ADPKD


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  • Clinical

    • age 30-40s
    • bilateral, enlarged kidneys
    • hepatic cysts, berry aneurysms

  • Histology

    • cysts
    • lining: single layer, flat to cuboidal
    • content: protein, blood, calcifications
    • parenchyma: varying degrees of interstitial fibrosis, lymphoid infiltrate, tubular atrophy, glomerulosclerosis, vascular sclerosis
    • can have papillary tufts, polyps

  • Molecular

    • PKD1, 16p13.3, polycystin1
    • PKD2, 4q21-23, polycystin2

  • Ddx

    • ACKD
      • renal failure from another cause
      • small kidney
      • lack hyperplastic foci or polyps
    • multiple simple cysts
    		•