Atypical Polycystic Kidney Disease as defined by Imaging.
Journal
Scientific reports
ISSN: 2045-2322
Titre abrégé: Sci Rep
Pays: England
ID NLM: 101563288
Informations de publication
Date de publication:
20 02 2023
20 02 2023
Historique:
received:
08
04
2022
accepted:
10
11
2022
entrez:
22
2
2023
pubmed:
23
2
2023
medline:
25
2
2023
Statut:
epublish
Résumé
Using age- and height-adjusted total kidney volume, the Mayo Clinic Imaging Classification provides a validated approach to assess the risk of chronic kidney disease (CKD) progression in autosomal dominant polycystic kidney disease (ADPKD), but requires excluding patients with atypical imaging patterns, whose clinical characteristics have been poorly defined. We report an analysis of the prevalence, clinical and genetic characteristics of patients with atypical polycystic kidney disease by imaging. Patients from the extended Toronto Genetic Epidemiology Study of Polycystic Kidney Disease recruited between 2016 and 2018 completed a standardized clinical questionnaire, kidney function assessment, genetic testing, and kidney imaging by magnetic resonance or computed tomography. We compared the prevalence, clinical features, genetics, and renal prognosis of atypical versus typical polycystic kidney disease by imaging. Forty-six of the 523 (8.8%) patients displayed atypical polycystic kidney disease by imaging; they were older (55 vs. 43 years; P < 0.001), and less likely to have a family history of ADPKD (26.1% vs. 74.6%; P < 0.001), a detectable PKD1 or PKD2 mutation (9.2% vs. 80.4%; P < 0.001), or progression to CKD stage 3 or stage 5 (P < 0.001). Patients with atypical polycystic kidney disease by imaging represent a distinct prognostic group with a low likelihood of progression to CKD.
Identifiants
pubmed: 36807559
doi: 10.1038/s41598-022-24104-w
pii: 10.1038/s41598-022-24104-w
pmc: PMC9941465
doi:
Substances chimiques
TRPP Cation Channels
0
Types de publication
Journal Article
Research Support, Non-U.S. Gov't
Langues
eng
Sous-ensembles de citation
IM
Pagination
2952Subventions
Organisme : CIHR
ID : PJT-376307
Pays : Canada
Informations de copyright
© 2022. The Author(s).
Références
J Am Soc Nephrol. 2009 Jan;20(1):205-12
pubmed: 18945943
Kidney Int. 2018 Mar;93(3):691-699
pubmed: 29290310
Kidney Int. 2020 Feb;97(2):261-263
pubmed: 31980075
Nephrol Dial Transplant. 2017 Nov 01;32(11):1857-1865
pubmed: 27484667
J Am Soc Nephrol. 2017 Sep;28(9):2768-2776
pubmed: 28522688
Kidney Int. 2008 Dec;74(11):1468-79
pubmed: 18818683
J Am Soc Nephrol. 2018 Oct;29(10):2593-2600
pubmed: 30135240
Kidney Int. 2020 Feb;97(2):370-382
pubmed: 31874800
Clin J Am Soc Nephrol. 2020 Jan 7;15(1):69-79
pubmed: 31791998
N Engl J Med. 2017 Nov 16;377(20):1930-1942
pubmed: 29105594
J Am Soc Nephrol. 2018 Oct;29(10):2458-2470
pubmed: 30228150
Nephrol Dial Transplant. 2017 Aug 01;32(8):1356-1363
pubmed: 27325254
Nephrol Dial Transplant. 2016 Mar;31(3):337-48
pubmed: 26908832
Kidney Int Rep. 2017 Feb 21;2(3):451-460
pubmed: 29142972
Genes (Basel). 2014 Dec 11;5(4):1064-94
pubmed: 25513881
J Am Soc Nephrol. 2015 Mar;26(3):746-53
pubmed: 25074509
Lancet. 2019 Mar 2;393(10174):919-935
pubmed: 30819518
Clin J Am Soc Nephrol. 2021 May 8;16(5):790-799
pubmed: 32690722
J Am Soc Nephrol. 2015 Jan;26(1):160-72
pubmed: 24904092
Ann Intern Med. 2009 May 5;150(9):604-12
pubmed: 19414839
J Am Soc Nephrol. 2012 May;23(5):915-33
pubmed: 22383692
Can J Kidney Health Dis. 2018 Oct 12;5:2054358118801589
pubmed: 30345064
N Engl J Med. 2012 Dec 20;367(25):2407-18
pubmed: 23121377
Trends Genet. 2001 Feb;17(2):79-82
pubmed: 11173116