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BMC Nephrology
Published in: BMC Nephrology 1/2015

Open Access 01-12-2015 | Research article

A systematic review of the predictors of disease progression in patients with autosomal dominant polycystic kidney disease

Authors: Claire Woon, Ashleigh Bielinski-Bradbury, Karl O’Reilly, Paul Robinson

Published in: BMC Nephrology | Issue 1/2015

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Abstract

Background

Autosomal dominant polycystic kidney disease (ADPKD) is a genetic disorder characterised by progressive renal cyst formation leading to renal failure in the majority of patients. The likelihood and rate of ADPKD progression is difficult to predict and there is a clear need to identify prognostic indicators that could be used to anticipate ADPKD progression, to aid the management of patients in clinical practice.

Methods

A systematic literature review was conducted to identify publications detailing the natural history of ADPKD, including diagnosis, prognosis and progression. Publications were identified and filtered, and data were extracted, based on a predefined research protocol.

Results

The review identified 2799 journal articles and 444 conference abstracts; 254 articles, including observational studies, clinical trials and reviews, proceeded to data extraction. Disease progression was associated with a variety of prognostic indicators, most commonly age and total kidney volume (TKV). In the identified clinical trials, the absence of a consistent measure of disease progression led to variation in the primary endpoints used. Consequently, there was difficulty in consistently and effectively demonstrating and comparing the efficacy of investigational treatments across studies. More consistency was found in the observational studies, where disease progression was most frequently measured by TKV and glomerular filtration rate.

Conclusions

This systematic review identified age and TKV as the most commonly cited prognostic indicators in the published ADPKD literature. It is envisaged that this review may inform future research, trial design and predictive models of ADPKD natural history, helping to optimise patient care.
Appendix
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Metadata
Title
A systematic review of the predictors of disease progression in patients with autosomal dominant polycystic kidney disease
Authors
Claire Woon
Ashleigh Bielinski-Bradbury
Karl O’Reilly
Paul Robinson
Publication date
01-12-2015
Publisher
BioMed Central
Published in
BMC Nephrology / Issue 1/2015
Electronic ISSN: 1471-2369
DOI
https://doi.org/10.1186/s12882-015-0114-5

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