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Primary pigmented nodular adrenocortical disease

Disease definition

Primary pigmented nodular adrenocortical disease (PPNAD) is a form of bilateral adrenocortical hyperplasia that is often associated with adrenocorticotrophin hormone (ACTH) independent Cushing syndrome (see this term) and is characterized by small to normal sized adrenal glands containing multiple small cortical pigmented nodules (less than 1 cm in diameter).

ORPHA:189439

  • Synonym(s):
    • PPNAD
  • Prevalence: <1 / 1 000 000
  • Inheritance: Autosomal dominant 
  • Age of onset: All ages
  • ICD-10: E24.8
  • OMIM: 610475  610489  614190  615830
  • UMLS: -
  • MeSH: -
  • GARD: 10906
  • MedDRA: -

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