Abstract:
BACKGROUND:Atypical hemolytic uremic syndrome (aHUS) is a rare, genetically-mediated systemic disease most often caused by chronic, uncontrolled complement activation that leads to systemic thrombotic microangiopathy (TMA) and renal and other end-organ damage. METHODS:The global aHUS Registry, initiated in April 2012, is an observational, noninterventional, multicenter registry designed to collect demographic characteristics, medical and disease history, treatment effectiveness and safety outcomes data for aHUS patients. The global aHUS Registry will operate for a minimum of 5 years of follow-up. Enrollment is open to all patients with a clinical diagnosis of aHUS, with no requirement for identified complement gene mutations, polymorphisms or autoantibodies or particular type of therapy/management. RESULTS:As of September 30, 2014, 516 patients from 16 countries were enrolled. At enrollment, 315 (61.0 %) were adults (≥18 years) and 201 (39.0 %) were <18 years of age. Mean (standard deviation [SD]) age at diagnosis was 22.7 (20.5) years. Nineteen percent of patients had a family history of aHUS, 60.3 % had received plasma exchange/plasma infusion, 59.5 % had a history of dialysis, and 19.6 % had received ≥1 kidney transplant. Overall, 305 patients (59.1 %) have received eculizumab. CONCLUSIONS:As enrollment and follow-up proceed, the global aHUS Registry is expected to yield valuable baseline, natural history, medical outcomes, treatment effectiveness and safety data from a diverse population of patients with aHUS. TRIAL REGISTRATION:US National Institutes of Health www.ClinicalTrials.gov Identifier NCT01522183 . Registered January 18, 2012.
journal_name
BMC Nephroljournal_title
BMC nephrologyauthors
Licht C,Ardissino G,Ariceta G,Cohen D,Cole JA,Gasteyger C,Greenbaum LA,Johnson S,Ogawa M,Schaefer F,Vande Walle J,Frémeaux-Bacchi Vdoi
10.1186/s12882-015-0195-1subject
Has Abstractpub_date
2015-12-10 00:00:00pages
207issn
1471-2369pii
10.1186/s12882-015-0195-1journal_volume
16pub_type
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