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      Mayo imaging classification is a good predictor of rapid progress among Korean patients with autosomal dominant polycystic kidney disease: results from the KNOW-CKD study

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          Abstract

          Background

          Mayo imaging classification (MIC) is a useful biomarker to predict disease progression in autosomal dominant polycystic kidney disease (ADPKD). This study was performed to validate MIC in the prediction of renal outcome in a prospective Korean ADPKD cohort and evaluate clinical parameters associated with rapid disease progression.

          Methods

          A total of 178 ADPKD patients were enrolled and prospectively observed for an average duration of 6.2 ± 1.9 years. Rapid progressor was defined as MIC 1C through 1E while slow progressor was defined as 1A through 1B. Renal composite outcome (doubling of serum creatinine, 50% decline of estimated glomerular filtration rate [eGFR], or initiation of renal replacement therapy) as well as the annual percent change of height-adjusted total kidney volume (mHTKV-α) and eGFR decline (mGFR-α) were compared between groups.

          Results

          A total of 110 patients (61.8%) were classified as rapid progressors. These patients were younger and showed a higher proportion of male patients. Rapid progressor was an independent predictor for renal outcome (hazard ratio, 4.09; 95% confidence interval, 1.23–13.54; p = 0.02). The mGFR-α was greater in rapid progressors (–3.58 mL/min per year in 1C, –3.7 in 1D, and –4.52 in 1E) compared with that in slow progressors (–1.54 in 1A and –2.06 in 1B). The mHTKV-α was faster in rapid progressors (5.3% per year in 1C, 9.4% in 1D, and 11.7% in 1E) compared with that in slow progressors (1.2% in 1A and 3.8% in 1B).

          Conclusion

          MIC is a good predictive tool to define rapid progressors in Korean ADPKD patients.

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          Most cited references27

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          Imaging classification of autosomal dominant polycystic kidney disease: a simple model for selecting patients for clinical trials.

          The rate of renal disease progression varies widely among patients with autosomal dominant polycystic kidney disease (ADPKD), necessitating optimal patient selection for enrollment into clinical trials. Patients from the Mayo Clinic Translational PKD Center with ADPKD (n=590) with computed tomography/magnetic resonance images and three or more eGFR measurements over ≥6 months were classified radiologically as typical (n=538) or atypical (n=52). Total kidney volume (TKV) was measured using stereology (TKVs) and ellipsoid equation (TKVe). Typical patients were randomly partitioned into development and internal validation sets and subclassified according to height-adjusted TKV (HtTKV) ranges for age (1A-1E, in increasing order). Consortium for Radiologic Imaging Study of PKD (CRISP) participants (n=173) were used for external validation. TKVe correlated strongly with TKVs, without systematic underestimation or overestimation. A longitudinal mixed regression model to predict eGFR decline showed that log2HtTKV and age significantly interacted with time in typical patients, but not in atypical patients. When 1A-1E classifications were used instead of log2HtTKV, eGFR slopes were significantly different among subclasses and, except for 1A, different from those in healthy kidney donors. The equation derived from the development set predicted eGFR in both validation sets. The frequency of ESRD at 10 years increased from subclass 1A (2.4%) to 1E (66.9%) in the Mayo cohort and from 1C (2.2%) to 1E (22.3%) in the younger CRISP cohort. Class and subclass designations were stable. An easily applied classification of ADPKD based on HtTKV and age should optimize patient selection for enrollment into clinical trials and for treatment when one becomes available.
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            Autosomal-dominant polycystic kidney disease (ADPKD): executive summary from a Kidney Disease: Improving Global Outcomes (KDIGO) Controversies Conference

