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%)

Charles E. Kahn, Jr., MD - 2 February 1995
Last updated: 1 September 2006