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      Kidney Volume Estimations with Ellipsoid Equations by Magnetic Resonance Imaging in Autosomal Dominant Polycystic Kidney Disease

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          Abstract

          Background: Kidney volume (KV) becomes clinically relevant in autosomal dominant polycystic kidney disease (ADPKD) management. KV can be conveniently estimated (ceKV) using ellipsoid volume equations with three axes measurements; however, the accuracy and reliability are unknown. Methods: KVs of 347 kidneys in 177 consecutive ADPKD patients were determined with a volumetric method (standard-KV), and ceKV was calculated using six different ellipsoid equations with three axes measurements using magnetic resonance imaging. The inter- and intraobserver reliabilities were analyzed using intraclass correlation coefficients (ICCs). Ellipsoid-KVs were obtained by linear regression analysis between standard-KV and ceKVs, and six ellipsoid-KVs were validated with a bootstrap model. Results: The ICCs of intra- and interobserver reliabilities in standard-KV and axes measurements were highly reliable. All ceKVs underestimated standard-KV and % differences between ceKV and standard-KV were reduced by ellipsoid-KVs. Bootstrap analyses suggested that six ellipsoid-KVs reliably simulated standard-KV. Conclusion: Among six ellipsoid-KVs, ellipsoid-KV<sub>3</sub> = 84 + 1.01 x π/24 × Length × (sum of two width measurements)<sup>2</sup> relatively accurately simulated the standard-KV. Kidney volume estimation using ellipsoid equations is reliably applied to clinical management of ADPKD while recognizing wide scattering in the difference between estimated and volumetrically measured kidney volume.

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          Unified criteria for ultrasonographic diagnosis of ADPKD.

          York Pei, James Obaji, Annie Dupuis … (2009)
          Individuals who are at risk for autosomal dominant polycystic kidney disease are often screened by ultrasound using diagnostic criteria derived from individuals with mutations in PKD1. Families with mutations in PKD2 typically have less severe disease, suggesting a potential need for different diagnostic criteria. In this study, 577 and 371 at-risk individuals from 58 PKD1 and 39 PKD2 families, respectively, were assessed by renal ultrasound and molecular genotyping. Using sensitivity data derived from genetically affected individuals and specificity data derived from genetically unaffected individuals, various diagnostic criteria were compared. In addition, data sets were created to simulate the PKD1 and PKD2 case mix expected in practice to evaluate the performance of diagnostic criteria for families of unknown genotype. The diagnostic criteria currently in use performed suboptimally for individuals with mutations in PKD2 as a result of reduced test sensitivity. In families of unknown genotype, the presence of three or more (unilateral or bilateral) renal cysts is sufficient for establishing the diagnosis in individuals aged 15 to 39 y, two or more cysts in each kidney is sufficient for individuals aged 40 to 59 y, and four or more cysts in each kidney is required for individuals > or = 60 yr. Conversely, fewer than two renal cysts in at-risk individuals aged > or = 40 yr is sufficient to exclude the disease. These unified diagnostic criteria will be useful for testing individuals who are at risk for autosomal dominant polycystic kidney disease in the usual clinical setting in which molecular genotyping is seldom performed.
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            Imaging classification of autosomal dominant polycystic kidney disease: a simple model for selecting patients for clinical trials.

            Maria Irazabal, Laureano J Rangel, Eric J Bergstralh … (2014)
            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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              Volume progression in polycystic kidney disease.

