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      Sickle Cell Nephropathy in the Pediatric Population

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          Abstract

          Background: Compared to the past, patients with sickle cell disease (SCD) currently live longer due to improvements in diagnosis and comprehensive care. Due to these advances, long-term chronic complications pose a greater challenge in the management of patients with SCD. In particular, sickle cell nephropathy (SCN) is associated with significant morbidity and mortality across all age groups. Furthermore, SCN is an understudied condition with relatively few symptoms and therefore requires close surveillance. In this review, we sought to explore the epidemiology, natural history, and treatment options for SCN with an emphasis on the pediatric population. Summary: SCN invariably begins in childhood with evidence of structural changes detected as early as infancy. These indolent changes can progress undetected to advanced chronic kidney disease by late adolescence or early adulthood. The risk factors for progression are not well defined, but significant albuminuria (which is also the most common presentation in childhood) is a key factor in progression. One of the main challenges in understanding SCN in children is the poor correlation between estimated and measured glomerular filtration rates. Another challenge is the lack of large-scale longitudinal studies that track the clinical outcomes of pediatric patients over time. Several studies aim to identify early biomarkers of SCN in children, as albuminuria presents only following significant chronic damage. The utility of angiotensin converting enzyme inhibitors and hydroxyurea in treating albuminuria is addressed here as well as novel treatments that may be of benefit.

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          Author and article information

          Journal
          BPU
          Blood Purif
          10.1159/issn.0253-5068
          Blood Purification
          S. Karger AG
          0253-5068
          1421-9735
          2019
          March 2019
          05 December 2018
          : 47
          : 1-3
          : 205-213
          Affiliations
          [_a] aDivision of Nephrology, Massachusetts General Hospital, Harvard Medical School, Boston, Massachusetts, USA
          [_b] bRenal-Electrolyte and Hypertension Division, Perelman School of Medicine, University of Pennsylvania, Philadelphia, Pennsylvania, USA
          [_c] cDepartment of Medicine, Mt. Sinai Hospital, Icahn School of Medicine, New York, New York, USA
          [_d] dDivision of Hematology, Brigham and Women’s Hospital, Harvard Medical School, Boston, Massachusetts, USA
          [_e] eDivision of Pediatric Nephrology, Massachusetts General Hospital for Children, Harvard Medical School, Boston, Massachusetts, USA
          [_f] fDepartment of Biomedical Sciences, Cedars-Sinai Medical Center, Los Angeles, California, USA
          Author notes
          *Kabir Olaniran, MD and Ravi Thadhani, MD, MPH, Massachusetts General Hospital, Renal Associates, 165 Cambridge Street Ste 302, Boston, MA 02114 (USA), E-Mail kolaniran@mgh.harvard.edu and rthadhani@mgh.harvard.edu
          Article
          494581 Blood Purif 2019;47:205–213
          10.1159/000494581
          30517931
          16067566-ceac-44ca-9c60-a6b6ae0729d7
          © 2018 S. Karger AG, Basel

          Copyright: All rights reserved. No part of this publication may be translated into other languages, reproduced or utilized in any form or by any means, electronic or mechanical, including photocopying, recording, microcopying, or by any information storage and retrieval system, without permission in writing from the publisher. 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
          Page count
          Figures: 1, Tables: 4, Pages: 9
          Categories
          Review – Advances in CKD 2019

          Cardiovascular Medicine,Nephrology
          Sickle cell anemia,Sickle cell nephropathy,Chronic kidney disease,Sickle cell disease,Pediatrics

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