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Published ahead of print on October 19, 2005
Clin J Am Soc Nephrol 1: 148-157, 2006
© 2006 American Society of Nephrology
doi: 10.2215/CJN.00330705

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Special Features

Volume Progression in Autosomal Dominant Polycystic Kidney Disease: The Major Factor Determining Clinical Outcomes

Jared J. Grantham*, Arlene B. Chapman{dagger}, and Vicente E. Torres{ddagger}

* Kansas University Medical Center, Kansas City, Kansas; {dagger} Emory University, Atlanta, Georgia; and {ddagger} Mayo Clinic College of Medicine, Rochester, Minnesota

Address correspondence to: Dr. Jared J. Grantham, The Kidney Institute, Mailstop 3018, University of Kansas, Kansas City, Kansas 66103. Phone: 913-588-7605; Fax: 913-588-7606; jgrantha{at}kumc.edu

Autosomal dominant polycystic kidney disease (PKD) is a hereditary condition characterized by the progressive enlargement of innumerable renal cysts that contribute to life-altering morbidity early in the course of the disease. Evidence indicates that the rate of increase in kidney volume can be reliably measured by magnetic resonance or computed tomography imaging, thus providing objective means to judge the effectiveness of therapies that are targeted to the aberrant growth of renal tubules. It is now possible, therefore, to monitor the effectiveness of potential therapies on the signature abnormality in autosomal dominant PKD before irreversible damage has been done by the cysts. Evidence accumulated from human cross-sectional and longitudinal studies and longitudinal studies of PKD models in animals provide strong support for the view that reducing the rate of kidney volume enlargement will ameliorate the late-stage development of renal insufficiency.


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