Presence of C1-inhibitor polymers in a subset of patients suffering from hereditary angioedema. [PDF]
Hereditary angioedema (HAE) is a potentially life-threatening disease caused by mutations in the gene encoding the serine protease inhibitor (serpin) C1 inhibitor (C1-inh).
Daniel Elenius Madsen +5 more
doaj +1 more source
Fulminant systemic capillary leak syndrome due to C1 inhibitor deficiency complicating acute dermatomyositis: a case report [PDF]
INTRODUCTION: Dermatomyositis is a chronic inflammatory disorder characterized by muscular and dermatologic symptoms with variable internal organ involvement.
Andreas Brunauer +5 more
core +1 more source
Treatment of hereditary angioedema with plasma-derived C1 inhibitor
Michael J Prematta, Tracy Prematta, Timothy J CraigSection of Allergy and Immunology, Penn State University, Milton S. Hershey Medical Center, PA, USABackground: Plasma-derived C1 inhibitor (C1-INH) concentrate is a treatment option for acute hereditary ...
Michael J Prematta +2 more
doaj
Complement, contact activation, coagulation, and fibrinolysis are serum protein cascades that need strict regulation to maintain human health. Serum glycoprotein, a C1 inhibitor (C1-INH), is a key regulator (inhibitor) of serine proteases of all the ...
Arun Dhillon +6 more
doaj +1 more source
Enzimreakciók vizsgálata a moduláris szerveződés, az atomi kölcsönhatás és a kvantummechanika szintjein. A fehérje biofizika tudományos iskolája = Insight into the Enzyme Action at Levels of modular Organization, Atomic Interactions and Quantum-Mechanics. School of Protein Biophysics [PDF]
Az elmúlt 3 év koherens kutató munkája során születtek speciális tudományos eredmények és levontunk ezekből általános következtetéseket is. Munkánk mérlege a nemzetközi folyóiratokban megjelent 30 közlemény összesen 130 IF-al. Molekuláris immunológiai
Barna, László +18 more
core
On demand treatment and home therapy of hereditary angioedema in Germany - the Frankfurt experience [PDF]
Background: Manifestation of acute edema in hereditary angioedema (HAE) is characterized by interindividual and intraindividual variability in symptom expression over time. Flexible therapy options are needed.
Aygören-Pürsün, Emel +4 more
core +2 more sources
Regulation of C1 Inhibitor Synthesis
The primary biologic roles of C1 inhibitor (C1-INH) are the regulation of activation of the classical complement pathway and of the contact system of kinin formation. Heterozygosity for deficiency or dysfunction of C1-INH results in hereditary angioedema (HAE).
A E, Prada, K, Zahedi, A E, Davis
openaire +2 more sources
Classification, diagnosis, and approach to treatment for angioedema: Consensus report from the Hereditary Angioedema International Working Group [PDF]
Angioedema is defined as localized and self-limiting edema of the subcutaneous and submucosal tissue, due to a temporary increase in vascular permeability caused by the release of vasoactive mediator(s). When angioedema recurs without significant wheals,
Adam +151 more
core +1 more source
C1-Inhibitor and Transplantation
Excessive activation of the protein cascade systems has been associated with post-transplantation inflammatory disorders. There is increasing evidence that complement not only significantly contributes to ischemia/reperfusion injury upon cold storage of the organ but also, although to a different degree, to allograft rejection. Complement activation is
openaire +2 more sources
A danazolkezelés hatása C1-inhibitor-hiány okozta hereditaer angiooedemás gyermekek növekedésére [PDF]
Absztrakt: Bevezetés: Az attenuált androgéneket gyakran alkalmazzák C1-inhibitor-hiányos hereditaer angiooedema akut epizódjainak megelőzésére. Praepubertason túli alkalmazásuk az epifízisfugák
Farkas, Henriette +4 more
core +2 more sources

