Journal Article

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

Jan T. Kielstein, 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 and Reinhard Brunkhorst

in Nephrology Dialysis Transplantation

Published on behalf of European Renal Association - European Dialysis and Transplant Assoc

Volume 27, issue 10, pages 3807-3815
Published in print October 2012 | ISSN: 0931-0509
Published online October 2012 | e-ISSN: 1460-2385 | DOI: http://dx.doi.org/10.1093/ndt/gfs394
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

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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.

Keywords: AKI; complement; mechanical ventilation; seizures; survival

Journal Article.  4668 words.  Illustrated.

Subjects: Nephrology

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