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

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 rel...

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Bibliographic Details
Published in:Clinical journal of the American Society of Nephrology 2006-01, Vol.1 (1), p.148-157
Main Authors: Grantham, Jared J, Chapman, Arlene B, Torres, Vicente E
Format: Article
Language:English
Subjects:
Disease Progression
Humans
Organ Size
Polycystic Kidney, Autosomal Dominant - complications
Polycystic Kidney, Autosomal Dominant - etiology
Polycystic Kidney, Autosomal Dominant - pathology
Prognosis
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Summary: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.
ISSN:1555-9041
1555-905X
DOI:10.2215/CJN.00330705