              Jared J Grantham, Vicente Torres, Arlene Chapman … (2006)
              Autosomal dominant polycystic kidney disease (ADPKD) is characterized by progressive enlargement of cyst-filled kidneys. In a three-year study, we measured the rates of change in total kidney volume, total cyst volume, and iothalamate clearance in patients with ADPKD. Of a total of 241 patients, in 232 patients without azotemia who were 15 to 46 years old at baseline we used magnetic-resonance imaging to correlate the total kidney volume and total cyst volume with iothalamate clearance. Statistical methods included analysis of variance, Pearson correlation, and multivariate regression analysis. Total kidney volume and total cyst volume increased exponentially, a result consistent with an expansion process dependent on growth. The mean (+/-SD) total kidney volume was 1060+/-642 ml at baseline and increased by a mean of 204+/-246 ml (5.27+/-3.92 percent per year, P<0.001) over a three-year period among 214 patients. Total cyst volume increased by 218+/-263 ml (P<0.001) during the same period among 210 patients. The baseline total kidney volume predicted the subsequent rate of increase in volume, independently of age. A baseline total kidney volume above 1500 ml in 51 patients was associated with a declining glomerular filtration rate (by 4.33+/-8.07 ml per minute per year, P<0.001). Total kidney volume increased more in 135 patients with PKD1 mutations (by 245+/-268 ml) than in 28 patients with PKD2 mutations (by 136+/-100 ml, P=0.03). Kidney enlargement resulting from the expansion of cysts in patients with ADPKD is continuous and quantifiable and is associated with the decline of renal function. Higher rates of kidney enlargement are associated with a more rapid decrease in renal function. Copyright 2006 Massachusetts Medical Society.
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                Author and article information

                Journal
                Journal ID (publisher-id): NEF
                Journal ID (nlm-ta): Nephron
                Journal ID (doi): 10.1159/issn.1660-8151
                Title: Nephron
                Publisher: S. Karger AG
                ISSN (Print): 1660-8151
                ISSN (Electronic): 2235-3186
                Publication date Subscription-year: 2015
                Publication date (Print): April 2015
                Publication date (Electronic): 16 April 2015
                Volume: 129
                Issue: 4
                Pages: 253-262
                Affiliations
                aDepartment of ADPKD Research, Kyorin University School of Medicine, bDepartment of Urology, Kyorin University School of Medicine, cDepartment of Radiology, Kyorin University School of Medicine, and dDepartment of Medical Radiological Technology, Faculty of Health Sciences, Kyorin University, Tokyo, Japan
                Author notes
                *Prof. Eiji Higashihara, Department of ADPKD Research, Kyorin University School of Medicine, 6-20-2 Shinkawa, Mitaka, Tokyo 181-8611 (Japan), E-Mail ehigashi@ks.kyorin-u.ac.jp
                Article
                Publisher ID: 381476 Other ID: Nephron 2015;129:253-262
                DOI: 10.1159/000381476
                PubMed ID: 25895545
                SO-VID: be298060-72d3-46a2-be3e-7512029355b2
                Copyright © © 2015 S. Karger AG, Basel
                License:

                Open Access License: This is an Open Access article licensed under the terms of the Creative Commons Attribution-NonCommercial 3.0 Unported license (CC BY-NC) ( http://www.karger.com/OA-license), applicable to the online version of the article only. Distribution permitted for non-commercial purposes only. Drug Dosage: The authors and the publisher have exerted every effort to ensure that drug selection and dosage set forth in this text are in accord with current recommendations and practice at the time of publication. However, in view of ongoing research, changes in government regulations, and the constant flow of information relating to drug therapy and drug reactions, the reader is urged to check the package insert for each drug for any changes in indications and dosage and for added warnings and precautions. This is particularly important when the recommended agent is a new and/or infrequently employed drug. Disclaimer: The statements, opinions and data contained in this publication are solely those of the individual authors and contributors and not of the publishers and the editor(s). The appearance of advertisements or/and product references in the publication is not a warranty, endorsement, or approval of the products or services advertised or of their effectiveness, quality or safety. The publisher and the editor(s) disclaim responsibility for any injury to persons or property resulting from any ideas, methods, instructions or products referred to in the content or advertisements.

                History
                Date received : 09 September 2014
                Date accepted : 07 March 2015
                Page count
                Figures: 4, Tables: 5, References: 25, Pages: 10
                Categories
                Subject: Clinical Practice: Original Paper

                ScienceOpen disciplines: Cardiovascular Medicine,Nephrology
                Keywords: Magnetic resonance imaging,Autosomal dominant polycystic kidney disease,Kidney volume,Ellipsoid volume equation
                Data availability:
                ScienceOpen disciplines: Cardiovascular Medicine, Nephrology
                Keywords: Magnetic resonance imaging, Autosomal dominant polycystic kidney disease, Kidney volume, Ellipsoid volume equation

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