Blog
About

4
views
0
recommends
+1 Recommend
1 collections
    0
    shares
      • Record: found
      • Abstract: found
      • Article: found

      Atypical Hemolytic Uremic Syndrome in Children Aged <2 Years

      Read this article at

      ScienceOpenPublisherPubMed
      Bookmark
          There is no author summary for this article yet. Authors can add summaries to their articles on ScienceOpen to make them more accessible to a non-specialist audience.

          Abstract

          Background: There are limited data on infants with atypical hemolytic uremic syndrome (aHUS). The aim of this study was to determine the clinical and laboratory features, and to evaluate treatment modalities and outcomes in infants with aHUS. Materials and Methods: Relevant data on patients with onset of aHUS at age <2 years were obtained from the Turkish Pediatric aHUS Registry. Results: Among the 146 patients included in the Registry, 53 (36%) (23 male and 30 female) were enrolled for the study. Age at disease onset was ≤1 year in 29 of the patients. In all, 21 (40%) of the patients developed neurological symptoms. Disease-causing mutations were noted in 14 (36%) of the 39 patients in which genetic analysis was performed. Plasma therapy was performed in 42 (79%) patients; eculizumab therapy was administered to treat the first episode of aHUS in 33 (62%) patients and in 5 patients as the first- line therapy. In total, 38 (72%) patients received renal replacement therapy (RRT), 3 (6%) died due to acute illness, and 4 (8%) were discharged from hospital with RRT. Follow-up visit data were available for 46 patients and the median duration was 23 months (range 3–129 months). End-stage renal disease developed only in 1 patient. Proteinuria and hypertension persisted in 17 (37%) and 20 patients (44%) respectively. Eculizumab treatment was continued in 25 of the 39 patients during the follow-up period. Conclusion: One-third<sup></sup> of the aHUS patients had disease onset during infancy. The prognosis of this life-threatening disease seems to get better with improved treatment modalities.

          Related collections

          Author and article information

          Journal
          NEF
          Nephron
          10.1159/issn.1660-8151
          Nephron
          S. Karger AG
          1660-8151
          2235-3186
          2018
          June 2018
          13 March 2018
          : 139
          : 3
          : 211-218
          Affiliations
          aDepartment of Pediatric Nephrology, Ankara University Faculty of Medicine, Ankara, Turkey
          bDepartment of Pediatric Nephrology, Hacettepe University Faculty of Medicine, Ankara, Turkey
          cDepartment of Pediatric Nephrology, Akdeniz University Faculty of Medicine, Ankara, Turkey
          dDepartment of Pediatric Nephrology, Kirikkale University Faculty of Medicine, Kirikkale, Turkey
          eDepartment of Pediatric Nephrology, Karadeniz Technical University Faculty of Medicine, Trabzon, Turkey
          fDepartment of Pediatric Nephrology, Nephrogenetics Laboratory, Hacettepe University Faculty of Medicine, Ankara, Turkey
          gDepartment of Pediatric Nephrology, Istanbul University, Cerrahpasa Faculty of Medicine, Istanbul, Turkey
          hDepartment of Pediatric Nephrology, Gazi University Faculty of Medicine, Ankara, Turkey
          iHacettepe University Center for Biobanking and Genomics, Ankara, Turkey
          Author notes
          *Dr. Nilgun Çakar, Department of Pediatric Nephrology, Ankara University Faculty of Medicine, TR–06100 Cebeci, Ankara (Turkey), E-Mail nilguncakar@hotmail.com
          Article
          487609 Nephron 2018;139:211–218
          10.1159/000487609
          29533929
          © 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.

          Page count
          Tables: 4, Pages: 8
          Categories
          Clinical Practice: Original Paper

          Comments

          Comment on this article