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An audit analysis of a guideline for the investigation and initial therapy of diarrhea negative (atypical) hemolytic uremic syndrome

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Abstract

Background

In 2009, the European Paediatric Study Group for Haemolytic Uraemic Syndrome (HUS) published a clinical practice guideline for the investigation and initial therapy of diarrhea-negative HUS (now more widely referred to as atypical HUS, aHUS). The therapeutic component of the guideline (comprising early, high-volume plasmapheresis) was derived from anecdotal evidence and expert consensus, and the authors committed to auditing outcome.

Methods

Questionnaires were distributed to pediatric nephrologists across Europe, North America, and the Middle East, who were asked to complete one questionnaire per patient episode of aHUS between July 1, 2009 and December 31, 2010. Comprehensive, anonymous demographic and clinical data were collected.

Results

Seventy-one children were reported with an episode of aHUS during the audit period. Six cases occurred on a background of influenza A H1N1 infection. Of 71 patients, 59 (83 %) received plasma therapy within the first 33 days, of whom ten received plasma infusion only. Complications of central venous catheters occurred in 16 out of 51 patients with a catheter in-situ (31 %). Median time to enter hematological remission was 11.5 days, and eight of 71 (11 %) patients did not enter hematological remission by day 33. Twelve patients (17 %) remained dialysis dependent at day 33.

Conclusions

This audit provides a snapshot of the early outcome of a group of children with aHUS in the months prior to more widespread use of eculizumab.

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Declaration of interests

The following authors declare interests. CMT and MB were scientific advisers to Thallion Pharmaceuticals. SJ, NvdK, GA, JVW, C. Licht, and MR have served on Advisory Boards for Alexion Pharmaceuticals. DK, C. Licht, and C. Loirat were the national coordinators of the multi-center Eculizumab trials in aHUS in Sweden, Canada, and France, respectively.

The European Paediatric Study Group for HUS is now known as HUS International and comprises pediatric nephrologists from Europe, North America, and the Middle East.

Contributors

Anne-Laure Adra1, Klaus Arbeiter2, Yelda Bilginer3, Heiko Billing4, Maria Bitsori5, Bernard Boudailliez6, Francisco de la Cerda – Ojeda7, Gérard Champion8, Amit Dagan9, Bradley Dixon, MD10, Philippe Eckart11, Israel Eisenstein12, Arnaud Garnier13, Clara Gomes14, Karsten Häffner15, Jerome Harambat16, Carol Inward17, Rafael Krmar18, Theresa Kwon19, Anne-Laure Lapeyraque20, Sebastian Loos21, Meeta Malik22, Mignon McCulloch23, David Milford24, Danko Milosevic25, Carla Nester26, François Nobili27, Pedro J Ortega28, Fotios Papachristou29, Antonia Peña - Carrión30, Ludmila Podracká31, Nikoleta Printza29, Bruno Ranchin32, Christopher Reid23, Caroline Rousset-Rouvière33, Rémi Salomon34, Anne-Laure Sellier-Leclerc35, James Springate36, Sophie Taque37, Despoina Tramma38, Sibylle Tschumi39, Ulrike Walden40, Kay Tyerman41, Aoife Waters42, Donald J Weaver Jr43

1Pediatric nephrology department, Hôpital A. de Villeneuve, Montpellier, France

2Medical University Vienna, Pediatric Nephrology, Vienna, Austria

3Hacettepe University Faculty of Medicine Pediatric Nephrology, Ankara, Turkey

4University Hospital Heidelberg, Pediatric Nephrology, Heidelberg, Germany

5Department of Pediatrics, University Hospital of Heraklion, Greece

6Department of Pediatrics, Centre Hospitalier Universitaire Nord, Amiens , France

7Virgen del Rocío Children´s Hospital, Division of Pediatric Nephrology, Seville, Spain

8Department of Pediatrics, Centre Hospitalier Universitaire, Angers, France

9Schneider Children's Hospital, Petach Tikvah (Tel Aviv University), Israel

10Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio, USA

11Department of Pediatrics, Centre Hospitalier Universitaire, Caen, France

12Myers Children's Hospital (Rambam Medical Center), Haifa (Technion University), Israel

13Pediatric nephrology department, Centre Hospitalier Universitaire Purpan, Hôpital des enfants, Toulouse, France

14Coimbra Pediatric Hospital, Division of Pediatric Nephrology, Coimbra, Portugal

15University Hospital Freiburg, Department of Pediatrics, Freiburg, Germany

16Department of Pediatrics, Centre Hospitalier Universitaire, Bordeaux, France

17Department of Paediatric Nephrology, Bristol Royal Hospital for Children, Bristol, UK

