There are many different types of kidney cysts that can be classified in various ways. An ideal classification system would take into account morphological features, pathogenesis, and therapeutic potential. Autosomal dominant polycystic kidney disease (ADPKD) is the most common cystic kidney disease and is characterized by multiple bilateral cysts of varying sizes in the kidneys and liver. It has a prevalence of 1 in 400 to 1,000 people and is caused by mutations in the PKD1 or PKD2 genes.

10. Disease Kidney Size Cyst Size Cyst Location Liver Nephronophthisis Small 1MM-2CM Medullary Normal Acquired Cysts Normal/Small 0.5-3CM Any Normal Medullary Sponge Normal/Enlarged MM Precalyceal Normal ARPKD Enlarged MM Any Fibrosis Multicystic Dysplastic Enlarged 1MM-10CM Any Normal ADPKD Enlarged MM-10CM Any Cysts

74. Classifications of glomerulocystic kidney disease Example Familial nonsyndromic Autosomal dominant polycystic kidney disease in young infants Associated with inheritable malformation syndromes Tuberous sclerosis complex Syndromic, non-Mendelian Trisomy 9, 13, or 18 Sporadic New mutations Acquired and dysplastic kidneys Hemolytic uremic syndrome and obstructive uropathy