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Hereditary angioedema with C1 inhibitor (C1-INH) deficit: the strength of recognition (51 cases) BJMBR
Fragnan,N.T.M.L.; Tolentino,A.L.N.; Borba,G.B.; Oliveira,A.C.; Simões,J.A.; Palma,S.M.U.; Constantino-Silva,R.N.; Grumach,A.S..
Hereditary angioedema (HAE) is a rare autosomal dominant disease due to C1 esterase inhibitor deficiency (C1-INH). The disease is characterized by subcutaneous and submucosal edema in the absence of urticaria due to the accumulation of bradykinin. This descriptive study aimed to evaluate the clinical characteristics of patients with a confirmed diagnosis of HAE referred to our Outpatient Clinic between December 2009 and November 2017. Fifty-one patients (38 F, 13 M) with a mean age of 32 years (range: 7–70 y) were included. Family history of HAE was reported in 70% (36/51) of the cases; 33/46 patients became symptomatic by 18 years of age. The median time between onset of symptoms and diagnosis was 13 years (3 mo–50 y). The most frequent triggering factors...
Tipo: Info:eu-repo/semantics/article Palavras-chave: Hereditary angioedema; C1 esterase inhibitor; Diagnosis; Treatment; Complement.
Ano: 2018 URL: http://www.scielo.br/scielo.php?script=sci_arttext&pid=S0100-879X2018001200607
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