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

Johnson, Sally; Stojanovic, Jelena; Ariceta, Gema; Bitzan, Martin; Besbas, Nesrin; Frieling, Michelle; Karpman, Diana LU ; Landau, Daniel; Langman, Craig and Licht, Christoph, et al. (2014) In Pediatric Nephrology 29(10). p.1967-1978
Abstract
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... (More)
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. (Less)
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Contribution to journal
publication status
published
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keywords
Atypical hemolytic-uremic syndrome, Alternative complement pathway, Plasmapheresis, Complement dysregulation, Chronic kidney disease, Thrombotic microangiopathy
in
Pediatric Nephrology
volume
29
issue
10
pages
1967 - 1978
publisher
Springer
external identifiers
  • wos:000342228000011
  • scopus:84930238594
ISSN
1432-198X
DOI
10.1007/s00467-014-2817-4
language
English
LU publication?
yes
id
d928cbe4-bba9-4cae-aacb-8a4b13609c7c (old id 4699710)
date added to LUP
2014-11-03 07:20:26
date last changed
2017-06-25 03:57:31
@article{d928cbe4-bba9-4cae-aacb-8a4b13609c7c,
  abstract     = {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.},
  author       = {Johnson, Sally and Stojanovic, Jelena and Ariceta, Gema and Bitzan, Martin and Besbas, Nesrin and Frieling, Michelle and Karpman, Diana and Landau, Daniel and Langman, Craig and Licht, Christoph and Pecoraro, Carmine and Riedl, Magdalena and Siomou, Ekaterini and van de Kar, Nicole and Vande Walle, Johan and Loirat, Chantal and Taylor, C. Mark},
  issn         = {1432-198X},
  keyword      = {Atypical hemolytic-uremic syndrome,Alternative complement pathway,Plasmapheresis,Complement dysregulation,Chronic kidney disease,Thrombotic microangiopathy},
  language     = {eng},
  number       = {10},
  pages        = {1967--1978},
  publisher    = {Springer},
  series       = {Pediatric Nephrology},
  title        = {An audit analysis of a guideline for the investigation and initial therapy of diarrhea negative (atypical) hemolytic uremic syndrome},
  url          = {http://dx.doi.org/10.1007/s00467-014-2817-4},
  volume       = {29},
  year         = {2014},
}