English (United Kingdom)

https://curated-unify.zendy.io/wp-json/zendy-region/v1/featured_content/oa?rat=en

https://curated-unify.zendy.io/wp-json/zendy-region/v1/highlighted_journal/

Zendy Plus

Presents the access of premium content as premium feature

Premium Content

Presents the keyphrase highlighting as premium feature

Keyphrase Highlighting

Presents the summarisation as premium feature

Summarisation

Insights

Presents the pdf analysis as premium feature

PDF Analysis

Presents the zaia usage as premium feature

ZAIA

Zendy Tools

Zendy Open

Background  Recommended management of attacks of hereditary angioedema ( HAE ) due to  C 1 esterase inhibitor ( C 1‐ INH ) deficiency (C1‐ INH ‐ HAE ) includes therapy with exogenous  C 1 INH . Thrombotic/thromboembolic events ( TEE ) have been reported with plasma‐derived  C 1 INH , but so far none with recombinant human C1 INH  (rhC1 INH ). This phase  III , randomized, placebo (saline)‐controlled study evaluated the safety of rhC1 INH  50  IU /kg for the treatment of acute attacks in 74 patients with C1‐ INH ‐ HAE .    Methods  Monitoring for  TEE  and assessment of risk of deep vein thrombosis ( DVT ) by the Wells prediction rule were performed, and levels of fibrin degradation products (plasma  D ‐dimers) were assessed before study drug administration (baseline), 2 h, and 7 days posttreatment.    Results  Plasma D‐dimer levels were elevated in 80% of the patients (median [25th–75th percentiles]: 2149 [480–5105] μg/l; normal ≤250 μg/l) and were higher in patients with submucosal (abdominal, oropharyngeal–laryngeal) attacks (3095 [890–10000] μg/l;  n  = 29) compared with subcutaneous (peripheral, facial) attacks (960 [450–4060] μg/l;  n  = 35). Median plasma D‐dimer levels were comparable across treatment groups at baseline (1874 [475–4568] μg/l rhC1 INH ; 2259 [586–7533] μg/l saline) and 2 h postinfusion (2389 [760–4974] μg/l rhC1 INH ; 2550 [310–8410] μg/l saline); median plasma D‐dimer levels were decreased by Day 7 in both groups (425 [232–3240] μg/l rhC1 INH ; 418 [246–2318] μg/l saline). No increased risk of  DVT  was identified, nor any  TEE  reported in rhC1 INH  treated or controls.    Conclusion  Elevated plasma D‐dimer levels were associated with acute C1‐ INH ‐ HAE  attacks, particularly with submucosal involvement. However, rhC1 INH  therapy was not associated with thrombotic events.

Elevated D ‐dimers in attacks of hereditary angioedema are not associated with increased thrombotic risk