Journal Article

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

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

2014

Overview

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.

• Publisher: Springer Berlin Heidelberg,Springer,Springer Nature B.V
• Subject: Adolescent / Apheresis / Atypical Hemolytic Uremic Syndrome - therapy / Audits / Care and treatment / Case reports / Catheters / Child / Child, Preschool / Clinical Audit / Clinical Medicine / Complications and side effects / Development and progression / Diarrhea / Female / Guideline Adherence - statistics & numerical data / Hematology / Hemolytic-uremic syndrome / Hospitals / Humans / Infant / Infant, Newborn / Infections / Kinases / Klinisk medicin / Male / Medical and Health Sciences / Medicin och hälsovetenskap / Medicine / Medicine & Public Health / Mutation / Nephrology / Original Article / Pathogenesis / Patient outcomes / Patients / Pediatrics / Pediatrik / Plasmapheresis / Practice Guidelines as Topic / Proteins / Questionnaires / Remission (Medicine) / Risk factors / Surveys and Questionnaires / Urology