隐性遗传性多囊肾的切面。囊肿相当小,但广泛分布于实质。因此,本病通常表现为对称性,两侧肾脏明显增大。因为它是常染色体隐性遗传的,所以它在家族中的发病率是25%。受累婴儿大多存活不长。
Here is a cut section of a kidney with recessive polycystic kidney disease (RPKD). Note that the cysts are fairly small but uniformly distributed throughout the parenchyma so that the disease is usually symmetrical in appearance, with both kidneys markedly enlarged. The recurrence risk for this disease is, of course, 25% because of the autosomal recessive inheritance pattern. Affected babies usually do not survive long.