Abstract:
:Haemophagocytic lymphohistiocytosis (HLH) is rare disorder characterised by immune activation leading to cytokine storm and end-organ failure. It is associated with poor prognosis. HLH is predominantly a disease of early childhood, where it often results from genetic defects that impair cytotoxic function of natural killer cells and T cells. In adults, clinical manifestations are similar to those observed in children, although the aetiology is often unclear. We hypothesised that the poor prognosis is in part due to lack of awareness of the disorder, which results in incomplete investigation and failure to implement timely treatment. We evaluated diagnosis of HLH according to standard criteria in a single centre. In this retrospective case series, we present the clinical characteristics, treatment, and outcome of a cohort of adult-onset HLH identified in a tertiary hospital in Australia. In our cohort, hyperferritinaemia ≥10,000 μg/L was highly sensitive in detecting patients with adult-onset HLH, however the majority of patients who had hyperferritinaemia ≥10,000 μg/L did not have adult-onset HLH. We highlight that incomplete assessment of HLH criteria is frequently seen. Greater awareness of this rare disease and its diagnostic criteria may improve patient outcomes. This article is protected by copyright. All rights reserved.
journal_name
Intern Med Jjournal_title
Internal medicine journalauthors
Lee WI,Talaulikar D,Cook MCdoi
10.1111/imj.14891subject
Has Abstractpub_date
2020-05-10 00:00:00eissn
1444-0903issn
1445-5994pub_type
杂志文章abstract:BACKGROUND:Management of acute ischaemic stroke is time critical. Reducing time to treatment with thrombolysis is strongly associated with improved outcomes in properly selected patients. However, there are barriers to ensuring timely treatment in the hospital setting. AIM:To determine if simple, no-cost protocol chan...
journal_title:Internal medicine journal
pub_type: 杂志文章
doi:10.1111/imj.14290
更新日期:2019-11-01 00:00:00
abstract:BACKGROUND:Exacerbations requiring hospital admission for chronic obstructive pulmonary disease (COPD) contribute to a decline in health status and are costly to the community. Long-term trends in admissions and associated outcomes are difficult to establish because of frequent readmissions, high case fatality and pote...
journal_title:Internal medicine journal
pub_type: 杂志文章
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journal_title:Internal medicine journal
pub_type: 杂志文章
doi:10.1111/imj.14159
更新日期:2019-08-01 00:00:00
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journal_title:Internal medicine journal
pub_type: 杂志文章
doi:10.1111/imj.15016
更新日期:2020-10-01 00:00:00
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更新日期:2020-07-01 00:00:00
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journal_title:Internal medicine journal
pub_type: 杂志文章,评审
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更新日期:2004-05-01 00:00:00
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pub_type: 杂志文章
doi:10.1111/j.1445-5994.2007.01518.x
更新日期:2008-04-01 00:00:00
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更新日期:2018-11-01 00:00:00
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journal_title:Internal medicine journal
pub_type: 杂志文章
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更新日期:2013-04-01 00:00:00
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pub_type: 杂志文章
doi:10.1111/imj.12753
更新日期:2015-06-01 00:00:00
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pub_type: 杂志文章,评审
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更新日期:2013-03-01 00:00:00
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pub_type: 杂志文章,评审
doi:10.1111/j.1445-5994.2012.02750.x
更新日期:2013-01-01 00:00:00
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pub_type: 杂志文章
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更新日期:2020-12-21 00:00:00
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pub_type: 杂志文章
doi:10.1111/imj.12179
更新日期:2013-08-01 00:00:00
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pub_type: 杂志文章
doi:10.1111/imj.13156
更新日期:2016-08-01 00:00:00
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pub_type: 杂志文章
doi:10.1111/imj.14017
更新日期:2018-09-01 00:00:00
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journal_title:Internal medicine journal
pub_type: 杂志文章
doi:10.1111/j.1445-5994.2006.01006.x
更新日期:2006-01-01 00:00:00
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pub_type: 杂志文章
doi:10.1111/j.1445-5994.2009.02061.x
更新日期:2010-10-01 00:00:00
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pub_type: 杂志文章
doi:10.1111/imj.13531
更新日期:2017-09-01 00:00:00
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更新日期:2020-06-01 00:00:00
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pub_type: 杂志文章,meta分析
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更新日期:2013-05-01 00:00:00
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更新日期:2018-08-01 00:00:00
abstract::Waldenström macroglobulinaemia (WM) is an indolent B-cell malignancy characterised by the presence of immunoglobulin M (IgM) paraprotein and bone marrow infiltration by clonal small B lymphocytes, plasmacytoid lymphocytes and plasma cells. The symptoms of WM are protean, often follow an asymptomatic phase and may incl...
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更新日期:2017-01-01 00:00:00
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doi:10.1111/j.1445-5994.2009.02143.x
更新日期:2010-04-01 00:00:00
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更新日期:2007-11-01 00:00:00
abstract:BACKGROUND:Non-alcoholic fatty liver disease (NAFLD) is a common cause of incidental liver test abnormalities. General practitioners (GP) have a key role in identifying people with NAFLD at risk of significant liver disease. Recent specialist guidelines emphasise the use of fibrosis algorithms or serum biomarkers rathe...
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pub_type: 杂志文章
doi:10.1111/imj.13667
更新日期:2018-02-01 00:00:00