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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 |
Published version: | http://dx.doi.org/10.1111/nep.13722 |
Appears in Collections: | Aurora harvest 4 Medicine publications |
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