Please use this identifier to cite or link to this item: https://hdl.handle.net/2440/126176
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Type: Journal article
Title: Baseline characteristics of patients with atypical haemolytic uraemic syndrome (aHUS): the Australian cohort in a global aHUS registry
Author: Soraru, J.
Isbel, N.
Wong, G.
Coates, P.T.
Mantha, M.
Abraham, A.
Juneja, R.
Hsu, D.
Brown, F.
Bose, B.
Mudge, D.
Carroll, R.
Kausman, J.
Hughes, P.
Barbour, T.
Durkan, A.
Mount, P.
Lee, D.
Larkins, N.
Ranganathan, D.
et al.
Citation: Nephrology, 2020; 25(9):683-690
Publisher: © 2020 Asian Pacific Society of Nephrology
Issue Date: 2020
ISSN: 1320-5358
1440-1797
Statement of
Responsibility: 
Jacqueline Soraru, Nicole Isbel, Germaine Wong, Patrick Toby Coates, Murty Mantha ... Robert Carroll ... et al.
Abstract: AIMS:To describe the baseline characteristics and treatment of Australian patients diagnosed with atypical haemolytic uraemic syndrome (aHUS) reported to the Global aHUS Registry. METHODS:Descriptive analysis of the Australian cohort with aHUS (n = 106) was undertaken for demographics, disease characteristics and prior treatment with eculizumab; comparing with the global cohort (n = 1688) for certain pre-specified disease characteristics. RESULTS:In Australia, almost two-thirds of patients diagnosed with aHUS were female and over 80% of patients were Caucasians, with similar proportions reported in the global cohort. Less than 6% of patients in the Australia and global cohorts were reported to have a history of autoimmune disease (4% vs 2%, respectively; P = .21) or cancer (5% vs 5%, respectively; P = .93), conditions that have been associated with secondary HUS. In the Australian cohort, 26% had received a kidney transplant and 68% of patients had received eculizumab. Kidneys were the most common organ involvement, followed by gastrointestinal tract (26%) and cardiovascular system (19%), with 35% of patients reported to have had at least two organs involved within 6 months prior to baseline visit or entry into the registry. Complement factor H was the most common pathogenic complement gene variant in the Australian patients. CONCLUSION:Data from the aHUS registry confirms and defines region-specific disease characteristics among a selected group of Australian children and adults with aHUS reported to the registry. Ongoing and more inclusive data will provide further information about temporal trends and treatment outcomes, representing a unique opportunity for clinicians and researchers to further develop knowledge surrounding this rare disease.
Keywords: atypical haemolytic uraemic syndrome
complement gene mutation
eculizumab
kidney transplant
registry
Rights: © 2020 Asian Pacific Society of Nephrology
DOI: 10.1111/nep.13722
Grant ID: http://purl.org/au-research/grants/nhmrc/Career Development Fellowship
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