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      Reninoma: An Uncommon Cause of Renin-Mediated Hypertension

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          Abstract

          Reninoma (juxtaglomerular cell tumor) is a rare cause of renin-mediated hypertension. Reninomas are usually diagnosed in adolescents and young adults with occasional reports in younger children. Patients typically present with a long history of headaches leading to a diagnosis of severe hypertension that responds well to antihypertensive treatment targeting the renin-angiotensin-aldosterone system. The clue to clinical diagnosis is the presence of hypokalemia and metabolic alkalosis on the first blood sample drawn before any treatment is instituted. Elevated blood levels of renin and aldosterone confirm the clinical suspicion of renin-mediated hypertension. Diagnostic imaging is employed to identify the source of excessive renin production. While renal ultrasound can miss reninoma, contrast CT or magnetic resonance imaging of the kidneys are diagnostic modalities of choice leading to the correct diagnosis. Renal vein renin sampling with lateralization might help to identify the site of excessive renin production. Nephron-sparing surgery is curative with maintenance of normal blood pressure after discontinuation of antihypertensive medications in the majority of patients. In this paper, we present the case of reninoma in an adolescent girl emphasizing clinical presentation, diagnostic evaluation, and medical and surgical treatment of this rare tumor. We also discuss important points in the management of children presenting with renin-mediated hypertension.

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          Most cited references27

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          Comparison of 1,800 laparoscopic and open partial nephrectomies for single renal tumors.

          Laparoscopic partial nephrectomy is an increasingly performed, minimally invasive alternative to open partial nephrectomy. We compared early postoperative outcomes in 1,800 patients undergoing open partial nephrectomy by experienced surgeons with the initial experience with laparoscopic partial nephrectomy in patients with a single renal tumor 7 cm or less. Data on 1,800 consecutive open or laparoscopic partial nephrectomies were collected prospectively or retrospectively in tumor registries at 3 large referral centers. Demographic, intraoperative, postoperative and followup data were compared between the 2 groups. Compared to the laparoscopic partial nephrectomy group of 771 patients the 1,028 undergoing open partial nephrectomy were a higher risk group with a greater percent presenting symptomatically with decreased performance status, impaired renal function and tumor in a solitary functioning kidney (p<0.0001). More tumors in the open partial nephrectomy group were more than 4 cm and centrally located and more proved to be malignant (p<0.0001 and 0.0003, respectively). Based on multivariate analysis laparoscopic partial nephrectomy was associated with shorter operative time (p<0.0001), decreased operative blood loss (p<0.0001) and shorter hospital stay (p<0.0001). The chance of intraoperative complications was comparable in the 2 groups. However, laparoscopic partial nephrectomy was associated with longer ischemia time (p<0.0001), more postoperative complications, particularly urological (p<0.0001), and an increased number of subsequent procedures (p<0.0001). Renal functional outcomes were similar 3 months after laparoscopic and open partial nephrectomy with 97.9% and 99.6% of renal units retaining function, respectively. Three-year cancer specific survival for patients with a single cT1N0M0 renal cell carcinoma was 99.3% and 99.2% after laparoscopic and open partial nephrectomy, respectively. Early experience with laparoscopic partial nephrectomy is promising. Laparoscopic partial nephrectomy offered the advantages of less operative time, decreased operative blood loss and a shorter hospital stay. When applied to patients with a single renal tumor 7 cm or less, laparoscopic partial nephrectomy was associated with additional postoperative morbidity compared to open partial nephrectomy. However, equivalent functional and early oncological outcomes were achieved.
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            The changing face of pediatric hypertension in the era of the childhood obesity epidemic.

            Historically, hypertension in childhood was thought to be an uncommon diagnosis, usually related to an underlying condition, most often parenchymal renal disease. Primary hypertension in childhood was felt to be quite rare. However, the worldwide childhood obesity epidemic has had a profound impact on the frequency of hypertension and other obesity-related conditions with the result that primary hypertension should now be viewed as one of the most common health conditions in the young. This review will present updated data on the prevalence of hypertension in children and adolescents, the impact of the childhood obesity epidemic on hypertension prevalence and blood pressure levels, shifts in how often primary hypertension is being diagnosed in childhood, and an overview of the pathophysiology of obesity-related hypertension. It is hoped that improved understanding of the significance of these issues will lead to improved recognition and treatment, which will be the key to averting an epidemic of cardiovascular disease in adulthood.
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              Hypertension due to a renin-secreting renal tumour.

                Author and article information

                Contributors
                Journal
                Front Pediatr
                Front Pediatr
                Front. Pediatr.
                Frontiers in Pediatrics
                Frontiers Media S.A.
                2296-2360
                15 August 2014
                2014
                : 2
                : 89
                Affiliations
                [1] 1Department of Paediatrics and Child Health, The University of Queensland , Brisbane, QLD, Australia
                [2] 2Department of Paediatrics, Mater Children’s Hospital , Brisbane, QLD, Australia
                [3] 3Department of Medical Imaging, Mater Children’s Hospital , Brisbane, QLD, Australia
                [4] 4Department of Pathology, Mater Adult Hospital , Brisbane, QLD, Australia
                [5] 5Department of Paediatric Surgery, Royal Children’s Hospital , Brisbane, QLD, Australia
                Author notes

                Edited by: Michael L. Moritz, Children’s Hospital of Pittsburgh of UPMC, USA

                Reviewed by: François Cachat, University Hospital of Lausanne, Switzerland; Donald Lee Batisky, Emory University, USA

                *Correspondence: Peter Trnka, Department of Paediatrics and Child Health, The University of Queensland, Royal Children’s Hospital, Level 5, Woolworths Building, Herston Road, Herston, QLD 4029, Australia e-mail: peter.trnka@ 123456health.qld.gov.au

                This article was submitted to Pediatric Nephrology, a section of the journal Frontiers in Pediatrics.

                Article
                10.3389/fped.2014.00089
                4133731
                25177679
                edc31df4-806e-42c2-8cea-db8b611ec181
                Copyright © 2014 Trnka, Orellana, Walsh, Pool and Borzi.

                This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY). The use, distribution or reproduction in other forums is permitted, provided the original author(s) or licensor are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with these terms.

                History
                : 26 May 2014
                : 04 August 2014
                Page count
                Figures: 4, Tables: 0, Equations: 0, References: 26, Pages: 7, Words: 4822
                Categories
                Pediatrics
                Clinical Case Study

                hypertension,blood pressure,kidney,renin,reninoma,juxtaglomerular cell tumor

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