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Pediatric Nephrology

, Volume 28, Issue 9, pp 1771–1783 | Cite as

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

  • Max Christoph LiebauEmail author
  • Andreas Lucas Serra
Educational Review

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.

Keywords

Magnetic resonance imaging Polycystic kidney disease Autosomal dominant polycystic kidney disease Autosomal recessive polycystic kidney disease Mammalian target of rapamycin Review Cilia 

Notes

Acknowledgment

We would like to thank Nikolaos Andriopoulos (Cologne) and Jens Koenig (Münster) for helpful comments during the preparation of the manuscript. We thank Bernd Hoppe (Cologne/Bonn) and Lisa Guay-Woodford (Washington) for providing picture material.

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Copyright information

© IPNA 2012

Authors and Affiliations

  1. 1.Department of PediatricsUniversity Hospital of CologneCologneGermany
  2. 2.Nephrology Research Laboratory, Renal Division, Department of Medicine IIUniversity Hospital of CologneCologneGermany
  3. 3.Division of NephrologyUniversity HospitalZürichSwitzerland

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