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Course and treatment of autosomal dominant polycystic kidney disease

Authors
Arlene B Chapman, MD
Frederic F Rahbari-Oskoui, MD, MSCR
William M Bennett, MD
Section Editor
Ronald D Perrone, MD
Deputy Editor
Alice M Sheridan, MD

INTRODUCTION

Polycystic kidney disease (PKD) includes inherited diseases that cause an irreversible decline in kidney function. PKD may be inherited as an autosomal dominant or recessive trait. The autosomal dominant form (ADPKD) is the most common genetic cause of chronic kidney disease (CKD) [1,2]. The majority of individuals with PKD eventually require renal replacement therapy [1].

The course and treatment of ADPKD in adults are discussed here. The diagnosis, genetics, and pathogenesis of ADPKD and autosomal recessive PKD in children are discussed elsewhere. (See "Diagnosis of and screening for autosomal dominant polycystic kidney disease" and "Genetics of autosomal dominant polycystic kidney disease and mechanisms of cyst growth" and "Autosomal recessive polycystic kidney disease in children".)

EPIDEMIOLOGY

ADPKD occurs in all races and has a reported prevalence of 1:400 to 1:1000 [3]. ADPKD is the underlying cause of kidney disease in approximately 5 percent of patients who initiate dialysis annually in the United States [4,5].

ADPKD is caused by mutations of either PKD1 (which encodes polycystin-1), on chromosome 16, or PKD2 (which encodes polycystin-2), on chromosome 4 [4]. PKD1 mutations are more common (approximately 85 percent) than PKD2 mutations (approximately 15 percent). The relative frequency of PKD1 and PKD2 mutations depends on the age of the population being studied since patients with PKD2 mutations present at an older age and progress more slowly than patients with PKD1 mutations. As a result, PKD2 mutations may be underreported, compared with PKD1, and the frequency of PKD2 underestimated among populations in which only symptomatic individuals are tested. This is demonstrated by the following:

PKD1 mutations were present in 85 percent and PKD2 mutation in 15 percent of cases, respectively, in a study of patients who had known PKD [6]. By contrast, the frequency of PKD2 mutations was higher (27 percent) in population-based studies that included individuals who were at risk for ADPKD, but had no evidence of clinical renal disease [3,6].

                               

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Literature review current through: Nov 2016. | This topic last updated: Wed Aug 10 00:00:00 GMT+00:00 2016.
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