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).
Kamyar Zahedi +2 more
openaire +2 more sources
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 +3 more sources
Angioedema related to a deficiency in the C1-inhibitor protein is characterized by its lack of response to therapies including antihistamine, steroids, and epinephrine. In the case of laryngeal edema, mortality rate is approximately 30 percent. The first
Bassel Hallak +4 more
doaj +1 more source
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
Recombinant and Plasma-Purified Human C1 Inhibitor for the Treatment of Hereditary Angioedema
Background Agents for prophylaxis of hereditary angioedema (HAE) have been available in the United States for several decades, but their usefulness is limited by side effects and they cannot be used at all in some patients.
Michael M Frank, MD
doaj +1 more source
International consensus on the diagnosis and management of pediatric patients with hereditary angioedema with C1-Inhibitor deficiency [PDF]
BACKGROUND: The consensus documents published to date on hereditary angioedema with C1-inhibitor deficiency (C1-INH-HAE) have focused on adult patients. Many of the previous recommendations have not been adapted to pediatric patients.
Agostoni +122 more
core +3 more sources
Characterization of carbohydrate chains of C1‐inhibitor and of desialylated C1‐inhibitor
Carbohydrate chains of C1‐inhibitor were identified with a binding assay using different lectins. Lectins from Sambucus nigra (SNA) and Maackia amurensis (MAA) that are specific for sialic acids bound to C1‐inhibitor. Lectin from Datura strantonium (DSA) reacted also with the inhibitor indicating complex and hybrid sugar structures.
openaire +3 more sources
Reciprocal control of viral infection and phosphoinositide dynamics
Phosphoinositides, although scarce, regulate key cellular processes, including membrane dynamics and signaling. Viruses exploit these lipids to support their entry, replication, assembly, and egress. The central role of phosphoinositides in infection highlights phosphoinositide metabolism as a promising antiviral target.
Marie Déborah Bancilhon, Bruno Mesmin
wiley +1 more source
Possible role of extracellularly released phagocytic proteinases in the coagulation disorder during liver transplantation [PDF]
Orthotopic liver transplantation is frequently associated with a complex coagulation disorder, influencing the outcome of the procedure. In this respect, disseminated intravascular coagulation (DIC) had been suggested to be of causative importance for ...
Blumhardt, G. +6 more
core
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

