The term polycystic kidneys was first coined by Felix Lejars in 1888 to describe the clinical signs of bulky kidneys following earlier anatomical and pathological descriptions. Polycystic kidney disease (PKD) is now known to encompass not only the autosomal dominant form (ADPKD) but also includes many rare forms. Since the work of Lejars and his contemporaries, the pathogenesis of PKD has continued to provoke much debate and study.1
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