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Looking at the (w)hole: magnet resonance imaging in polycystic kidney disease


Liebau, Max Christoph; Serra, Andreas Lucas (2013). Looking at the (w)hole: magnet resonance imaging in polycystic kidney disease. Pediatric Nephrology, 28(9):1771-1783.

Abstract

Inherited cystic kidney diseases, including autosomal dominant polycystic kidney disease (ADPKD) and autosomal recessive polycystic kidney disease (ARPKD), are the most common monogenetic causes of end-stage renal disease (ESRD) in children and adults. While ARPKD is a rare and usually severe pediatric disease, the more common ADPKD typically shows a slowly progressive course leading to ESRD in adulthood. At the present time there is no established disease-modifying treatment for either ARPKD or ADPKD. Various therapeutic approaches are currently under investigation, such as V2 receptor antagonists, somatostatins, and mTOR inhibitors. Renal function remains stable for decades in ADPKD, and thus clinically meaningful surrogate markers to assess therapeutic efficacy are needed. Various studies have pointed out that total kidney volume (TKV) is a potential surrogate parameter for disease severity in ADPKD. Recent trials have therefore measured TKV by magnet resonance imaging (MRI) to monitor and to predict disease progression. Here, we discuss novel insights on polycystic kidney disease (PKD), the value of MRI, and the measurement of TKV in the diagnosis and follow-up of PKD, as well as novel emerging therapeutic strategies for ADPKD.

Abstract

Inherited cystic kidney diseases, including autosomal dominant polycystic kidney disease (ADPKD) and autosomal recessive polycystic kidney disease (ARPKD), are the most common monogenetic causes of end-stage renal disease (ESRD) in children and adults. While ARPKD is a rare and usually severe pediatric disease, the more common ADPKD typically shows a slowly progressive course leading to ESRD in adulthood. At the present time there is no established disease-modifying treatment for either ARPKD or ADPKD. Various therapeutic approaches are currently under investigation, such as V2 receptor antagonists, somatostatins, and mTOR inhibitors. Renal function remains stable for decades in ADPKD, and thus clinically meaningful surrogate markers to assess therapeutic efficacy are needed. Various studies have pointed out that total kidney volume (TKV) is a potential surrogate parameter for disease severity in ADPKD. Recent trials have therefore measured TKV by magnet resonance imaging (MRI) to monitor and to predict disease progression. Here, we discuss novel insights on polycystic kidney disease (PKD), the value of MRI, and the measurement of TKV in the diagnosis and follow-up of PKD, as well as novel emerging therapeutic strategies for ADPKD.

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Additional indexing

Item Type:Journal Article, refereed, further contribution
Communities & Collections:04 Faculty of Medicine > University Hospital Zurich > Clinic for Nephrology
Dewey Decimal Classification:610 Medicine & health
Language:English
Date:2013
Deposited On:14 Feb 2014 14:00
Last Modified:05 Apr 2016 17:40
Publisher:Springer
ISSN:0931-041X
Publisher DOI:https://doi.org/10.1007/s00467-012-2370-y
PubMed ID:23239392

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