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QJM Advance Access originally published online on December 12, 2006
QJM 2007 100(1):1-9; doi:10.1093/qjmed/hcl129
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© The Author 2006. Published by Oxford University Press on behalf of the Association of Physicians. All rights reserved. For Permissions, please email: journals.permissions@oxfordjournals.org

Autosomal dominant polycystic kidney disease: pathophysiology and treatment

J. Rapoport

Department of Nephrology & Hypertension, Kaplan Medical Center, Rehovot, and the Faculty of Medicine, Hebrew University, Jerusalem, Israel

Address correspondence to Dr J. Rapoport, Department of Nephrology & Hypertension, Kaplan Medical Center, POB 1, Rehovot 76100, Israel. e-mail: jayson_r@clalit.org.il

The first 150 words of the full text of this article appear below.


   Introduction
 
Autosomal dominant polycystic disease (ADPKD) is one of the most common monogenic disorders, and globally is the third most common cause of end-stage kidney disease. Until recently, the causes of this disease remained obscure. However, the past decade has seen enormous advances in the understanding of the pathophysiology and genetics of this condition, and recent studies have suggested the possibility of specific treatment for slowing cyst growth. This review attempts to address three central questions: (i) how cysts are created; (ii) how mutated gene products give rise to cysts; and (iii) the prospects for treatment.


   Cyst creation
 
Cysts usually develop from the collecting duct,1,2 and are highly focal in nature, only developing in a very small proportion of nephrons. They generally increase in size and number throughout life, and encroach on normal renal tissue, causing macrophage infiltration, neovascularization, progressive fibrosis and a slow deterioration in renal function.3,4 The disease only reaches the . . . [Full Text of this Article]


   How do the mutated cell products give rise to cysts?
 

   Prospects for treatment
 
Hypertensive control
Use of statins
C-myc antisense oligonucleotide treatment
EGFR tyrosine kinase inhibitors
Sirolimus
Somatostatin
V2 receptor inhibition

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