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      Call for Papers: Green Renal Replacement Therapy: Caring for the Environment

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      Nephrectomy in Autosomal Dominant Polycystic Kidney Disease: A Patient with Exceptionally Large, Still Functioning Kidneys

      case-report

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          Abstract

          Autosomal dominant polycystic kidney disease (ADPKD) is the most common hereditary kidney disease. It is characterized by progressive cyst formation in both kidneys, often leading to end-stage kidney disease. Indications for surgical removal of an ADPKD kidney include intractable pain, hematuria, infection, or exceptional enlargement and small abdominal cavity hampering implantation of a donor kidney. We report the case of an extraordinarily large ADPKD kidney weighing 8.7 kg (19.3 lb) with a maximal length of 48 cm (19 inch), and with cysts filled with both clear and bloody fluid.

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          Clinical practice. Autosomal dominant polycystic kidney disease.

          Alaina J. Grantham (2008)
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            Early renal abnormalities in autosomal dominant polycystic kidney disease.

            M B Rook, Evert de Jonge, Esther Meijer … (2010)
            Potential therapeutic interventions are being developed for autosomal dominant polycystic kidney disease (ADPKD). A pivotal question will be when to initiate such treatment, and monitoring disease progression will thus become more important. Therefore, the prevalence of renal abnormalities in ADPKD at different ages was evaluated. Included were 103 prevalent ADPKD patients (Ravine criteria). Measured were mean arterial pressure (MAP), total renal volume (TRV), GFR, effective renal plasma flow (ERPF), renal vascular resistance (RVR), and filtration fraction (FF). Twenty-four-hour urine was collected. ADPKD patients were compared with age- and gender-matched healthy controls. Patients and controls were subdivided into quartiles of age (median ages 28, 37, 42, and 52 years). Patients in the first quartile of age had almost the same GFR when compared with controls, but already a markedly decreased ERPF and an increased FF (GFR 117 +/- 32 versus 129 +/- 17 ml/min, ERPF 374 +/- 119 versus 527 +/- 83 ml/min, FF 32% +/- 4% versus 25% +/- 2%, and RVR 12 (10 to 16) versus 8 (7 to 8) dynes/cm(2), respectively). Young adult ADPKD patients also had higher 24-hour urinary volumes, lower 24-hour urinary osmolarity, and higher urinary albumin excretion (UAE) than healthy controls, although TRV in these young adult patients was modestly enlarged (median 1.0 L). Already at young adult age, ADPKD patients have marked renal abnormalities, including a decreased ERPF and increased FF and UAE, despite modestly enlarged TRV and near-normal GFR. ERPF, FF, and UAE may thus be better markers for disease severity than GFR.
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              Images in clinical medicine. Autosomal dominant polycystic kidney disease.

              Burcin Ekser, Paolo Rigotti (2010)
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                Author and article information

                Journal
                Journal ID (nlm-ta): Case Rep Nephrol Urol
                Journal ID (iso-abbrev): Case Rep Nephrol Urol
                Journal ID (publisher-id): CRU
                Title: Case Reports in Nephrology and Urology
                Publisher: S. Karger AG (Allschwilerstrasse 10, P.O. Box · Postfach · Case postale, CH–4009, Basel, Switzerland · Schweiz · Suisse, Phone: +41 61 306 11 11, Fax: +41 61 306 12 34, karger@karger.ch )
                ISSN (Electronic): 1664-5510
                Publication date Collection: May-Aug 2014
                Publication date (Electronic): 4 June 2014
                Publication date PMC-release: 4 June 2014
                Volume: 4
                Issue: 2
                Pages: 109-112
                Affiliations
                [1] aDepartment of Nephrology, University Medical Center Groningen, University of Groningen, Groningen, The Netherlands
                [2] bDepartment of Surgery, University Medical Center Utrecht, Utrecht, The Netherlands
                [3] cDepartment of Pathology, University Medical Center Utrecht, Utrecht, The Netherlands
                Author notes
                *Edwin M. Spithoven, Department of Nephrology, University Medical Center Groningen, University of Groningen, PO Box 30.001, NL-9700 RB Groningen (The Netherlands), E-Mail e.m.spithoven@ 123456umcg.nl
                Article
                Publisher ID: cru-0004-0109
                DOI: 10.1159/000363378
                PMC ID: 4086038
                SO-VID: 45768822-75c8-4ed7-8e57-b25bb0d3544a
                Copyright © Copyright © 2014 by S. Karger AG, Basel
                License:

                This is an Open Access article licensed under the terms of the Creative Commons Attribution-NonCommercial 3.0 Unported license (CC BY-NC) (www.karger.com/OA-license), applicable to the online version of the article only. Users may download, print and share this work on the Internet for noncommercial purposes only, provided the original work is properly cited, and a link to the original work on http://www.karger.com and the terms of this license are included in any shared versions.

                History
                Page count
                Figures: 2, References: 3, Pages: 4
                Categories
                Subject: Published online: June, 2014

                ScienceOpen disciplines: Nephrology
                Keywords: autosomal dominant polycystic kidney disease,nephrectomy,transplantation
                Data availability:
                ScienceOpen disciplines: Nephrology
                Keywords: autosomal dominant polycystic kidney disease, nephrectomy, transplantation

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