18Karolinska University Hospital Huddinge, Stockholm, Sweden

19Pediatric nephrology department Hôpital Robert Debré, Paris, France

20Centre Hospitalier Universitaire Sainte Justine, Montreal Quebec

21University Hospital Hamburg, Pediatric Nephrology, Hamburg, Germany

22Children's Renal & Urology Unit, Nottingham University Hospitals, Nottingham, UK

23Paediatric Nephro-urology Unit, Evelina Children’s Hospital, London, UK

24Birmingham Children’s Hospital, Birmingham, UK

25University Hospital Center Zagreb, Croatia

26The University of Iowa, Iowa City, Iowa, USA

27Pediatric Nephrology, Centre Hospitalier Universitaire, Besançon, France

28La Fe Hospital, Division of Pediatric Nephrology, Valencia, Spain

291st Department of Paediatrics, Hippokration General Hospital, Aristotle University of Thessaloniki, Thessaloniki, Greece

30La Paz Universitary Hospital, Division of Pediatric Nephrology, Madrid, Spain

31Children's Hospital of P.J. Safarik's University, 1st Pediatric Department, Košice, Slovakia

32Pediatric nephrology department, Hospices Civils de Lyon, Lyon, France

33Pediatric nephrology department, Hôpital de la Timone, Marseille, France

34Pediatric nephrology department, Hôpital Necker-Enfants Malades, Paris, France

35Pediatric nephrology department, Hôpital Trousseau, Paris, France

36Division of Nephrology, Women & Children's Hospital, Buffalo, New York, USA

37Department of Pediatrics, Centre Hospitalier Universitaire, Rennes, France

38Aristotle University of Thessaloniki, Thessaloniki, Greece

39Paediatric Nephrology, University Children's Hospital Bern, Switzerland

40Childrens Hospital Augsburg, Augsburg, Germany

41Department of Paediatric Nephrology, Leeds General Infirmary, Leeds, UK

42Department of Paediatric Nephrology, Great Ormond Street Hospital, London, UK

43Levine Children's Hospital at Carolinas Medical Center, Charlotte, North Carolina, USA

Author information

Authors and Affiliations

  1. Great North Children’s Hospital, Newcastle Upon Tyne, UK

    Sally Johnson & Jelena Stojanovic

  2. Hospital Vall d’ Hebron Barcelona, Barcelona, Spain

    Gema Ariceta

  3. Montreal Children’s Hospital and McGill University, Montreal, Canada

    Martin Bitzan

  4. Hacettepe University Faculty of Medicine, Department of Pediatric Nephrology, Ankara, Turkey

    Nesrin Besbas

  5. The Hospital For Sick Children, Toronto, Canada

    Michelle Frieling & Christoph Licht

  6. Department of Pediatrics, Clinical Sciences, Lund University, Lund, Sweden

    Diana Karpman

  7. Soroka University Medical Center, Beer Sheva, Israel

    Daniel Landau

  8. Feinberg School of Medicine, Northwestern University, Chicago, IL, USA

    Craig Langman

  9. The Ann and Robert H. Lurie Children’s Hospital of Chicago, Chicago, IL, USA

    Craig Langman

  10. Santobono Children’s Hospital, Naples, Italy

    Carmine Pecoraro

  11. Department of Paediatrics, Innsbruck Medical University, Innsbruck, Austria

    Magdalena Riedl

  12. University Hospital of Ioannina, Ioannina, Greece

    Ekaterini Siomou

  13. Radboud University Medical Center, Nijmegen, The Netherlands

    Nicole van de Kar

  14. University Hospital Gent, Ghent, Belgium

    Johan Vande Walle

  15. Assistance Publique-Hôpitaux de Paris, Hôpital Robert Debré, Paris, France

    Chantal Loirat

  16. Birmingham Children’s Hospital, Birmingham, UK

    C. Mark Taylor

  17. Department of Pediatric Nephrology, Great North Children’s Hospital, Newcastle Upon Tyne Hospitals NHS Foundation Trust, Newcastle Upon Tyne, NE1 4LP, UK

    Sally Johnson

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  1. Sally Johnson
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  2. Jelena Stojanovic
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  3. Gema Ariceta
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  6. Michelle Frieling
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  7. Diana Karpman
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Correspondence to Sally Johnson.

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Johnson, S., Stojanovic, J., Ariceta, G. et al. An audit analysis of a guideline for the investigation and initial therapy of diarrhea negative (atypical) hemolytic uremic syndrome. Pediatr Nephrol 29, 1967–1978 (2014). https://doi.org/10.1007/s00467-014-2817-4

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  • DOI: https://doi.org/10.1007/s00467-014-2817-4

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