRA: -
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