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      Co-existing autosomal dominant polycystic kidney disease and nephrotic syndrome in a Nigerian patient with lupus nephritis

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          Abstract

          A little over 30 cases on co-existing nephrotic syndrome and autosomal dominant polycystic kidney disease (ADPKD) have been reported from different regions of the world since 1957. We present a case report on co-existence of nephrotic syndrome (secondary to lupus nephritis) with ADPKD in a 24-year-old woman from Nigeria. She was positive for anti-double stranded DNA. Renal histology showed International Society of Nephrology/Renal Pathology Society Class II lupus nephritis. The co-existence of nephrotic syndrome and ADPKD may have been overlooked in Africa in the past. There is a need to screen for nephrotic syndrome in patients with ADPKD among clinicians in the African setting.

          Résumé

          Une peu plus de trente cas de la coexistence du le syndrome néphrotique et maladie polyktose rénale autosomique dominante (ADPKD) a été reporté dans divers regions du monde depuis 1957. Nous presentons un cas de la coexistence du syndrome néphrotique (secondaire à néphrite lupique) avec l’ADPKD sur une femme de 24 ans au Nigeria. Elle était positif pour ADN double brin anti. L’histologie rénale a presenté ce cas a la Société Internationale de Néphrologie/Société Rénale de Pathologie Classe II néphrite lupique. La coexistence du syndrome néphrotique et l’ADPKD peut avoir été négligé en Afrique dans le passé. Il existe un besoin pour cribler le syndrome néphrotique chez les patients du ADPKD parmi le Cliniciens dans le contexte Africain.

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          Overt proteinuria and microalbuminuria in autosomal dominant polycystic kidney disease.

          The amount of proteinuria is a prognostic indicator in a variety of glomerular disorders. To examine the importance of urinary protein excretion in autosomal dominant polycystic kidney disease, this study determined the clinical characteristics of autosomal dominant polycystic kidney disease patients with established proteinuria and the frequency of microalbuminuria in hypertensive autosomal dominant polycystic kidney disease patients without proteinuria. In 270 autosomal dominant polycystic kidney disease patients, mean 24-h urinary protein excretion was 259 +/- 22 mg/day. Forty-eight of 270 autosomal dominant poly-cystic kidney disease patients had over proteinuria (> 300 mg/day). The patients with established proteinuria had higher mean arterial pressures, larger renal volumes, and lower creatinine clearances than did their nonproteinuric counterparts (all P < 0.0001), a greater pack year smoking history (P < 0.05), and the projection of a more aggressive course of renal disease (P < 0.05). All autosomal dominant polycystic kidney disease patients with established proteinuria were hypertensive, as compared with 67% without established proteinuria (P < 0.001). Forty-nine patients with hypertension and left ventricular hypertrophy without established proteinuria were examined for microalbuminuria; 41% demonstrated microalbuminuria. Those with microalbuminuria had higher mean arterial pressure, larger renal volumes and increased filtration fraction. Therefore, established proteinuria and microalbuminuria in autosomal dominant polycystic kidney disease patients are associated with increased mean arterial pressure and more severe renal cystic involvement.
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            Bilateral polycystic disease of the kidneys; a follow-up of two hundred and eighty-four patients and their families.

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              Autosomal dominant polycystic kidney disease with diffuse proliferative glomerulonephritis - an unusual association: a case report and review of the literature

              Introduction Autosomal dominant polycystic kidney disease is an inherited disorder that is characterized by the development and growth of cysts in the kidneys and other organs. Urinary protein excretion is usually less than 1 g/24 hours in autosomal dominant polycystic kidney disease, and an association of nephrotic syndrome with this condition is considered rare. There are only anecdotal case reports of autosomal dominant polycystic kidney disease associated with nephrotic syndrome, with focal segmental glomerulosclerosis being the most commonly reported histopathological diagnosis. Nephrotic-range proteinuria in the presence of autosomal dominant polycystic kidney disease, with or without an accompanying decline in renal function, should be investigated by open renal biopsy to exclude coexisting glomerular disease. To the best of our knowledge, this is the first case of autosomal dominant polycystic kidney disease with histologically proven diffuse proliferative glomerulonephritis presenting with nephrotic-range proteinuria. No other reports of this could be found in a global electronic search of the literature. Case presentation We report the case of a 35-year-old Indo-Aryan man with autosomal dominant polycystic kidney disease associated with nephrotic syndrome and a concomitant decline in his glomerular filtration rate. Open renal biopsy revealed diffuse proliferative glomerulonephritis. An accurate diagnosis enabled us to manage him conservatively with a successful outcome, without the use of corticosteroid which is the standard treatment and the drug most commonly used to treat nephrotic syndrome empirically. Conclusion Despite the reluctance of physicians to carry out a renal biopsy on patients with autosomal dominant polycystic kidney disease, our case supports the idea that renal biopsy is needed in patients with polycystic kidney disease with nephrotic-range proteinuria to make an accurate diagnosis. It also illustrates the importance of open renal biopsy in planning appropriate treatment for patients with autosomal dominant polycystic kidney disease with nephrotic-range proteinuria. The treatment for various histological subtypes leading to nephrotic syndrome is different, and in this modern era we should practice evidence-based medicine and should avoid empirical therapy with its associated adverse effects.
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                Author and article information

                Journal
                Ann Afr Med
                Ann Afr Med
                AAM
                Annals of African Medicine
                Medknow Publications & Media Pvt Ltd (India )
                1596-3519
                0975-5764
                Apr-Jun 2016
                : 15
                : 2
                : 83-86
                Affiliations
                [1]Kidney Care Centre, University of Medical Sciences, Ondo State, Nigeria
                [1 ]Department of Radiology, Trauma Centre, University of Medical Sciences, Ondo State, Nigeria
                [2 ]Department of Morbid Anatomy, Centre University of Medical Sciences, Ondo State, Nigeria
                [3 ]Department of Morbid Anatomy, University of Medical Science, Ondo State, Nigeria
                Author notes
                Correspondence to: Dr. A. A. Akinbodewa, PMB 542, Medical Village, 23434 Ondo Township, Ondo State, Nigeria. E-mail: ayoakinbodewa@ 123456yahoo.com
                Article
                AAM-15-83
                10.4103/1596-3519.179735
                5402818
                27044732
                aee810ac-70e1-4486-a82f-a5e6aa1aa031
                Copyright: © 2016 Annals of African Medicine

                This is an open access article distributed under the terms of the Creative Commons Attribution-NonCommercial-ShareAlike 3.0 License, which allows others to remix, tweak, and build upon the work non-commercially, as long as the author is credited and the new creations are licensed under the identical terms.

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                Case Report

                africa,autosomal dominant polycystic kidney disease,lupus nephritis,nephrotic syndrome,renal biopsy,afrique,maladie polyktose rénale autosomique dominante,néphrite lupique,le syndrome néphrotique,biopsie rénale

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