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A systematic review of the predictors of disease progression in patients with autosomal dominant polycystic kidney disease
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 pr...
Autores principales: | , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
BioMed Central
2015
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4536696/ https://www.ncbi.nlm.nih.gov/pubmed/26275819 http://dx.doi.org/10.1186/s12882-015-0114-5 |
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author | Woon, Claire Bielinski-Bradbury, Ashleigh O’Reilly, Karl Robinson, Paul |
author_facet | Woon, Claire Bielinski-Bradbury, Ashleigh O’Reilly, Karl Robinson, Paul |
author_sort | Woon, Claire |
collection | PubMed |
description | 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. ELECTRONIC SUPPLEMENTARY MATERIAL: The online version of this article (doi:10.1186/s12882-015-0114-5) contains supplementary material, which is available to authorized users. |
format | Online Article Text |
id | pubmed-4536696 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2015 |
publisher | BioMed Central |
record_format | MEDLINE/PubMed |
spelling | pubmed-45366962015-08-15 A systematic review of the predictors of disease progression in patients with autosomal dominant polycystic kidney disease Woon, Claire Bielinski-Bradbury, Ashleigh O’Reilly, Karl Robinson, Paul BMC Nephrol Research Article 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. ELECTRONIC SUPPLEMENTARY MATERIAL: The online version of this article (doi:10.1186/s12882-015-0114-5) contains supplementary material, which is available to authorized users. BioMed Central 2015-08-15 /pmc/articles/PMC4536696/ /pubmed/26275819 http://dx.doi.org/10.1186/s12882-015-0114-5 Text en © Woon et al. 2015 Open Access This article is distributed under the terms of the Creative Commons Attribution 4.0 International License (http://creativecommons.org/licenses/by/4.0), which permits unrestricted use, distribution, and reproduction in any medium, provided you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons license, and indicate if changes were made. The Creative Commons Public Domain Dedication waiver (http://creativecommons.org/publicdomain/zero/1.0/) applies to the data made available in this article, unless otherwise stated. |
spellingShingle | Research Article Woon, Claire Bielinski-Bradbury, Ashleigh O’Reilly, Karl Robinson, Paul A systematic review of the predictors of disease progression in patients with autosomal dominant polycystic kidney disease |
title | A systematic review of the predictors of disease progression in patients with autosomal dominant polycystic kidney disease |
title_full | A systematic review of the predictors of disease progression in patients with autosomal dominant polycystic kidney disease |
title_fullStr | A systematic review of the predictors of disease progression in patients with autosomal dominant polycystic kidney disease |
title_full_unstemmed | A systematic review of the predictors of disease progression in patients with autosomal dominant polycystic kidney disease |
title_short | A systematic review of the predictors of disease progression in patients with autosomal dominant polycystic kidney disease |
title_sort | systematic review of the predictors of disease progression in patients with autosomal dominant polycystic kidney disease |
topic | Research Article |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4536696/ https://www.ncbi.nlm.nih.gov/pubmed/26275819 http://dx.doi.org/10.1186/s12882-015-0114-5 |
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