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Abstract
<jats:title>Abstract</jats:title> <jats:p>This chapter outlines a practical approach to the diagnosis and management of genetic conditions with renal malformations. Structural abnormalities of the kidneys are common and can result from a number of etiologies that may have a genetic basis. Appropriate workup and management of an affected patient require an understanding of the different types of renal malformations. Based on the clinical findings, the user is shepherded through a step-by-step approach to a differential diagnosis. Flowchart diagrams graphically depict the diagnostic approach. Suggested laboratory and/or imaging studies are presented. Health supervision and management of the most common conditions are suggested, where appropriate.</jats:p>