Pediatric Nephrology

, Volume 29, Issue 10, pp 1967–1978 | Cite as

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
Original Article



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.


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.


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.


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.


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


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Copyright information

© IPNA 2014

Authors and Affiliations

  • Sally Johnson
    • 1
    • 17
  • Jelena Stojanovic
    • 1
  • Gema Ariceta
    • 2
  • Martin Bitzan
    • 3
  • Nesrin Besbas
    • 4
  • Michelle Frieling
    • 5
  • Diana Karpman
    • 6
  • Daniel Landau
    • 7
  • Craig Langman
    • 8
    • 9
  • Christoph Licht
    • 5
  • Carmine Pecoraro
    • 10
  • Magdalena Riedl
    • 11
  • Ekaterini Siomou
    • 12
  • Nicole van de Kar
    • 13
  • Johan Vande Walle
    • 14
  • Chantal Loirat
    • 15
  • C. Mark Taylor
    • 16
  1. 1.Great North Children’s HospitalNewcastle Upon TyneUK
  2. 2.Hospital Vall d’ Hebron BarcelonaBarcelonaSpain
  3. 3.Montreal Children’s Hospital and McGill UniversityMontrealCanada
  4. 4.Hacettepe University Faculty of Medicine, Department of Pediatric NephrologyAnkaraTurkey
  5. 5.The Hospital For Sick ChildrenTorontoCanada
  6. 6.Department of Pediatrics, Clinical SciencesLund UniversityLundSweden
  7. 7.Soroka University Medical CenterBeer ShevaIsrael
  8. 8.Feinberg School of MedicineNorthwestern UniversityChicagoUSA
  9. 9.The Ann and Robert H. Lurie Children’s Hospital of ChicagoChicagoUSA
  10. 10.Santobono Children’s HospitalNaplesItaly
  11. 11.Department of PaediatricsInnsbruck Medical UniversityInnsbruckAustria
  12. 12.University Hospital of IoanninaIoanninaGreece
  13. 13.Radboud University Medical CenterNijmegenThe Netherlands
  14. 14.University Hospital GentGhentBelgium
  15. 15.Assistance Publique-Hôpitaux de ParisHôpital Robert DebréParisFrance
  16. 16.Birmingham Children’s HospitalBirminghamUK
  17. 17.Department of Pediatric Nephrology, Great North Children’s HospitalNewcastle Upon Tyne Hospitals NHS Foundation TrustNewcastle Upon TyneUK

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