Practical Management of C1 Inhibitor Deficiency
نویسندگان
چکیده
منابع مشابه
[Practical management of C1 inhibitor deficiency].
C1 inhibitor deficiency is a rare syndrome clinically characterized by recurrent episodes of swelling of subcutaneous tissue or angioedema. It can involve the skin, upper respiratory airways and abdomen. There are two main types: hereditary and acquired. Angioedema can involve practically any part of the cutaneous surface, it can cause lethal laryngeal edema and can present as gastrointestinal ...
متن کاملCLINICAL REVIEW Acquired C1 Esterase Inhibitor Deficiency
Acquired C1 esterase inhibitor deficiency is a rare condition associated with autoimmune or low-grade lymphoproliferative disorders. Adults or elderly patients are most commonly affected. The diagnosis is suspected when patients present with recurrent angioedema and low serum levels of C4 with normal levels of C3. Low levels of C1q and low C1 esterase inhibitor activity confirm the diagnosis. I...
متن کاملLymphoproliferative disease and acquired C1 inhibitor deficiency.
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 gammopathies of unknown significance and 9 have non-Hodgkin's lymphoma. Our series suggest that different forms of B cell disorders coexist and/or ...
متن کاملangioedema in a patient with c1 esterase inhibitor deficiency
angioedema is characterized by recurrent, circumscribed, solitary or multiple subcutaneous and mucosal swelling, involving the extremities, face, larynx, bowel wall. angioedema is due to hereditary or acquired varieties of c1 esterase inhibitor (c1inh) deficiency. a case of atypical acquired angioedema in a 49-year old man, responding favourably to cinnarizine and alcohol abstinence, is present...
متن کاملHereditary angioedema without deficiency of C1 inhibitor: response to therapy
Results Nineteen patients have been identified (2M:17F; 20-60 years old). The following therapies were oriented: combined contraceptive substitution for progestagen (10/19); treatment with progestagen (2/19); tranexamic acid (15/19): 1250mg (2), 1000mg (1), 750 (5), 500 mg (4), 250 mg (1); oxandrolon (5/19) (0.5 mg-5mg/day), danazol 200mg/day (1/19) and combined therapy woth oxandrolon and tran...
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ژورنال
عنوان ژورنال: Actas Dermo-Sifiliográficas (English Edition)
سال: 2007
ISSN: 1578-2190
DOI: 10.1016/s1578-2190(07)70437-7