Eculizumab in atypical hemolytic uremic syndrome: strategies toward restrictive use.

With the introduction of the complement C5-inhibitor eculizumab, a new era was entered for patients with atypical hemolytic uremic syndrome (aHUS). Eculizumab therapy very effectively reversed thrombotic microangiopathy and reduced mortality and morbidity. Initial guidelines suggested lifelong treat...

Full description

Bibliographic Details
Published in:Pediatric Nephrology Vol. 34; no. 11; pp. 2261 - 2278
Main Authors: Wijnsma, Kioa L., Duineveld, Caroline, Wetzels, Jack F. M., van de Kar, Nicole C. A. J.
Format: protocol questions and answers review tables/charts Journal Article
Published: Springer Nature Nov2019
Online Access:View this record in EBSCOhost
fields @attributes:
  recordID: 1
pdfLink:
plink: https://search.ebscohost.com/login.aspx?direct=true&db=ccm&AN=139138132&site=ehost-live
header:
  @attributes:
    shortDbName: ccm
    uiTerm: 139138132
    longDbName: CINAHL Complete
    uiTag: AN
  controlInfo:
    bkinfo:
    dissinfo:
    jinfo:
      jid:
        0931041X
        EF1
      jtl: Pediatric Nephrology
      issn: 0931041X
      maglogo: N
    pubinfo:
      dt: Nov2019
      vid: 34
      iid: 11
      pid: 237
      pub: Springer Nature
      place: New York, New York
    artinfo:
      ui:
        139138132
        139138132
        144052046
        139138132
        10.1007/s00467-018-4091-3
        139138132
      ppf: 2261
      ppct: 17
      formats:
        fmt:
          – @attributes:
              type: T
          – @attributes:
              type: P
      tig:
        atl: Eculizumab in atypical hemolytic uremic syndrome: strategies toward restrictive use.
      aug:
        au:
          Wijnsma, Kioa L.
          Duineveld, Caroline
          Wetzels, Jack F. M.
          van de Kar, Nicole C. A. J.
        affil: Radboud Institute for Molecular Life Sciences, Amalia Children's Hospital, Department of Pediatric Nephrology, Radboud University Medical Center, P.O. Box 9101, 6500 HB, Nijmegen, The Netherlands
      sug:
        subj:
          Atypical Hemolytic Uremic Syndrome Drug Therapy
          Antibodies, Monoclonal Therapeutic Use
          Treatment Outcomes
          Thrombocytopenia Drug Therapy
          Thrombocytopenia Mortality
          Mortality Prevention and Control
          Kidney Transplantation
          Transplant Recipients
          Treatment Duration
          Individualized Medicine
          Protocols
          Economics, Pharmaceutical
          Antibodies, Monoclonal Adverse Effects
      ab: With the introduction of the complement C5-inhibitor eculizumab, a new era was entered for patients with atypical hemolytic uremic syndrome (aHUS). Eculizumab therapy very effectively reversed thrombotic microangiopathy and reduced mortality and morbidity. Initial guidelines suggested lifelong treatment and recommended prophylactic use of eculizumab in aHUS patients receiving a kidney transplant. However, there is little evidence to support lifelong therapy or prophylactic treatment in kidney transplant recipients. Worldwide, there is an ongoing debate regarding the optimal dose and duration of treatment, particularly in view of the high costs and potential side effects of eculizumab. An increasing but still limited number of case reports and small cohort studies suggest that a restrictive treatment regimen is feasible. We review the current literature and focus on the safety and efficacy of restrictive use of eculizumab. Our current treatment protocol is based on restrictive use of eculizumab. Prospective monitoring will provide more definite proof of the feasibility of such restrictive treatment.
      pubtype: Academic Journal
      doctype:
        protocol
        questions and answers
        review
        tables/charts
        Journal Article
      ougenre: Article
    language: English
    refInfo:
    holdings:
      @attributes:
        islocal: N