Baseline characteristics of patients with atypical haemolytic uraemic syndrome ( aHUS ): The Australian cohort in a global aHUS registry

Autor:	Peter Hughes, Robert P. Carroll, Jacqueline Soraru, Joshua Kausman, Darren Lee, Wai H. Lim, Dwarakanathan Ranganathan, Nicholas Larkins, Nicole M. Isbel, Bhadran Bose, Peter F Mount, Thomas D. Barbour, Fiona G. Brown, Patrick T. Coates, Murty Mantha, Anne M. Durkan, Germaine Wong, David W. Mudge, Danny Hsu, Abu Abraham, Rajiv Juneja
Rok vydání:	2020
Předmět:	Adult Male medicine.medical_specialty 030232 urology & nephrology Disease 030204 cardiovascular system & hematology Antibodies Monoclonal Humanized Kidney 03 medical and health sciences 0302 clinical medicine Internal medicine Humans Medicine Registries Child Atypical Hemolytic Uremic Syndrome Demography Autoimmune disease business.industry Australia Cancer General Medicine Eculizumab medicine.disease Kidney Transplantation Discontinuation Gastrointestinal Tract Complement Inactivating Agents Nephrology Complement Factor H Factor H Mutation Cohort Female business medicine.drug Rare disease
Zdroj:	Nephrology. 25:683-690
ISSN:	1440-1797 1320-5358
DOI:	10.1111/nep.13722
Popis:	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.
Databáze:	OpenAIRE
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