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Angioedema due to C1 inhibitor deficiency in 2010

Internal and Emergency Medicine, 2010
Angioedema is a recurrent, non-pitting, non-pruritic, self-limiting swelling due to transient increase of endothelial permeability in the capillaries of the deep cutaneous and mucosal layers. Two main groups of angioedema should be distinguished based on the response to treatment: those responding to antihistamine and those that do not.
M. Cicardi, A. Zanichelli
openaire   +2 more sources

First case of homozygous C1 inhibitor deficiency

Journal of Allergy and Clinical Immunology, 2006
C1 Inhibitor (C1-Inh) deficiency causes angioedema and can be hereditary (HAE), caused by mutations in the C1-Inh gene (C1NH), or acquired (AAE). Patients with HAE show a complement profile different from that of patients with AAE with normal levels of C1 (C1q, C1r, and C1s).We sought to characterize the complement profile of a patient with HAE and a ...
Blanch, Alvaro   +5 more
openaire   +3 more sources

Acquired C1 Inhibitor Deficiency: Postmortem Diagnosis

Dermatologica, 2009
Acquired C1 esterase inhibitor deficiency is a clinical syndrome closely resembling hereditary angioedema in which most patients have an associated malignancy of B cell lineage. Sera from 33 patients with B cell neoplasms were assayed for C1 esterase inhibitor level by rocket immunoelectrophoresis.
D P, Fivenson, R O, Dillman, I, Gigli
openaire   +2 more sources

Epitope mapping of C1 inhibitor autoantibodies from patients with acquired C1 inhibitor deficiency

The Journal of Immunology, 1996
Abstract We report six patients with acquired C1 inhibitor (C1-inh) deficiency associated with serum C1-inh autoantibodies and circulating cleaved (96 kDa), functionally inactive C1-inh. In three patients, all of whom had IgG-kappa paraproteins in their sera, the Abs were IgG-kappa. In the remaining three patients, the Abs were IgM (2
S, He   +5 more
openaire   +2 more sources

Angioedema and C1 inhibitor deficiency.

Annals of allergy, 1992
Deficiency of C1 inhibitor resulting in episodes of angioedema causes significant morbidity and mortality in affected patients, yet often goes undiagnosed for years. As biochemical understanding of the disorder has improved, competing theories for the pathophysiologic mechanism of angioedema have emerged.
N A, Orfan, G B, Kolski
openaire   +1 more source

C1 inhibitor deficiency

Clinical and Experimental Dermatology, 2005
M, Darling, M L, Price
openaire   +2 more sources

Cardiac Surgery and C1-Inhibitor Deficiency

Journal of Cardiothoracic and Vascular Anesthesia, 2014
Chamaraux-Tran, Thiên-Nga   +5 more
openaire   +3 more sources

The C1 inhibitor deficiency. A review.

European journal of clinical chemistry and clinical biochemistry : journal of the Forum of European Clinical Chemistry Societies, 1993
C1 inhibitor (C1I), a member of the serine protease inhibitor superfamily, is the principal regulator of the activation classical pathway of complement by reducing the proteolytic activity of activated C1r and C1s. A deficiency of active C1 inhibitor is the most commonly identified genetic defect of the complement system.
openaire   +1 more source

Inherited C1 inhibitor deficiency.

Complement and inflammation, 1989
The paper reports our experience of a 15-year follow-up of 179 patients with hereditary angioedema (HAE). The disease is transmitted as an autosomal dominant trait and two variants have been described: type I characterized by functional and antigenic C1-inhibitor (C1-INH) deficiency and type II with normal or upper normal C1-INH antigenic levels but no
openaire   +1 more source

Angioedema Due to Acquired Deficiency of C1-Inhibitor: A Cohort Study in Spain and a Comparison With Other Series

Journal of Allergy and Clinical Immunology: in Practice, 2022
Mar Guilarte   +2 more
exaly  

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