مورد إلكتروني
Diagnosis and management of thrombotic thrombocytopenic purpura (TTP) in Australia: Findings from the first 5 years of the Australian TTP/thrombotic microangiopathy registry.
| العنوان: | Diagnosis and management of thrombotic thrombocytopenic purpura (TTP) in Australia: Findings from the first 5 years of the Australian TTP/thrombotic microangiopathy registry. |
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| بيانات النشر: | Blackwell Publishing Australia 2016-02-12 |
| تفاصيل مُضافة: | Polizzotto M.N. Phillips L.E. Wood E. Engelbrecht S. Cohney S. Mcquilten Z. Pepperell D. Kivivali L. Blombery P. |
| نوع الوثيقة: | Electronic Resource |
| مستخلص: | Background: Thrombotic thrombocytopenic purpura (TTP) is a rare, life-threatening thrombotic microangiopathy (TMA). In 2009, the Australian TTP/TMA registry was established to collect data on patients presenting with TTP/TMA throughout Australia. Aim(s): To summarise information on the diagnosis and management of patients with TTP collected in the first 5years (2009-2014) of the Australian TTP registry. Method(s): Registry data from June 2009 to October 2014 were reviewed. Result(s): Fifty-seven patients were identified with TTP (defined as ADAMTS13 activity <10%), accounting for 72 clinical episodes. ADAMTS13 inhibitor testing was performed in nine out of 57 patients (16%), reflecting the limited availability of accredited testing facilities. Sixty-seven out of 72 episodes were treated with therapeutic plasma exchange (PEx) using cryodepleted plasma (40% of episodes), fresh frozen plasma (36%) or a mixture (22%). Median exposure to plasma products was 55.9L. PEx was commenced >=2days from stated diagnosis in 15% of episodes. Adverse reactions to PEx were common with documented allergic reactions (including life threatening) in 21% of episodes. Adjunctive immunosuppression was documented in 76% of episodes (corticosteroid 71% and rituximab 39%). Platelet transfusion was administered in 15% of episodes. Conclusion(s): Data from the Australian TTP/TMA registry suggest a heterogenous approach to the diagnosis and management of TTP in Australia over the assessed period. These observations highlight areas for improvement and standardisation of practice, including comprehensive diagnostic testing, more immediate access to PEx and a more uniform approach to adjunctive immunosuppression and supportive care.Copyright © 2016 Royal Australasian College of Physicians. |
| مصطلحات الفهرس: | thrombotic thrombocytopenic purpura/ep [Epidemiology], thrombotic thrombocytopenic purpura/th [Therapy], treatment duration, corticosteroid/dt [Drug Therapy], cyclophosphamide/dt [Drug Therapy], rituximab/dt [Drug Therapy], vincristine/dt [Drug Therapy], von Willebrand factor cleaving proteinase/ec [Endogenous Compound], medical history, adult, allergic reaction/co [Complication], article, Australia, Australian, disease association, disease registry, female, human, immunosuppressive treatment, laboratory diagnosis, major clinical study, male, patient information, plasma transfusion, plasmapheresis, practice guideline, priority journal, thrombocyte transfusion, thrombotic thrombocytopenic purpura/di [Diagnosis], thrombotic thrombocytopenic purpura/dt [Drug Therapy], Article |
| URL: | Internal Medicine Journal LibKey Link |
| الإتاحة: | Open access content. Open access content Copyright 2019 Elsevier B.V., All rights reserved. |
| أرقام أخرى: | AUSHL oai:repository.monashhealth.org:1/39661 Internal Medicine Journal. 46 (1) (pp 71-79), 2016. Date of Publication: 01 Jan 2016. 1444-0903 https://repository.monashhealth.org/monashhealthjspui/handle/1/39661Test 26477687 [http://www.ncbi.nlm.nih.gov/pubmed/?term=26477687Test] 608037247 (Blombery) Department of Haematology, Peter MacCallum Cancer Centre, Melbourne, Australia (Blombery, Kivivali, Pepperell, Mcquilten, Engelbrecht, Polizzotto, Phillips, Wood, Cohney) Department of Epidemiology and Preventive Medicine, Monash University, Melbourne, Australia (Pepperell) Fiona Stanley Hospital, Perth, Australia (Engelbrecht) Gold Coast University Hospital, QLD, Australia (Polizzotto) St Vincent's Hospital, Sydney, Australia (Polizzotto) The Kirby Institute, University of New South Wales, Sydney, Australia (Wood) Monash Medical Centre, Melbourne, Australia (Cohney) Department of Medicine, University of Melbourne, Melbourne, Australia (Cohney) Departments of Nephrology, Western and Royal Melbourne Hospitals, Melbourne, Australia Blombery P.; piers.blombery@petermac.org 1305113592 |
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| رقم الانضمام: | edsoai.on1305113592 |
| قاعدة البيانات: | OAIster |
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