Literature DB >> 23114903

Best supportive care and therapeutic plasma exchange with or without eculizumab in Shiga-toxin-producing E. coli O104:H4 induced haemolytic-uraemic syndrome: an analysis of the German STEC-HUS registry.

Jan T Kielstein1, Gernot Beutel, Susanne Fleig, Jürgen Steinhoff, Tobias N Meyer, Carsten Hafer, Uwe Kuhlmann, Jörn Bramstedt, Ulf Panzer, Martin Vischedyk, Veit Busch, Wolfgang Ries, Steffen Mitzner, Stefan Mees, Sylvia Stracke, Jens Nürnberger, Peter Gerke, Monika Wiesner, Bernd Sucke, Miriam Abu-Tair, Andreas Kribben, Norbert Klause, Ralf Schindler, Frank Merkel, Sabine Schnatter, Eiske M Dorresteijn, Ola Samuelsson, Reinhard Brunkhorst.   

Abstract

BACKGROUND: May 22nd marks the beginning of a Shiga-toxin-producing Escherichia coli (STEC) O104:H4 outbreak in Northern Germany. By its end on 27 July, it had claimed 53 deaths among 2987 STEC and 855 confirmed haemolytic-uraemic syndrome (HUS) cases.
METHODS: To describe short-term effectiveness of best supportive care (BSC), therapeutic plasma exchange (TPE) and TPE with eculizumab (TPE-Ecu) in 631 patients with suspected HUS treated in 84 hospitals in Germany, Sweden and the Netherlands using the web-based registry of the DGfN (online since 27 May).
RESULTS: Of 631 entries, 491 fulfilled the definition of HUS (median age 46 years; 71% females). The median (inter-quartile range) hospital stay was 22 (14-31) days. Two hundred and eighty-one (57%) patients underwent dialysis and 114 (23%) mechanical ventilation. Fifty-seven patients received BSC, 241 TPE and 193 TPE-Ecu. Treatment strategy was dependent on disease severity (laboratory signs of haemolysis, thrombocytopenia, peak creatinine level, need for dialysis, neurological symptoms, frequency of seizures) which was lower in BSC than in TPE and TPE-Ecu patients. At study endpoint (hospital discharge or death), the median creatinine was lower in BSC [1.1 mg/dL (0.9-1.3)] than in TPE [1.2 mg/dL (1.0-1.5), P < 0.05] and TPE-Ecu [1.4 mg/dL (1.0-2.2), P < 0.001], while need for dialysis was not different between BSC (0.0%, n = 0), TPE (3.7%; n = 9) and TPE-Ecu (4.7%, n = 9). Seizures were absent in BSC and rare in TPE (0.4%; n = 1) and TPE-Ecu (2.6%; n = 5) patients. Total hospital mortality in HUS patients was 4.1% (n = 20) and did not differ significantly between the TPE and TPE-Ecu groups.
CONCLUSIONS: Despite frequent renal impairment, advanced neurological disorders and severe respiratory failure, short-term outcome was better than expected when compared with previous reports. Within the limitations of a retrospective registry analysis, our data do not support the notion of a short-term benefit of Ecu in comparison to TPE alone in the treatment of STEC-HUS. A randomized trial comparing BSC, TPE and Ecu seems to be prudent and necessary prior to establishing new treatment guidelines for STEC-HUS.

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Year:  2012        PMID: 23114903     DOI: 10.1093/ndt/gfs394

Source DB:  PubMed          Journal:  Nephrol Dial Transplant        ISSN: 0931-0509            Impact factor:   5.992


  74 in total

1.  Ultrasound findings in EHEC-associated hemolytic-uremic syndrome and their clinical relevance.

Authors:  Ansgar Reising; Carsten Hafer; Marcus Hiss; Jan T Kielstein; Jan Menne; Faikah Gueler; Jan Beneke; Silvia Linnenweber-Held; Hermann Haller; Gunilla Einecke
Journal:  Int Urol Nephrol       Date:  2016-01-12       Impact factor: 2.370

Review 2.  Shiga toxin-producing Escherichia coli O104:H4: an emerging pathogen with enhanced virulence.

Authors:  Dakshina M Jandhyala; Vijay Vanguri; Erik J Boll; Yushuan Lai; Beth A McCormick; John M Leong
Journal:  Infect Dis Clin North Am       Date:  2013-07-24       Impact factor: 5.982

3.  [Globosides as key players in the pathophysiology of Shiga toxin-associated acute kidney failure and Fabry disease].

Authors:  S Porubsky
Journal:  Pathologe       Date:  2014-11       Impact factor: 1.011

4.  Extra-corporeal membrane oxygenation and Eculizumab: Atypical treatments for typical haemolytic uraemic syndrome.

Authors:  Matthew D Kelham; Liam Gleeson; Inma Alcalde; Rosalba Spiritoso; Alastair G Proudfoot; Marie Scully
Journal:  J Intensive Care Soc       Date:  2019-03-07

Review 5.  Plasma exchange in thrombotic microangiopathies (TMAs) other than thrombotic thrombocytopenic purpura (TTP).

Authors:  Jeffrey L Winters
Journal:  Hematology Am Soc Hematol Educ Program       Date:  2017-12-08

Review 6.  Shiga toxin triggers endothelial and podocyte injury: the role of complement activation.

Authors:  Carlamaria Zoja; Simona Buelli; Marina Morigi
Journal:  Pediatr Nephrol       Date:  2017-12-06       Impact factor: 3.714

Review 7.  Hemolytic uremic syndrome.

Authors:  Caterina Mele; Giuseppe Remuzzi; Marina Noris
Journal:  Semin Immunopathol       Date:  2014-02-14       Impact factor: 9.623

Review 8.  A new paradigm: Diagnosis and management of HSCT-associated thrombotic microangiopathy as multi-system endothelial injury.

Authors:  Sonata Jodele; Benjamin L Laskin; Christopher E Dandoy; Kasiani C Myers; Javier El-Bietar; Stella M Davies; Jens Goebel; Bradley P Dixon
Journal:  Blood Rev       Date:  2014-11-28       Impact factor: 8.250

9.  Shiga toxin promotes podocyte injury in experimental hemolytic uremic syndrome via activation of the alternative pathway of complement.

Authors:  Monica Locatelli; Simona Buelli; Anna Pezzotta; Daniela Corna; Luca Perico; Susanna Tomasoni; Daniela Rottoli; Paola Rizzo; Debora Conti; Joshua M Thurman; Giuseppe Remuzzi; Carlamaria Zoja; Marina Morigi
Journal:  J Am Soc Nephrol       Date:  2014-02-27       Impact factor: 10.121

Review 10.  Long-term outcomes of Shiga toxin hemolytic uremic syndrome.

Authors:  Joann M Spinale; Rebecca L Ruebner; Lawrence Copelovitch; Bernard S Kaplan
Journal:  Pediatr Nephrol       Date:  2013-01-04       Impact factor: 3.714

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