Genetic Aspects of Hydronephrosis Associated with Renal Agenesis

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Abstract
Summary: Two families with hereditary hydronephrosis are reported and a further 4 families form the literature reviewed. In these families the condition may be unilateral or bilateral, and if unilateral, is more common on the left side.Hereditary pelvi‐ureteric obstruction is due to an autosomal dominant gene with variable penetrance. Evidence from animal studies relating to the pathogenesis of pelvi‐ureteric obstruction is discussed.It is suggested that the mild form of the condition may have more clinical significance than has been previously realised. The association of renal agenesis and hereditary hydronephrosis is also noted.