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Measurement of C1-Inhibitor function alone is sufficient for diagnosis of hereditary angioedema

Kiani-Alikhan, S; Walker, E; Hickey, A; Grigoriadou, S; Buckland, M; Scott, C; (2021) Measurement of C1-Inhibitor function alone is sufficient for diagnosis of hereditary angioedema. Journal of Clinical Pathology 10.1136/jclinpath-2021-207538. (In press). Green open access

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Abstract

The World Allergy Organisiation/European Academy of Allergy and Clinical Immunology (WAO/EAACI) 2017/2018 guidelines recommend measuring complement4 levels, followed by C1-inhibitor level and function for diagnosis of hereditary angioedema (HAE). We analysed 6 months’ worth of data generated in our laboratory which is a specialist regional immunology service and also provides laboratory service for the Barts Health immunology department, which is a GA2LEN/HAEi-Angioedema Centre of Excellence and Reference (ACARE) and hence, investigates a large number of patients for HAE. We found that an efficient and sensitive approach for laboratory diagnosis of HAE is to only test the C1-inhibitor function. This approach had a sensitivity of 100% and reduced the cost of laboratory investigations for HAE diagnosis by 45%.

Type: Article
Title: Measurement of C1-Inhibitor function alone is sufficient for diagnosis of hereditary angioedema
Open access status: An open access version is available from UCL Discovery
DOI: 10.1136/jclinpath-2021-207538
Publisher version: http://dx.doi.org/10.1136/jclinpath-2021-207538
Language: English
Additional information: This version is the author accepted manuscript. For information on re-use, please refer to the publisher’s terms and conditions.
Keywords: Pathology, complement system proteins, medical laboratory science, skin, immune system diseases, diagnosis
UCL classification: UCL
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Population Health Sciences > UCL GOS Institute of Child Health
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Population Health Sciences > UCL GOS Institute of Child Health > Infection, Immunity and Inflammation Dept
URI: https://discovery.ucl.ac.uk/id/eprint/10141714
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