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formation and expansion of cysts in the renal cortex and medulla compression of renal vessels with activation of the renin-angiotensin-aldosterone system (RAAS), ischemia, and destruction of the kidney parenchyma [11] ARPKD Inherited mutation in the PKHD1 gene defective fibrocystin (also called polyductin) cystic dilatation of collecting ducts and bile ducts (intrahepatic and extrahepatic bile ducts) [12] Clinical features ADPKD [1] [11] Clinical manifestations of ADPKD usually directly correlate with the progression of cystic degeneration

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