Nelson Textbook of Pediatrics 22nd Edition | PKD1 (85%, chromosome 16) and PKD2 (15%, chromosome 4) mutations. Enlarged kidneys with macrocysts, hypertension, hematuria, proteinuria, extrarenal: hepatic cysts, intracranial berry aneurysms (5-10%), mitral valve prolapse. Tolvaptan (V2 receptor antagonist) in adults, ACE inhibitors, kidney transplantation.
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