Show simple item record

dc.contributor.authorSoraru, Jacqueline
dc.contributor.authorIsbel, Nicole
dc.contributor.authorWong, Germaine
dc.contributor.authorCoates, Patrick Toby
dc.contributor.authorMantha, Murty
dc.contributor.authorAbraham, Abu
dc.contributor.authorJuneja, Rajiv
dc.contributor.authorHsu, Danny
dc.contributor.authorBrown, Fiona
dc.contributor.authorBose, Bhadran
dc.contributor.authorMudge, David
dc.contributor.authorCarroll, Robert
dc.contributor.authorKausman, Joshua
dc.contributor.authorHughes, Peter
dc.contributor.authorBarbour, Thomas
dc.contributor.authorDurkan, Anne
dc.contributor.authorMount, Peter
dc.contributor.authorLee, Darren
dc.contributor.authorLarkins, Nicholas
dc.contributor.authorRanganathan, Dwarakanathan
dc.contributor.authorLim, Wai H
dc.date.accessioned2020-05-11T01:19:46Z
dc.date.available2020-05-11T01:19:46Z
dc.date.issued2020
dc.identifier.issn1320-5358
dc.identifier.doi10.1111/nep.13722
dc.identifier.urihttp://hdl.handle.net/10072/393737
dc.description.abstractAIMS: 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 = 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. 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.
dc.description.peerreviewedYes
dc.languageEnglish
dc.language.isoeng
dc.publisherWiley
dc.relation.ispartofjournalNephrology
dc.subject.fieldofresearchClinical Sciences
dc.subject.fieldofresearchcode1103
dc.subject.keywordsatypical haemolytic uraemic syndrome
dc.subject.keywordscomplement gene mutation
dc.subject.keywordseculizumab
dc.subject.keywordskidney transplant
dc.subject.keywordsregistry
dc.titleBaseline characteristics of patients with atypical haemolytic uraemic syndrome (aHUS): the Australian cohort in a global aHUS registry.
dc.typeJournal article
dc.type.descriptionC1 - Articles
dcterms.bibliographicCitationSoraru, J; Isbel, N; Wong, G; Coates, PT; Mantha, M; Abraham, A; Juneja, R; Hsu, D; Brown, F; Bose, B; Mudge, D; Carroll, R; Kausman, J; Ranganathan, D; et al., Baseline characteristics of patients with atypical haemolytic uraemic syndrome (aHUS): the Australian cohort in a global aHUS registry., Nephrology, 2020
dcterms.dateAccepted2020-04-14
dc.date.updated2020-05-11T00:54:47Z
dc.description.versionAccepted Manuscript (AM)
gro.description.notepublicThis publication was entered as an advanced online version.
gro.rights.copyright© 2020 Asian Pacific Society of Nephrology. This is the peer reviewed version of the following article: Baseline characteristics of patients with atypical haemolytic uraemic syndrome (aHUS): the Australian cohort in a global aHUS registry, Nephrology, 2020, which has been published in final form at https://doi.org/10.1111/nep.13722. This article may be used for non-commercial purposes in accordance with Wiley Terms and Conditions for Self-Archiving (http://olabout.wiley.com/WileyCDA/Section/id-828039.html)
gro.hasfulltextFull Text
gro.griffith.authorRanganathan, Dwarakanathan


Files in this item

This item appears in the following Collection(s)

  • Journal articles
    Contains articles published by Griffith authors in scholarly journals.

Show simple item record