title: An audit analysis of a guideline for the investigation and initial therapy of diarrhea negative (atypical) hemolytic uremic syndrome.
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.
authors.affiliation: Great North Children's Hospital, Newcastle Upon Tyne, UK, sally.johnson@nuth.nhs.uk.
authors.forename: Sally
authors.initials: S
authors.lastname: Johnson
authors.forename: Jelena
authors.initials: J
authors.lastname: Stojanovic
authors.forename: Gema
authors.initials: G
authors.lastname: Ariceta
authors.forename: Martin
authors.initials: M
authors.lastname: Bitzan
authors.forename: Nesrin
authors.initials: N
authors.lastname: Besbas
authors.forename: Michelle
authors.initials: M
authors.lastname: Frieling
authors.forename: Diana
authors.initials: D
authors.lastname: Karpman
authors.forename: Daniel
authors.initials: D
authors.lastname: Landau
authors.forename: Craig
authors.initials: C
authors.lastname: Langman
authors.forename: Christoph
authors.initials: C
authors.lastname: Licht
authors.forename: Carmine
authors.initials: C
authors.lastname: Pecoraro
authors.forename: Magdalena
authors.initials: M
authors.lastname: Riedl
authors.forename: Ekaterini
authors.initials: E
authors.lastname: Siomou
authors.forename: Nicole
authors.initials: N
authors.lastname: van de Kar
authors.forename: Johan Vande
authors.initials: JV
authors.lastname: Walle
authors.forename: Chantal
authors.initials: C
authors.lastname: Loirat
authors.forename: C Mark
authors.initials: CM
authors.lastname: Taylor
mesh_terms: D000293:Adolescent; D065766:Atypical Hemolytic Uremic Syndrome / Q000628:therapy*; D002648:Child; D002675:Child, Preschool; D054869:Clinical Audit; D005260:Female; D019983:Guideline Adherence / Q000706:statistics & numerical data*; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D017410:Practice Guidelines as Topic; D011795:Surveys and Questionnaires