Publications

Publications, Books, Book Chapters and Reviews by Prof. Marcus Maurer, MD

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Hereditary Angioedema Caused By C1-Esterase Inhibitor Deficiency: A Literature-Based Analysis and Clinical Commentary on Prophylaxis Treatment Strategies

Filename 53. Gower et al, HAE C1Est.Inh., WAO J 2011.pdf
Filesize 968 KB
Version r.053
Date added June 26, 2020
Downloaded 0 times
Category Reviews
Tags angioedema, attenuated androgen, C1-esterase inhibitor, HAE, Prophylaxis
Authors Gower, R. G., Busse, P. J., Aygören-Pürsün, E., Barakat, A. J., Caballero, T., Davis-Lorton, M., Farkas, H., Hurewitz, D. S., Jacobs, J. S., Johnston, D. T., Lumry, W., and Maurer, M.
Citation Gower, R. G., Busse, P. J., Aygören-Pürsün, E., Barakat, A. J., Caballero, T., Davis-Lorton, M., Farkas, H., Hurewitz, D. S., Jacobs, J. S., Johnston, D. T., Lumry, W., and Maurer, M.: Hereditary angioedema caused by C1-Esterase inhibitor deficiency: a literature-based analysis and clinical commentary on prophylaxis treatment strategies. WAO Journal 2011: 4; S9-S21.
Corresponding authors Gower, R. G.
DocNum R.53
DocType PDF
Edition; Page 4; S9-S21
IF N/A
Publisher WAO Journal
ReleaseDate 2011

Hereditary angioedema (HAE) caused by C1-esterase inhibitor deficiency is an autosomal-dominant disease resulting from a mutation in the C1-inhibitor gene. HAE is characterized by recurrent attacks of intense, massive, localized subcutaneous edema involving the extremities, genitalia, face, or trunk, or submucosal edema of upper airway or bowels. These symptoms may be disabling, have a dramatic impact on quality of life, and can be life-threatening when affecting the upper airways. Because the manifestations and severity of HAE are highly variable and unpredictable, patients need individualized care to reduce the burden of HAE on daily life. Although effective therapy for the treatment of HAE attacks has been available in many countries for more than 30 years, until recently, there were no agents approved in the United States to treat HAE acutely. Therefore, prophylactic therapy is an integral part of HAE treatment in the United States and for selected patients worldwide. Routine long-term prophylaxis with either attenuated androgens or C1-esterase inhibitor has been shown to reduce the frequency and severity of HAE attacks. Therapy with attenuated androgens, a mainstay of treatment in the past, has been marked by concern about potential adverse effects. C1-esterase inhibitor works directly on the complement and contact plasma cascades to reduce bradykinin release, which is the primary pathologic mechanism in HAE. Different approaches to long-term prophylactic therapy can be used to successfully manage HAE when tailored to meet the needs of the individual patient.

(Last update: 02.2021)

Number of publications (original work and reviews) in peer-reviewed journals: 601
Number of original publications in peer-reviewed journals: 432
Number of reviews in peer-reviewed journals: 169
Cumulative IF of publications (original work & reviews) in peer-reviewed journals: 3326,21
Cumulative IF for original publications in peer-reviewed journals: 2659,47
Cumulative IF for reviews in peer-reviewed journals: 666,74
Citations, Hirsch index: (view on Web of Science) 24104