            Autosomal-dominant polycystic kidney disease (ADPKD) affects up to 12 million individuals and is the fourth most common cause for renal replacement therapy worldwide. There have been many recent advances in the understanding of its molecular genetics and biology, and in the diagnosis and management of its manifestations. Yet, diagnosis, evaluation, prevention, and treatment vary widely and there are no broadly accepted practice guidelines. Barriers to translation of basic science breakthroughs to clinical care exist, with considerable heterogeneity across countries. The Kidney Disease: Improving Global Outcomes Controversies Conference on ADPKD brought together a panel of multidisciplinary clinical expertise and engaged patients to identify areas of consensus, gaps in knowledge, and research and health-care priorities related to diagnosis; monitoring of kidney disease progression; management of hypertension, renal function decline and complications; end-stage renal disease; extrarenal complications; and practical integrated patient support. These are summarized in this review.
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              Blood Pressure in Early Autosomal Dominant Polycystic Kidney Disease

              Hypertension is common in autosomal dominant polycystic kidney disease (ADPKD) and is associated with increased total kidney volume, activation of the renin-angiotensin-aldosterone system, and progression of kidney disease.
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                Author and article information

                Journal
                Kidney Res Clin Pract
                Kidney Res Clin Pract
                KRCP
                Kidney Research and Clinical Practice
                The Korean Society of Nephrology
                2211-9132
                2211-9140
                July 2022
                3 March 2022
                : 41
                : 4
                : 432-441
                Affiliations
                [1 ]Department of Internal Medicine, Hallym University Kangnam Sacred Heart Hospital, Seoul, Republic of Korea
                [2 ]Kidney Research Institute, Hallym University College of Medicine, Seoul, Republic of Korea
                [3 ]Rehabilitation Medical Research Center, Korea Workers’ Compensation and Welfare Service Incheon Hospital, Incheon, Republic of Korea
                [4 ]Department of Radiology, Seoul National University Hospital, Seoul, Republic of Korea
                [5 ]Department of Internal Medicine, Seoul National University Hospital, Seoul, Republic of Korea
                [6 ]Department of Prevention and Management, Inha University School of Medicine, Incheon, Republic of Korea
                [7 ]Department of Internal Medicine, Inje University Busan Paik Hospital, Busan, Republic of Korea
                [8 ]Department of Internal Medicine, Seoul National University Bundang Hospital, Seongnam, Republic of Korea
                [9 ]Department of Internal Medicine, Gachon University Gil Medical Center, Incheon, Republic of Korea
                [10 ]Department of Internal Medicine, National Medical Center, Seoul, Republic of Korea
                [11 ]Department of Internal Medicine, SMG-SNU Boramae Medical Center, Seoul, Republic of Korea
                Author notes
                Correspondence: Yun Kyu Oh Department of Internal Medicine, SMG-SNU Boramae Medical Center, 20 Boramae-ro 5-gil, Dongjak-gu, Seoul 07061, Republic of Korea. E-mail: yoonkyu@ 123456snu.ac.kr

                The abstract of this article was presented at the 2021 Fall Conference of the Korean Society of Nephrology.

                Author information
                http://orcid.org/0000-0002-1128-3750
                http://orcid.org/0000-0001-7182-9789
                http://orcid.org/0000-0002-8486-9832
                http://orcid.org/0000-0003-2148-4465
                http://orcid.org/0000-0003-3215-8681
                http://orcid.org/0000-0003-2784-3772
                http://orcid.org/0000-0002-4101-9993
                http://orcid.org/0000-0001-9401-892X
                http://orcid.org/0000-0001-7657-130X
                http://orcid.org/0000-0001-7033-1102
                http://orcid.org/0000-0001-9525-2179
                http://orcid.org/0000-0001-8632-5743
                Article
                j-krcp-21-261
                10.23876/j.krcp.21.261
                9346394
                35286789
                9647a22f-78ec-4e46-b06c-071041c49347
                Copyright © 2022 The Korean Society of Nephrology

                This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial and No Derivatives License ( http://creativecommons.org/licenses/by-nc-nd/4.0/) which permits unrestricted non-commercial use, distribution of the material without any modifications, and reproduction in any medium, provided the original works properly cited.

                History
                : 3 November 2021
                : 21 December 2021
                : 29 December 2021
                Categories
                Original Article

                autosomal dominant polycystic kidney,computer-assisted image interpretation,glomerular filtration rate,prognosis,renal insufficiency

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