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| Content Provider | Springer Nature Link |
|---|---|
| Author | Czúcz, Judit Schaffer, Gyula Csuka, Dorottya Walentin, Szilvia Kunde, Jan Prohászka, Zoltán Farkas, Henriette Cervenak, László |
| Copyright Year | 2011 |
| Abstract | The bradykinin pathway in the pathomechanism of hereditary angioedema due to C1-inhibitor deficiency (henceforward “hereditary angioedema”) has been thoroughly studied; however, much less is known about endothelial cell function. Enhanced endothelial cell permeability is obvious during edematous attacks, but not during inter-attack periods. Our knowledge about other endothelial characteristics is even more incomplete.Therefore the aim of this study was to characterize endothelial cell function in hereditary angioedema patients during symptom-free, inter-attack periods.We measured the serum levels of soluble E-selectin, endothelin-1, and von Willebrand factor along with collagen-binding activity in 49 hereditary angioedema patients and in 50 healthy controls.Endothelin-1 and von Willebrand factor level, as well as its collagen-binding activity, were similar in hereditary angioedema patients and in controls; however, we found elevated soluble E-selectin levels in the patients. Interestingly, soluble E-selectin concentration did not correlate with any of the inflammatory markers or smoking, and it is not the consequence of the known E-selectin/C1-inhibitor interaction (an analytical phenomenon). In a multiple logistic regression model, the difference in soluble E-selectin between hereditary angioedema patients and controls remained highly significant when adjusted for age, gender, smoking, C-reactive protein, and AB0 blood groups.These results demonstrate that in hereditary angioedema, the majority of endothelial functions are normal during inter-attack periods; however, soluble E-selectin levels are elevated. The higher soluble E-selectin plasma concentration is unlikely to result from inflammation; rather, it reflects enhanced shedding mechanisms. |
| Starting Page | 61 |
| Ending Page | 69 |
| Page Count | 9 |
| File Format | |
| ISSN | 02719142 |
| Journal | Journal of Clinical Immunology |
| Volume Number | 32 |
| Issue Number | 1 |
| e-ISSN | 15732592 |
| Language | English |
| Publisher | Springer US |
| Publisher Date | 2011-10-19 |
| Publisher Place | Boston |
| Access Restriction | One Nation One Subscription (ONOS) |
| Subject Keyword | Hereditary angioedema sE-selectin C1-inhibitor endothelial function Medical Microbiology Internal Medicine Infectious Diseases Immunology |
| Content Type | Text |
| Resource Type | Article |
| Subject | Immunology and Allergy Immunology |
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