Expression of platelet C1 inhibitor [PDF]
Abstract Human platelets contain a pool of C1 inhibitor (C1 INH) distinct from that in plasma. Twelve normal platelet samples washed by centrifugation had a mean platelet C1 INH antigen level of 19.3 +/- 2.8 ng (mean +/- SEM) per 10(8) platelets. These values contrast with the mean +/- SEM platelet C1 INH antigen level of 6.1 +/- 0.9 per
A H, Schmaier +4 more
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Plasma complement biomarkers distinguish multiple sclerosis and neuromyelitis optica spectrum disorder [PDF]
Background: Multiple sclerosis (MS) and neuromyelitis optica spectrum disorders (NMOSD) are autoimmune demyelinating diseases distinguished clinically by selective involvement in NMOSD of optic nerves and spinal cord. Early clinical manifestations are
Evans, David R. S. +6 more
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Biological activities of C1 inhibitor [PDF]
Broadly speaking, C1 inhibitor plays important roles in the regulation of vascular permeability and in the suppression of inflammation. Vascular permeability control is exerted largely through inhibition of two of the proteases involved in the generation of bradykinin, factor XIIa and plasma kallikrein (the plasma kallikrein-kinin system).
Alvin E, Davis +2 more
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Amniotic fluid embolism pathophysiology suggests the new diagnostic armamentarium: β-tryptase and complement fractions C3-C4 are the indispensable working tools [PDF]
Amniotic fluid embolism (AFE) is an uncommon obstetric condition involving pregnant women during labor or in the initial stages after delivery. Its incidence is estimated to be around 5.5 cases per 100,000 deliveries.
Busardo', FRANCESCO PAOLO +3 more
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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
Mechanisms of C1-Inhibitor Deficiency
C1 inhibitor (C1-Inh) is a protease inhibitor of the serpin family. It interacts and forms complexes with several serine proteases although not all these interactions were proved to be relevant in vivo. Based on studies in deficient patients, C1-Inh appears pivotal in regulating the activation of complement classical pathway and of contact system.
E. Pappalardo +5 more
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Antimicrobial peptides and complement in neonatal hypoxia-ischemia induced brain damage [PDF]
Hypoxic-ischemic encephalopathy (HIE) is a clinical condition in the neonate, resulting from oxygen deprivation around the time of birth. HIE affects 1-5/1000 live births worldwide and is associated with the development of neurological deficits ...
Hristova, M, Rocha-Ferreira, E
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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
The international WAO/EAACI guideline for the management of hereditary angioedema – the 2017 revision and update [PDF]
Hereditary Angioedema (HAE) is a rare and disabling disease. Early diagnosis and appropriate therapy are essential. This update and revision of the global guideline for HAE provides up-to-date consensus recommendations for the management of HAE.
Aabom +238 more
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The influence of trigger factors on hereditary angioedema due to C1-inhibitor deficiency [PDF]
Background: Hereditary angioedema (HAE) resulting from C1-inhibitor deficiency is characterized by attacks of subcutaneous and submucosal edema. Many factors have been presumed to induce edema.
Csuka, Dorottya +8 more
core +1 more source

