C1-inhibitor/C1-inhibitor antibody complexes in acquired angioedema due to C1-inhibitor deficiency [PDF]
Orphanet Journal of Rare Diseases, 2023 Background Autoantibodies against C1-inhibitor (C1-INH-Ab) have a diagnostic value in acquired angioedema due to C1-inhibitor deficiency (C1-INH-AAE), even though antibodies can circulate in complexes, which can be undetectable by proven methods. Our aim
Zsofia Polai +6 more
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Angioedema due to acquired C1-inhibitor deficiency: spectrum and treatment with C1-inhibitor concentrate [PDF]
Orphanet Journal of Rare Diseases, 2019 Background Acquired angioedema due to C1-inhibitor (C1-INH) deficiency (AAE-C1-INH) is a serious condition that may result in life-threatening asphyxiation due to laryngeal edema. It is associated with malignant B-cell lymphoma and other disorders.
Konrad Bork +2 more
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Characterization of carbohydrate chains of C1‐inhibitor and of desialylated C1‐inhibitor
FEBS Letters, 1992 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.
Oeyvind L. Schoenberger +1 more
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Treatment of hereditary angioedema with plasma-derived C1 inhibitor
Therapeutics and Clinical Risk Management, 2008 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
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Mechanisms of C1-Inhibitor Deficiency
Immunobiology, 2002 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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Edema of the Scrotum and Penile Shaft: An Uncommon Initial Presentation of Acquired Angioedema With Low C1-Inhibitor [PDF]
Case Reports in UrologyAcquired angioedema with low C1-inhibitor (AAE-C1-INH) is a rare disorder characterized by an acquired deficiency in the C1 esterase inhibitor (C1-INH). This case report describes a 79-year-old patient presenting to the emergency department for painless ...
Meghan V. Matheny +2 more
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Lymphoproliferative disease and acquired C1 inhibitor deficiency
Haematologica, 2007 Angioedema due to acquired deficiency of the C1-inhibitor is a bridging condition between autoimmunity and lymphoproliferation. We report 32 patients with acquired C1 inhibitor deficiency: 23 have anti C1-inhibitor autoantibodies; 13 have monoclonal ...
Roberto Castelli +4 more
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Study on separation and purification process of C1 esterase inhibitor
Zhongguo shuxue zazhi, 2022 Objective To study the technology of separating and purifying C1 esterase inhibitor (C1-INH) by using the waste washing liquid as raw materia during the preparation of human prothrombin complex (PCC) l.
Jiabin XU +4 more
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C1-inhibitor to prevent intracerebral hemorrhage-related secondary brain injury [PDF]
Fluids and Barriers of the CNSBackground Preclinical studies indicate that the systemic application of C1-inhibitor, clinically used to treat hereditary angioedema, reduces secondary brain injury after ischemic stroke. This study assessed the effect of C1-inhibitor on secondary brain
Kevin Akeret +9 more
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Editorial: C1 inhibitor deficiency and angioedema [PDF]
Frontiers in Allergy, 2022 Henriette Farkas +2 more
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