Fung, W.W.-S. orcid.org/0000-0001-7676-7628, Szeto, C.-C. orcid.org/0000-0002-8898-8505, Chow, K.-M. orcid.org/0000-0001-5310-5197 et al. (8 more authors) (2024) Clinical characteristics and kidney outcomes in Chinese patients with autosomal dominant polycystic kidney disease. Kidney360, 5 (5). pp. 715-723. ISSN 2641-7650
Abstract
Background
The management of autosomal dominant polycystic kidney disease (ADPKD) remains challenging with variable and uncertain genotype-phenotype correlations. The Mayo clinic imaging classification allows a more accurate risk stratification but is limited by the atypical imaging patterns. We aim to assess the clinical characteristics and the morphology of the cystic kidneys in a cohort of Chinese patients with ADPKD.
Method
Ninety-eight patients with ADPKD were recruited prospectively from August 2019 to December 2020 in Prince of Wales Hospital, Hong Kong. They were subsequently followed up every 6 months for a minimum of2 years. We reviewed the clinical characteristics and MRI imaging patterns at baseline and the kidney outcome at the end of the follow-up. Atypical imaging patterns included unilateral; segmental; asymmetric; lopsided and bilateral atrophy as defined by the Mayo Imaging Classification.
Result
Mean age was 51.5 ± 14.3 years old and the mean eGFR 68.7 ± 27.5 ml/min per 1.73 m2. The ninety-eight patients included 36 males:62 females. Seventy-six patients (77.6%) had a family history. Seventeen of the 98 (17.3%) patients had atypical imaging patterns. Compared to typical cases, atypical cases were older at the time of diagnosis (49.5 ± 16.0 vs 33.0 ± 13.0 years, p<0.001), at the time of starting antihypertensive medications (52.4 ± 14.8 vs 39.7 ± 11.0 years, p=0.001) and less likely to have a positive family history (58.8% vs 81.5%, p=0.042). Patients with atypical patterns showed a lower eGFR decline as compared to those with the typical pattern (-0.86 ± 4.34 vs -3.44 ± 4.07 ml/min per 1.73m2/year, p=0.022).
Conclusion
In this cohort of Chinese patients with ADPKD, an atypical imaging pattern was observed in 17% of the cases, associated with later presentation and a milder disease course. Future genotyping studies will help to define the genetic architecture and the basis for the phenotypic spectrum in Chinese ADPKD patients.
Metadata
Item Type: | Article |
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Authors/Creators: |
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Copyright, Publisher and Additional Information: | © 2024 The Author(s). Published by Wolters Kluwer Health, Inc. on behalf of the American Society of Nephrology. This is an open access article distributed under the terms of the Creative Commons Attribution-Non Commercial-No Derivatives License 4.0 (CCBY-NC-ND - https://creativecommons.org/licenses/by-nc-nd/4.0/), where it is permissible to download and share the work provided it is properly cited. The work cannot be changed in any way or used commercially without permission from the journal. |
Keywords: | Biomedical and Clinical Sciences; Clinical Sciences; Genetics; Kidney Disease; Polycystic Kidney Disease; Pediatric; Clinical Research; Renal and urogenital |
Dates: |
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Institution: | The University of Sheffield |
Academic Units: | The University of Sheffield > Faculty of Medicine, Dentistry and Health (Sheffield) > School of Medicine and Population Health |
Depositing User: | Symplectic Sheffield |
Date Deposited: | 01 May 2024 09:38 |
Last Modified: | 15 Nov 2024 15:14 |
Status: | Published |
Publisher: | Ovid Technologies (Wolters Kluwer Health) |
Refereed: | Yes |
Identification Number: | 10.34067/kid.0000000000000433 |
Related URLs: | |
Open Archives Initiative ID (OAI ID): | oai:eprints.whiterose.ac.uk:212019 |