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C1 inhibitor deficiency: management
Clinical and Experimental Dermatology, 2005This is the second of two articles on C1 inhibitor deficiency based on a recent UK consensus document covering its diagnosis and management in adults and children. This summary focuses on the management of the disorder including prophylaxis, emergency treatment and special situations such as pregnancy and dental care.
M M, Gompels, R J, Lock
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C1-inhibitor autoantibodies in SLE
Lupus, 2010The presence of anti-C1-inhibitor (anti-C1-INH) autoantibodies is a hallmark of acquired C1-inhibitor deficiency. However, only scarce data are available on their prevalence, diagnostic value, and/or significance in systemic lupus erythematosus (SLE).
T, Mészáros +14 more
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Structure and Function of C1-Inhibitor
Immunology and Allergy Clinics of North America, 2006C1-INH belongs to the family of serpins. Structural studies have yielded a clear understanding of the biochemical principle underlying the functional activities of these proteins. Although the crystal structure of C1-INH has yet to be revealed, homology modeling has provided a three-dimensional model of the serpin part of C1-INH.
Ineke G A, Wagenaar-Bos, C Erik, Hack
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C1 esterase inhibitor in pregnancy
Journal of Allergy and Clinical Immunology, 1992This patient had multiple diagnoses, including lymphadenopathic mastocytosis with involvement of the spleen, liver, abdominal lymph nodes, skeleton, bone marrow, skin, and gastrointestinal tract. He was also diagnosed with acute nonlymphocytic leukemia predated by the discovery of a biclonal gammopathy.
A J, Cohen, C, Laskin, S, Tarlo
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Acquired C1 Inhibitor Deficiency
Immunology and Allergy Clinics of North America, 2017Acquired angioedema due to C1-INH deficiency (C1-INH-AAE) can occur when there are acquired (not inherited) deficiencies of C1-INH. A quantitative or functional C1-INH deficiency with negative family history and low C1q is diagnostic of C1-INH-AAE. The most common conditions associated with C1-INH-AAE are autoimmunity and B-cell lymphoproliferative ...
Iris M, Otani, Aleena, Banerji
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Acute consumption of C1 inhibitor in a patient with acquired C1-inhibitor deficiency syndrome
Journal of Allergy and Clinical Immunology, 1991Acquired C1-inhibitor (C1 INH) deficiency is usually found in association with an underlying disease that is believed to be responsible for increased C1 INH catabolism, ultimately leading to the development of C1 INH deficiency. We report a remarkable patient with acquired C1 INH deficiency in whom a unique progression of complement- and contact-system
B L, Zuraw, L C, Altman
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Biological effects of C1 inhibitor
Drug News & Perspectives, 2004C1 inhibitor is a serine proteinase inhibitor (serpin) that regulates activation of both the complement and contact systems. Regulation of complement system activation takes place through inactivation of the classical pathway proteases, C1r and C1s, the lectin pathway protease, MASP2, and perhaps via inhibition of alternative pathway activation by ...
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Nihon rinsho. Japanese journal of clinical medicine, 2000
Ranol Zahedi, Alvin E. Davis
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Ranol Zahedi, Alvin E. Davis
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Interaction of C1-inhibitor with the C1r and C1s subcomponents in human C1.
Biochimica et biophysica acta, 19791. Insoluble IgG-ovalbumin aggregates were used to bind and activate C1 from human serum. The bound C1 provided a useful reagent for studying the interaction of C1 subcomponents with C1-inhibitor. 2. C1-inhibitor bound to both subcomponents (C1r and C1s in C1 and formed stable complexes of respective apparent molecular weights 197,000 and 185,000, as ...
G J, Arlaud +3 more
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