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The polycystic kidney diseases are among the most common life-threatening inherited diseases worldwide and frequently cause kidney failure. Autosomal dominant polycystic kidney disease (ADPKD) is seen predominantly in adults (Fig. 284-1), whereas autosomal recessive polycystic kidney disease (ARPKD) is mainly a disease of childhood. Renal cysts also are seen in several other hereditary kidney diseases (Table 284-1), some of which may have defects in a common signaling pathway with ADPKD and ARPKD. Other inherited tubular diseases manifest primarily with alterations in fluid, electrolyte, acid-base, and mineral balance (Table 284-2).
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Autosomal Dominant Polycystic Kidney Disease
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Etiology and Pathogenesis
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