The global aHUS registry: methodology and initial patient characteristics View Full Text


Ontology type: schema:ScholarlyArticle      Open Access: True


Article Info

DATE

2015-12

AUTHORS

Christoph Licht, Gianluigi Ardissino, Gema Ariceta, David Cohen, J. Alexander Cole, Christoph Gasteyger, Larry A. Greenbaum, Sally Johnson, Masayo Ogawa, Franz Schaefer, Johan Vande Walle, Véronique Frémeaux-Bacchi

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. More... »

PAGES

207

Identifiers

URI

http://scigraph.springernature.com/pub.10.1186/s12882-015-0195-1

DOI

http://dx.doi.org/10.1186/s12882-015-0195-1

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https://app.dimensions.ai/details/publication/pub.1044460496

PUBMED

https://www.ncbi.nlm.nih.gov/pubmed/26654630


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curl -H 'Accept: application/n-triples' 'https://scigraph.springernature.com/pub.10.1186/s12882-015-0195-1'

Turtle is a human-readable linked data format.

curl -H 'Accept: text/turtle' 'https://scigraph.springernature.com/pub.10.1186/s12882-015-0195-1'

RDF/XML is a standard XML format for linked data.

curl -H 'Accept: application/rdf+xml' 'https://scigraph.springernature.com/pub.10.1186/s12882-015-0195-1'


 

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