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We believe that the confusion regarding abnormal renal development could be reduced by more precise terminology. Therefore, we suggest precise definitions for dysgenesis, hypoplasia, dysplasia, hypodysplasia, aplasia and agenesis of the kidney. We suggest the term reflux nephropathy be a generic label for any instance of abnormal renal morphology (gross or microscopic) associated with vesicoureteral reflux. Hypoplasia and hypodysplasia can be subclassified on the basis of associated urological criteria.There have been many previous attempts to classify cystic disease of the kidney but none has been accepted collectively by pathologists, urologists, nephrologists and radiologists. On the basis of known patterns of inheritance, a classification is outlined in which renal cystic disease is divided into 2 major groups: genetic and nongenetic. Each entity is discussed.

Recommendations are made for the standardization of nomenclature describing duplex systems, ectopic ureters and ureteroceles. The elimination of some terms and redefinition of others are proposed in the hope to eliminate the ambiguity and confusion that exist currently.