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Title: Baseline characteristics of patients with atypical haemolytic uraemic syndrome (aHUS): the Australian cohort in a global aHUS registry.
Austin Authors: Soraru, Jacqueline;Isbel, Nicole;Wong, Germaine;Coates, Patrick Toby;Mantha, Murty;Abraham, Abu;Juneja, Rajiv;Hsu, Danny;Brown, Fiona;Bose, Bhadran;Mudge, David;Carroll, Robert;Kausman, Joshua;Hughes, Peter;Barbour, Thomas;Durkan, Anne;Mount, Peter F ;Lee, Darren;Larkins, Nicholas;Ranganathan, Dwarakanathan;Lim, Wai H
Affiliation: School of Public Health, University of Sydney, Sydney, Australia
Department of Haematology, Liverpool Hospital, Sydney, Australia
Flinders Medical Centre, Adelaide, Australia
Department of Nephrology and Renal Transplant, Fiona Stanley Hospital, Perth, Australia
Department of Nephrology, Cairns Base Hospital, Cairns, Australia
Adelaide Health and Medical Sciences, University of Adelaide, Adelaide, Australia
Centre for Transplant and Renal Research, Westmead Hospital, Sydney, Australia
Centre for Kidney Research, The Children's Hospital at Westmead, Sydney, Australia
Department of Nephrology, Princess Alexandra Hospital, Brisbane, Australia
Department of Renal Medicine, Sir Charles Gairdner Hospital, Perth, Australia
School of Medicine, University of Western Australia, Perth, Australia
Kidney Health Service, Royal Brisbane and Women's Hospital, Brisbane, School of Medicine, Griffith University, Australia
Department of Nephrology and Hypertension, Perth Children's Hospital, Perth, Australia
Department of Renal Medicine, Eastern Health Clinical School, Monash University Melbourne, Victoria, Australia
Department of Nephrology, The Children's Hospital at Westmead, Sydney, Australia
Department of Nephrology and Transplantation, The Royal Melbourne Hospital, Melbourne, Australia
Department of Nephrology and Renal Transplantation, The Royal Children's Hospital, Melbourne, Australia
Central and Northern Renal and Transplantation Service, Royal Adelaide Hospital, Adelaide, Australia
Department of Nephrology, Nepean Hospital, Blue Mountains, Australia
Department of Nephrology, Monash Medical Centre, Melbourne, Australia
Issue Date: Sep-2020
Date: 2020-05-07
Publication information: Nephrology 2020
Abstract: To describe the baseline characteristics and treatment of Australian patients diagnosed with atypical haemolytic uraemic syndrome (aHUS) reported to the Global aHUS Registry. 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. 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 = 0.21) or cancer (5% vs 5%, respectively; P = 0.93), 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 2 organs involved within 6 months prior to baseline visit or entry into the registry. Complement factor H (CFH) was the most common pathogenic complement gene variant in the Australian patients. 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. This article is protected by copyright. All rights reserved.
DOI: 10.1111/nep.13722
ORCID: 0000-0001-6308-0691
Journal: Nephrology
PubMed URL: 32378251
Type: Journal Article
Subjects: atypical haemolytic uraemic syndrome
complement gene mutation
kidney transplant
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