DEWTON DE MORAES VASCONCELOS
Projetos de Pesquisa
Unidades Organizacionais
Instituto Central, Hospital das Clínicas, Faculdade de Medicina - Médico
LIM/56 - Laboratório de Investigação em Dermatologia e Imunodeficiências, Hospital das Clínicas, Faculdade de Medicina
LIM/31 - Laboratório de Genética e Hematologia Molecular, Hospital das Clínicas, Faculdade de Medicina
LIM/56 - Laboratório de Investigação em Dermatologia e Imunodeficiências, Hospital das Clínicas, Faculdade de Medicina
LIM/31 - Laboratório de Genética e Hematologia Molecular, Hospital das Clínicas, Faculdade de Medicina
7 resultados
Resultados de Busca
Agora exibindo 1 - 7 de 7
- A brazilian nationwide multicenter study on deficiency of deaminase-2 (DADA2)(2023) MELO, Adriana; CARVALHO, Luciana Martins de; FERRIANI, Virginia Paes Leme; CAVALCANTI, Andre; APPENZELLER, Simone; OLIVEIRA, Valeria Rossato; NETO, Herberto Chong; ROSARIO, Nelson Augusto; POSWAR, Fabiano de Oliveira; GUIMARAES, Matheus Xavier; KOKRON, Cristina Maria; MAIA, Rayana Elias; SILVA, Guilherme Diogo; KELLER, Gabriel; FERREIRA, Mauricio Domingues; VASCONCELOS, Dewton Moraes; TOLEDO-BARROS, Myrthes Anna Maragna; BARROS, Samar Freschi; NETO, Nilton Salles Rosa; KRIEGER, Marta Helena; KALIL, Jorge; MENDONCA, Leonardo OliveiraIntroductionThe deficiency of ADA2 (DADA2) is a rare autoinflammatory disease provoked by mutations in the ADA2 gene inherited in a recessive fashion. Up to this moment there is no consensus for the treatment of DADA2 and anti-TNF is the therapy of choice for chronic management whereas bone marrow transplantation is considered for refractory or severe phenotypes. Data from Brazil is scarce and this multicentric study reports 18 patients with DADA2 from Brazil.Patients and methodsThis is a multicentric study proposed by the Center for Rare and Immunological Disorders of the Hospital 9 de Julho - DASA, Sao Paulo - Brazil. Patients of any age with a confirmed diagnosis of DADA2 were eligible for this project and data on clinical, laboratory, genetics and treatment were collected.ResultsEighteen patients from 10 different centers are reported here. All patients had disease onset at the pediatric age (median of 5 years) and most of them from the state of Sao Paulo. Vasculopathy with recurrent stroke was the most common phenotype but atypical phenotypes compatible with ALPS-like and Common Variable Immunodeficiency (CVID) was also found. All patients carried pathogenic mutations in the ADA2 gene. Acute management of vasculitis was not satisfactory with steroids in many patients and all those who used anti-TNF had favorable responses.ConclusionThe low number of patients diagnosed with DADA2 in Brazil reinforces the need for disease awareness for this condition. Moreover, the absence of guidelines for diagnosis and management is also necessary (t).
- II Brazilian Consensus on the use of human immunoglobulin in patients with primary immunodeficiencies(2017) GOUDOURIS, Ekaterini Simões; SILVA, Almerinda Maria do Rego; OURICURI, Aluce Loureiro; GRUMACH, Anete Sevciovic; CONDINO-NETO, Antonio; COSTA-CARVALHO, Beatriz Tavares; PRANDO, Carolina Cardoso de Mello; KOKRON, Cristina Maria; VASCONCELOS, Dewton de Moraes; TAVARES, Fabíola Scancetti; SEGUNDO, Gesmar Rodrigues Silva; BARRETO, Irma Cecília Douglas Paes; DORNA, Mayra de Barros; BARROS, Myrthes Anna Maragna Toledo; FORTE, Wilma Carvalho NevesABSTRACT In the last few years, new primary immunodeficiencies and genetic defects have been described. Recently, immunoglobulin products with improved compositions and for subcutaneous use have become available in Brazil. In order to guide physicians on the use of human immunoglobulin to treat primary immunodeficiencies, based on a narrative literature review and their professional experience, the members of the Primary Immunodeficiency Group of the Brazilian Society of Allergy and Immunology prepared an updated document of the 1st Brazilian Consensus, published in 2010. The document presents new knowledge about the indications and efficacy of immunoglobulin therapy in primary immunodeficiencies, relevant production-related aspects, mode of use (routes of administration, pharmacokinetics, doses and intervals), adverse events (major, prevention, treatment and reporting), patient monitoring, presentations available and how to have access to this therapeutic resource in Brazil.
