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Contemporary Reviews in Critical Care Medicine |

Expert statements on the standard of care in critically ill adult patients with atypical haemolytic uraemic syndrome OPEN ACCESS

Elie Azoulay, MD, PhD; Paul Knoebl, MD; José Garnacho-Montero, MD, PhD; Katerina Rusinova, MD, PhD; Gennadii Galstian, MD, PhD; Philippe Eggimann, MD, PhD; Fekri Abroug, MD, PhD; Dominique Benoit, MD, PhD; Michael von Bergwelt-Baildon, MD, PhD; Julia Wendon, MD; Marie Scully, MD
Author and Funding Information

Competing interests

This review article is an independent piece of work from the signing authors. This group of experts met at an advisory board to discuss thrombotic microangiopathies in critically ill patients. Together, they defined the content of this article, the scope of the systematic review, the elements needed to make this review didactical and thoughtful for the readers, as well as to select the content of each tables and figures. Senior authors have drafted the review and all authors have added their contribution and suggested changes. A medical writer has edited the sentences where needed and improved the language. Before submission, the paper circulated several times within authors and each single suggestion has been taken into account. Alexion has paid the travels and the hotel (for one night) to make the meeting happen. Alexion also paid the medical writer. The generation of this work was an offshoot from the medical attendees and not in any way directed by Alexion. Authors do not believe that industry could possibly benefit from the content of this review.

In addition:

Elie Azoulay is part of the board of Gilead France, and has received fees for lectures from Alexion and Astellas. His institution has received grants from Fisher & Payckle, Pfizer and Cubist.

Gennadii Galstian have received lecture honoraria from Alexion PHARMA INTERNATIONAL Sàrl and Alexion Pharma LLC, Russia

Paul Knoebl has served as an advisory board member, speaker and consultant for Alexion, Ablynx and Baxalta (now Shire).

Dominique Benoit has received speaker’s honoraria from Pfizer and MSD.

Marie Scully has received advisory board and speaker’s honoraria for Alexion, Ablyn, Baxalta and Novartis.

aFekri Abroug, Fattouma Bourguiba Teaching Hospital Monastir, Tunisia

bDominique Benoit, Ghent University Hospital, Ghent, Belgium

cMichael von Bergwelt-Baildon, Uniklinik, Cologne, Germany

dPhilippe Eggimann, University Hospital of Lausanne, Switzerland

eGennadii Galstian, National Research Center for Hematology, Russia

fJosé Garnacho-Montero, Virgen de Macarena University Hospital, Seville, Spain

gPaul Knoebl, Medical University of Vienna, Vienna General Hospital, Austria

hKaterina Rusinova, University Hospital, 1st Faculty of Medicine, Charles University in Prague, Czech Republic

iMarie Scully, University College London, United Kingdom

jJulia Wendon, King’s College Hospital, London, United Kingdom

kMedical Intensive Care Unit, Hôpital Saint-Louis, ECSTRA team, and clinical epidemiology, UMR 1153 (Center of Epidemiology and Biostatistics Sorbonne Paris Cité, CRESS), INSERM, Paris Diderot Sorbonne University

Author for Correspondence: Elie Azoulay, Medical Intensive Care Unit, Hôpital Saint-Louis, ECSTRA team, and clinical epidemiology, UMR 1153 (Center of Epidemiology and Biostatistics Sorbonne Paris Cité, CRESS), INSERM, Paris Diderot Sorbonne University Tel: +33 142 499 421, Fax: +33 142 499 426.


Copyright 2017, . All Rights Reserved.


Chest. 2017. doi:10.1016/j.chest.2017.03.055
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Abstract

Background  Atypical haemolytic uraemic syndrome (aHUS) presents similarly to thrombotic thrombocytopenic purpura (TTP), and other causes or conditions with thrombotic microangiopathy (TMA) such as DIC or sepsis. Similarity in clinical presentation may hinder diagnosis and optimal treatment selection in the urgent setting in the ICU. However, there is currently no consensus on the diagnosis or treatment of aHUS for ICU specialists. This review aims to summarise available data on the diagnosis and treatment strategies of aHUS in the ICU in order to enhance the understanding of aHUS diagnosis and outcomes in patients managed in the ICU.

Methods  A review of the recent literature (January 2009 – March 2016) was performed to select the most relevant articles for ICU physicians.

Results  Based on the paucity of adult aHUS cases overall and within the ICU, no specific recommendations could be formally graded for the critical care setting. However, the expert panel recognises a core set of skills required by intensivists for diagnosing and managing patients with aHUS: recognising thrombotic microangiopathies, differentiating aHUS from related conditions, recognising involvement of other organ systems, understanding the pathophysiology of aHUS, knowing the diagnostic workup and relevant outcomes in critically ill aHUS patients, and knowing the standard of care for patients with aHUS based on available data and guidelines.

Conclusions  Managing critically ill patients with aHUS requires basic skills that, in the absence of sufficient data from patients treated within the ICU, can be gleaned from an increasingly relevant literature outside the ICU. More data on critically ill patients with aHUS are needed to validate these conclusions within the ICU setting.


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