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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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Best Practice & Research Clinical Gastroenterology, 2005
Hereditary and acquired deficiencies of the C1 inhibitor result in a single prominent symptom, namely angioedema. Angioedema may involve the skin, the gastrointestinal tract or the upper airway. Genetically determined defects in C1INH cause hereditary angioedema.
Fred S, Rosen, Alvin E, Davis
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Hereditary and acquired deficiencies of the C1 inhibitor result in a single prominent symptom, namely angioedema. Angioedema may involve the skin, the gastrointestinal tract or the upper airway. Genetically determined defects in C1INH cause hereditary angioedema.
Fred S, Rosen, Alvin E, Davis
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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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Monocyte C1‐inhibitor synthesis in patients with C1‐inhibitor deficiency
European Journal of Clinical Investigation, 1989Abstract. Monocytes of seven out of eight patients with type 1 C1‐inhibitor (C1‐inh) deficiency (HAE) produced 40% as much C1‐inh as monocytes from normal donors (controls). In contrast, monocytes from three patients with type 2 and three patients with acquired C1‐inh deficiency produced similar amounts of Cl‐inh as controls. Recombinant γ‐interferon (
D F, Lappin +6 more
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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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C1-inhibitor deficiency and angioedema
Molecular Immunology, 2001C1-inhibitor deficiency can be inherited or acquired; both conditions lead to recurrent angioedema that can be life threatening when the larynx is involved (hereditary angioedema, HAE; acquired angioedema, AAE). The genetic defect is due to the heterozygous deficiency of C1-Inh that is transmitted as an autosomal dominant trait.
A. Carugati +3 more
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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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2014
Autoantibodies to C1 inhibitor (C1-INH) bind to epitopes on the reactive center of the C1-INH molecule. As a consequence of this binding, C1-INH is converted into an inactive substrate that can be cleaved by proteases. C1-INH is a serine protease inhibitor that plays a role in the complement, contact, fibrinolytic, and coagulation cascades ...
Zanichelli, A +3 more
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Autoantibodies to C1 inhibitor (C1-INH) bind to epitopes on the reactive center of the C1-INH molecule. As a consequence of this binding, C1-INH is converted into an inactive substrate that can be cleaved by proteases. C1-INH is a serine protease inhibitor that plays a role in the complement, contact, fibrinolytic, and coagulation cascades ...
Zanichelli, A +3 more
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1993
Publisher Summary This chapter discusses C1 inhibitor (C1 INH). C1 INH is a member of the serine proteinase inhibitor (serpin) superfamily. C1 INH is the only physiologically important plasma inhibitor of activated Clr and Cls, and it provides a major portion of the plasma inhibitory capacity toward kallikrein and factor XIIa, but probably is not ...
Alvin E. Davis +4 more
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Publisher Summary This chapter discusses C1 inhibitor (C1 INH). C1 INH is a member of the serine proteinase inhibitor (serpin) superfamily. C1 INH is the only physiologically important plasma inhibitor of activated Clr and Cls, and it provides a major portion of the plasma inhibitory capacity toward kallikrein and factor XIIa, but probably is not ...
Alvin E. Davis +4 more
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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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