conferenceObject The Most Frequent Primary Immunodeficiency Diseases (PIDDs) in Different Age Groups(2013) CARNEIRO-SAMPAIO, M.; JACOB, C. M. Abe; PASTORINO, A. C.; WATANABE, L.; DORNA, M.; DORIA-FILHO, U.; KOKRON, C. M.; TOLEDO-BARROS, M.; MORAES-VASCONCELOS, D.; DUARTE, A.- Comment to: II Brazilian Consensus on the use of human immunoglobulin in patients with primary immunodeficiencies. einstein (Sao Paulo). 2017; 15(1): 1-16(2017) GOUDOURIS, Ekaterini Simoes; SILVA, Almerinda Maria do Rego; OURICURI, Aluce Loureiro; GRUMACH, Anete Sevciovic; CONDINO-NETO, Antonio; COSTA-CARVALHO, Beatriz Tavares; PRANDO, Carolina Cardoso de Mello; KOKRON, Cristina Maria; VASCONCELOS, Dewton de Moraes; TAVARES, Fabiola Scancetti; SEGUNDO, Gesmar Rodrigues Silva; BARRETO, Irma Cecilia Douglas Paes; DORNA, Mayra de Barros; BARROS, Myrthes Anna Maragna Toledo; FORTE, Wilma Carvalho Neves
- Primary Immunodeficiency Diseases in Different Age Groups: A Report on 1,008 Cases from a Single Brazilian Reference Center(2013) CARNEIRO-SAMPAIO, Magda; MORAES-VASCONCELOS, Dewton; KOKRON, Cristina M.; JACOB, Cristina M. A.; TOLEDO-BARROS, Myrthes; DORNA, Mayra B.; WATANABE, Leticia A.; MARINHO, Ana Karolina B. B.; CASTRO, Ana Paula Moschione; PASTORINO, Antonio C.; SILVA, Clovis Artur A.; FERREIRA, Mauricio D.; RIZZO, Luiz V.; KALIL, Jorge E.; DUARTE, Alberto J. S.Primary immunodeficiencies (PIDs) represent a large group of diseases that affect all age groups. Although PIDs have been recognized as rare diseases, there is epidemiological evidence suggesting that their real prevalence has been underestimated. We performed an evaluation of a series of 1,008 infants, children, adolescents and adults with well-defined PIDs from a single Brazilian center, regarding age at diagnosis, gender and PID category according to the International Union of Immunological Societies classification. Antibody deficiencies were the most common category in the whole series (61 %) for all age groups, with the exception of <2-year-old patients (only 15 %). In the >30-year-old group, antibody deficiencies comprised 84 % of the diagnoses, mostly consisting of common variable immunodeficiency, IgA deficiency and IgM deficiency. Combined immunodeficiencies represented the most frequent category in <2-years-old patients. Most congenital defects of phagocytes were identified in patients <5 -years of age, as were the diseases of immune dysregulation, with the exception of APECED. DiGeorge syndrome and ataxia-telangiectasia were the most frequent entities in the category of well-defined syndromes, which were mostly identified in patients <10-years of age. Males represented three-quarters and two-thirds of <2 -years-old and 2-5-years -old patients, respectively, whereas females predominated among the >30-year-old patients. Our data indicated that some PIDs were only detected at early ages, likely because affected patients do not survive long. In addition, our data pointed out that different strategies should be used to search for PIDs in infants and young children as compared to older patients.
- II Brazilian Consensus on the use of human immunoglobulin in patients with primary immunodeficiencies (vol 15, pg 1, 2017)(2017) GOUDOURIS, Ekaterini Simoes; SILVA, Almerinda Maria do Rego; OURICURI, Aluce Loureiro; GRUMACH, Anete Sevciovic; CONDINO-NETO, Antonio; COSTA-CARVALHO, Beatriz Tavares; PRANDO, Carolina Cardoso de Mello; KOKRON, Cristina Maria; VASCONCELOS, Dewton de Moraes; TAVARES, Fabiola Scancetti; SEGUNDO, Gesmar Rodrigues Silva; BARRETO, Irma Cecelia Douglas Paes; SEGUNDO, Gesmar Rodrigues Silva; BARRETO, Irma Cecelia Douglas Paes; BARROS, Myrthes Anna Maragna Toledo; DORNA, Mayra de Barros; FORTE, Wilma Carvalho Neves
conferenceObject Organizing a Brazilian Network of Primary Immunodeficiency Reference Centers: ""CONSoRCIO Brasileiro De Centros De Referencia E Treinamento Em Imunodeficiencias Primarias"" - COBID(2014) CARNEIRO-SAMPAIO, Magda; OLIVEIRA, Joao Bosco; PINTO, J. A.; CUNHA, J. M.; VILELA, Maria Marluce dos Santos; ROXO JR., Persio; JACOB, Cristina Miuki Abe; KOKRON, C. M.; DEMORAES-VASCONCELOS, Dewton; DUARTE, Alberto Jose da Silva