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	Provedor de dados BJMBR (2) Autor Constantino-Silva,R.N. (2) Grumach,A.S. (2) Palma,S.M.U. (2) Borba,G.B. (1) Fragnan,N.T.M.L. (1) Oliveira,A.C. (1) Pimenta,F.M.C.A. (1) Simões,J.A. (1) Tolentino,A.L.N. (1) Mais... Palavra-chave Antibody defects (1) C1 esterase inhibitor (1) Chemotherapy (1) Common variable immunodeficiency (1) Complement (1) Diagnosis (1) Hereditary angioedema (1) Hypogammaglobulinemia (1) Immunoglobulin therapy (1) Primary immunodeficiency (1) Treatment (1) Mais... Tipo do documento Info:eu-repo/semantics/article (2) Ano 2019 (1) 2018 (1) País Brazil (2) Idioma Inglês (2)		Ordenar por:  Relevância Autor Título Ano Registros recuperados: 2 Primeira ... 1 ... Última Hypogammaglobulinemia: a diagnosis that must not be overlooked BJMBR Pimenta,F.M.C.A.; Palma,S.M.U.; Constantino-Silva,R.N.; Grumach,A.S.. Humoral immunological defects are frequent and important causes of hypogammaglobulinemia, leading to recurrent infections, autoimmunity, allergies, and neoplasias. Usually, its onset occurs in childhood or during the second and third decades of life; however, the diagnosis is made, on average, 6 to 7 years afterwards. As a consequence, antibody defects can lead to sequelae. Here we describe the clinical-laboratory characteristics, treatment, and prognoses of patients with hypogammaglobulinemia. An observational, cross-sectional, and retrospective study of patients attending the recently established outpatient group of Clinical Immunology between 2013 and 2018 was carried out. Patients with IgG levels below 2 standard deviations from the mean values for the... Tipo: Info:eu-repo/semantics/article Palavras-chave: Primary immunodeficiency; Common variable immunodeficiency; Hypogammaglobulinemia; Chemotherapy; Antibody defects; Immunoglobulin therapy. Ano: 2019 URL: http://www.scielo.br/scielo.php?script=sci_arttext&pid=S0100-879X2019001000608 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 Registros recuperados: 2 Primeira ... 1 ... Última

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