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

Sally Johnson*, Jelena Stojanovic, Gema Ariceta, Martin Bitzan, Nesrin Besbas, Michelle Frieling, Diana Karpman, Daniel Landau, Craig Langman, Christoph Licht, Carmine Pecoraro, Magdalena Riedl, Ekaterini Siomou, Nicole van de Kar, Johan Vande Walle, Chantal Loirat, C. Mark Taylor

*Corresponding author for this work

Research output: Contribution to journalArticlepeer-review

58 Scopus citations

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.

Original languageEnglish (US)
Pages (from-to)1967-1978
Number of pages12
JournalPediatric Nephrology
Volume29
Issue number10
DOIs
StatePublished - Oct 2014

Keywords

  • Alternative complement pathway
  • Atypical hemolytic-uremic syndrome
  • Chronic kidney disease
  • Complement dysregulation
  • Plasmapheresis
  • Thrombotic microangiopathy

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health
  • Nephrology

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