polycystic kidney disease

• autosomal recessive ("childhood") form:
  • Potter I; incidence 1 : 10000
  • affects both kidneys and liver (related to age of onset)

                             renal cysts     periportal fibrosis
             neonatal            +++                 +
             infantile           ++                  ++
             juvenile            +                   +++
    

• autosomal dominant ("adult") form:
  • Potter III; incidence 1:500
  • no periportal fibrosis
  • cysts in liver (in 50%), pancreas, spleen, lung
  • berry aneurysms (15%)

© 2002, C.E. Kahn, Jr.
2002-04-18