CRISTINA MIUKI ABE JACOB
Projetos de Pesquisa
Unidades Organizacionais
LIM/36 - Laboratório de Pediatria Clínica, Hospital das Clínicas, Faculdade de Medicina - Líder
5 resultados
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conferenceObject Novel Mutations in MVK Associated with Hyperimmunoglobulinemia D with Periodic Fever Syndrome Phenotype(2014) VASCONCELOS, D. Moraes; FUJIHIRA, E.; OLIVEIRA, J. B.; JESUS, A. A.; SILVA, C.; CASTRO, A. P. M.; DORNA, M. B.; WATANABE, L.; PONTILLO, A.; CHUFFI-BARROS, N.; JACOB, C. M. A.; CARNEIRO-SAMPAIO, M. M. S.; DUARTE, A. J.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.- 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.
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- Impaired CD8(+) T cell responses upon Toll-like receptor activation in common variable immunodeficiency(2016) LOLLO, Camila de; VASCONCELOS, Dewton de Moraes; OLIVEIRA, Luanda Mara da Silva; TITZ, Tiago de Oliveira; CARNEIRO-SAMPAIO, Magda; JACOB, Cristina Miuki Abe; DUARTE, Alberto Jose da Silva; SATO, Maria NotomiBackground: Infections caused by bacteria or viruses are frequent in common variable immunodeficiency (CVID) patients due to antibody deficiencies, which may be associated with altered T cell function. CVID patients are frequently in contact with pathogen-associated molecular patterns (PAMPs), leading to the activation of innate immunity through Toll-like receptors (TLR) affecting T cell activation. We evaluated the effect of TLR activation on T cells in CVID patients undergoing intravenous immunoglobulin (IVIg) replacement using synthetic ligands. Methods: Expression of exhaustion, activation and maturation markers on T cells from peripheral blood as well as regulatory T cells and follicular T cells in peripheral blood mononuclear cells (PBMCs) from CVID and healthy individuals were evaluated by flow cytometry. PBMCs cultured with TLR agonists were assessed for intracellular IFN-gamma, TNF, IL-10, IL-17a or IL-22 secretion as monofunctional or polyfunctional T cells (simultaneous cytokine secretion) by flow cytometry. Results: We found increased expression of the exhaustion marker PD-1 on effector memory CD4(+) T cells (CD45RA(-)CCR7(-)) in the peripheral blood and increased expression of CD38 in terminally differentiated CD8(+) T cells (CD45RA(+)CCR7(-)). Furthermore, a decreased frequency of naive regulatory T cells (CD45RA(+)Foxp3(low)), but not of activated regulatory T cells (CD45RA(-)Foxp3(high)) was detected in CVID patients with splenomegaly, the noninfectious manifestation in this CVID cohort (43.7 %). Moreover, the frequency of peripheral blood follicular helper T cells (CD3(+)CD4(+)CXCR5(+)PD-1(+)ICOS(+)) was similar between the CVID and control groups. Upon in vitro TLR3 activation, a decreased frequency of CD8(+) T cells secreting IFN-gamma, IL-17a or IL-22 was detected in the CVID group compared to the control group. However, a TLR7/TLR8 agonist and staphylococcal enterotoxin B induced an increased Th22/Tc22 (IL-22(+), IFN-gamma(-), IL-17a(-)) response in CVID patients. Both TLR2 and TLR7/8/CL097 activation induced an increased response of CD4(+) T cells secreting three cytokines (IL-17a, IL-22 and TNF) in CVID patients, whereas CD8(+) T cells were unresponsive to these stimuli. Conclusion: The data show that despite the unresponsive profile of CD8(+) T cells to TLR activation, CD4(+) T cells and Tc22/Th22 cells are responsive, suggesting that activation of innate immunity by TLRs could be a strategy to stimulate CD4(+) T cells in CVID.