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Angioedema associated with C1 inhibitor deficiency
Clinical Reviews in Allergy & Immunology, 1999Angioedema associated with C1 INH deficiency requires diagnosis as soon as the first symptoms of the disease are manifest, and laboratory measurement of complement component levels at the least suspicion, in order to institute preventive and curative treatments.
J, Laurent, M T, Guinnepain
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C1 inhibitor deficiency simulating systemic lupus erythematosus
British Journal of Dermatology, 1982A 48-year-old Japanese woman with systemic lupus erythematosus-like lesions of the skin and lips was found to have hereditary angio-oedema. Complement studies revealed low CH50, C1q, C4 and C1 inhibitor levels, with normal C3 and C5 levels. Dramatic clinical improvement followed fresh normal human blood transfusion and systemic betamethasone ...
S, Shiraishi +4 more
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Lupus Erythematosus Associated with C1 Inhibitor Deficiency
The Journal of Dermatology, 2002AbstractWe report here a patient with skin lesions of lupus erythematosus (LE) associated with a type 1 hereditary C1 inhibitor deficiency. She had not experienced any episodes of angioedema. A histological examination of the affected skin lesions demonstrated liquefaction of the basal cell layer in the perifollicule. Direct immunofluorescence staining
Masayo, Koide +5 more
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First case of homozygous C1 inhibitor deficiency
Journal of Allergy and Clinical Immunology, 2006C1 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
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Autoimmune C1 inhibitor deficiency: Report of eight patients
The American Journal of Medicine, 1993In this study, we investigated the clinical and biochemical features and the responses to treatment of eight patients with auto-antibody-mediated C1 inhibitor (C1-INH) deficiency and symptoms of angioedema.In addition to the 8 patients with acquired angioedema (AAE), we also studied 36 subjects with hereditary angioedema (HAE), 15 of them treated with ...
M. Cicardi +4 more
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Inherited C1 inhibitor deficiency.
Complement and inflammation, 1989The 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
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Angioedema and C1 inhibitor deficiency.
Annals of allergy, 1992Deficiency 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
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C1 esterase inhibitor deficiency: three presentations.
Annals of allergy, 1991We have presented three patients representing three of the four reported forms of C1 esterase inhibitor deficiency. The C1INH deficiencies are in general distinguishable from each other and from other forms of angioedema by their complement patterns. The acquired C1INH deficiency must be part of the differential in patients without a family history of ...
J, Baldwin +3 more
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Cardiac Surgery and C1-Inhibitor Deficiency
Journal of Cardiothoracic and Vascular Anesthesia, 2014Thiên-Nga, Chamaraux-Tran +5 more
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