مورد إلكتروني
Baseline characteristics of patients with atypical haemolytic uraemic syndrome (aHUS): The Australian cohort in a global aHUS registry.
| العنوان: | Baseline characteristics of patients with atypical haemolytic uraemic syndrome (aHUS): The Australian cohort in a global aHUS registry. |
|---|---|
| بيانات النشر: | Blackwell Publishing Australia 2020-09-07 |
| تفاصيل مُضافة: | Larkins N. Barbour T. Durkan A. Mount P. Lee D. Ranganathan D. Lim W.H. 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. |
| نوع الوثيقة: | Electronic Resource |
| مستخلص: | Aims: To describe the baseline characteristics and treatment of Australian patients diagnosed with atypical haemolytic uraemic syndrome (aHUS) reported to the Global aHUS Registry. Method(s): 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. Result(s): 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(s): 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.Copyright © 2020 Asian Pacific Society of Nephrology |
| مصطلحات الفهرس: | controlled study, demography, disease registry, ethnic difference, family history, female, gastrointestinal disease, genetic variability, hemodialysis, hemolytic uremic syndrome/dt [Drug Therapy], human, kidney disease/su [Surgery], kidney disease/th [Therapy], kidney graft, kidney transplantation, major clinical study, complement factor H/ec [Endogenous Compound], Caucasian, clinical feature, cohort analysis, priority journal, sex difference, trend study, cardiovascular disease, eculizumab/dt [Drug Therapy], adult, age distribution, article, Australian, autoimmune disease, male, malignant neoplasm, medical history, Article |
| URL: | Nephrology LibKey Link |
| الإتاحة: | Open access content. Open access content Copyright 2020 Elsevier B.V., All rights reserved. |
| أرقام أخرى: | AUSHL oai:repository.monashhealth.org:1/29108 Nephrology. 25 (9) (pp 683-690), 2020. Date of Publication: 01 Sep 2020. 1320-5358 https://repository.monashhealth.org/monashhealthjspui/handle/1/29108Test 32378251 [http://www.ncbi.nlm.nih.gov/pubmed/?term=32378251Test] 2005116118 (Soraru, Lim) Department of Renal Medicine, Sir Charles Gairdner Hospital, Perth, WA, Australia (Isbel, Mudge) Department of Nephrology, Princess Alexandra Hospital, Brisbane, QLD, Australia (Wong) Centre for Transplant and Renal Research, Westmead Hospital, Sydney, NSW, Australia (Wong) Centre for Kidney Research, The Children's Hospital at Westmead, Sydney, NSW, Australia (Wong) School of Public Health, University of Sydney, Sydney, NSW, Australia (Coates, Carroll) Central and Northern Renal and Transplantation Service, Royal Adelaide Hospital, Adelaide, SA, Australia (Coates) Adelaide Health and Medical Sciences, University of Adelaide, Adelaide, SA, Australia (Mantha) Department of Nephrology, Cairns Base Hospital, Cairns, QLD, Australia (Abraham) Department of Nephrology and Renal Transplant, Fiona Stanley Hospital, Perth, WA, Australia (Juneja) Flinders Medical Centre, Adelaide, SA, Australia (Hsu) Department of Haematology, Liverpool Hospital, Sydney, NSW, Australia (Brown) Department of Nephrology, Monash Medical Centre, Melbourne, VIC, Australia (Bose) Department of Nephrology, Nepean Hospital, Blue Mountains, NSW, Australia (Kausman) Department of Nephrology and Renal Transplantation, The Royal Children's Hospital, Melbourne, VIC, Australia (Hughes, Barbour) Department of Nephrology and Transplantation, The Royal Melbourne Hospital, Melbourne, VIC, Australia (Durkan) Department of Nephrology, The Children's Hospital at Westmead, Sydney, NSW, Australia (Mount) Department of Nephrology, Austin Health, Melbourne, Australia (Lee) Department of Renal Medicine, Eastern Health Clinical School, Monash University Melbourne, Melbourne, VIC, Australia (Larkins) Department of Nephrology and Hypertension, Perth Children's Hospital, Perth, WA, Australia (Larkins, Lim) School of Medicine, University of Western Australia, Perth, WA, Australia (Ranganathan) Kidney Health Service, Royal Brisbane and Women's Hospital, Brisbane, School of Medicine, Griffith University, Mount Soraru J.; jacqueline.soraru@health.wa.gov.au 1305116824 |
| المصدر المساهم: | MONASH HEALTH LIBRS From OAIster®, provided by the OCLC Cooperative. |
| رقم الانضمام: | edsoai.on1305116824 |
| قاعدة البيانات: | OAIster |
| الوصف غير متاح. |