DEWTON DE MORAES VASCONCELOS

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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

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Agora exibindo 1 - 10 de 79
  • 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
    Primary Immunodeficiency Disorders (PID) in a Specialized Dermatology Outpatient Unit in Sao Paulo, Brazil
    (2014) VASCONCELOS, D. Moraes; DOMINGUES-FERREIRA, M.; CHUFFI-BARROS, N.; BEZERRA, T. A.; BERTOLINI, D. L.; MUNIZ JUNIOR, R.; PRESTES-CARNEIRO, L. E.; DUARTE, A. J.
  • conferenceObject
    Ombination Efficacy of Voriconazole and Amphotericin B in the Experimental Disease In Immunodeficient Mice Caused by Fluconazole-Resistant Cryptococcus Neoformans
    (2012) SILVA, E. G.; SOARES, Maria Cecilia Pereira; MORAES-VASCONCELOS, Dewton de; DIAS, A. L. T.; CHANG, M. R.; RUIZ, L. S.; GAMBALE, Valderez; PRATES, R. A.; RIBEIRO, M. S.; PAULA, Claudete Rodrigues de
    The model of systemic cryptococcosis in BALB/c SCID mice is useful for immunological and therapeutic study of the disease in immunodeficient hosts. They are susceptible to experimental cryptococcosis by C. neoformans var. grubii and useful to evaluate treatment. All the treatments pro-longed the survival compared to the control groups (P < 0.05). Amphotericin B significantly prolonged the survival of the mice compared to all other treatments. The results obtained in this study, based on a significant increase in survival and significant reduction in the burden of yeasts in the lung and brain tissues, found in the groups treated with amphotericin B combined with voriconazole versus the groups treated only with amphotericin B or voriconazole, suggest that this therapy—using amphotericin B (1.5 mg/kg/ day) in association with voriconazole (40.0 mg/kg/day) —could be a promising alternative for the treatment of cryptococcosis, especially when fluconazole proves to be unsatisfactory.
  • article 8 Citação(ões) na Scopus
    Lomentospora prolificans fungemia in hematopoietic stem cell transplant patients: First report in South America and literature review
    (2018) PENTEADO, Fernando D.; LITVINOV, Nadia; SZTAJNBOK, Jaques; THOMAZ, Danilo Y.; SANTOS, Antonio M. dos; VASCONCELOS, Dewton M.; MOTTA, Adriana L.; ROSSI, Flavia; FERNANDES, Juliana F.; MARQUES, Heloisa Helena S.; BENARD, Gil; ALMEIDA JR., Joao N. de
    Lomentospora prolificans is a filamentous fungus and an emerging pathogen in immunocompromised patients. It is encountered most commonly in Australia, Spain, and USA. We described the first case of Lomentospora prolificans fungemia in South America. The patient was a hematopoietic stem cell transplantation (HSCT) recipient who developed the infection 37days after stem cells infusion. In addition, we performed a literature review of invasive lomentosporiosis in HSCT patients.
  • article 91 Citação(ões) na Scopus
    Disruption of an antimycobacterial circuit between dendritic and helper T cells in human SPPL2a deficiency
    (2018) KONG, Xiao-Fei; MARTINEZ-BARRICARTE, Ruben; KENNEDY, James; MELE, Federico; LAZAROV, Tomi; DEENICK, Elissa K.; MA, Cindy S.; BRETON, Gaelle; LUCERO, Kimberly B.; LANGLAIS, David; BOUSFIHA, Aziz; AYTEKIN, Caner; MARKLE, Janet; TROUILLET, Celine; JABOT-HANIN, Fabienne; ARLEHAMN, Cecilia S. Lindestam; RAO, Geetha; PICARD, Capucine; LASSEAU, Theo; LATORRE, Daniela; HAMBLETON, Sophie; DESWARTE, Caroline; ITAN, Yuval; ABARCA, Katia; MORAES-VASCONCELOS, Dewton; AILAL, Fatima; IKINCIOGULLARI, Aydan; DOGU, Figen; BENHSAIEN, Ibtihal; SETTE, Alessandro; ABEL, Laurent; BOISSON-DUPUIS, Stephanie; SCHROEDER, Bernd; NUSSENZWEIG, Michel C.; LIU, Kang; GEISSMANN, Frederic; TANGYE, Stuart G.; GROS, Philippe; SALLUSTO, Federica; BUSTAMANTE, Jacinta; CASANOVA, Jean-Laurent
    Human inborn errors of IFN-gamma immunity underlie mycobacterial diseases. We describe patients with Mycobacterium bovis (BCG) disease who are homozygous for loss-of-function mutations of SPPL2A. This gene encodes a transmembrane protease that degrades the N-terminal fragment (NTF) of CD74 (HLA invariant chain) in antigen-presenting cells. The CD74 NTF therefore accumulates in the HLA class II+ myeloid and lymphoid cells of SPPL2a-deficient patients. This toxic fragment selectively depletes IL-12-and IL-23-producing CD1c(+) conventional dendritic cells (cDC2s) and their circulating progenitors. Moreover, SPPL2a-deficient memory T(H)1(star) cells selectively fail to produce IFN-gamma when stimulated with mycobacterial antigens in vitro. Finally, Sppl2a(-/-) mice lack cDC2s, have CD4(+) T cells that produce small amounts of IFN-gamma after BCG infection, and are highly susceptible to infection with BCG or Mycobacterium tuberculosis. These findings suggest that inherited SPPL2a deficiency in humans underlies mycobacterial disease by decreasing the numbers of cDC2s and impairing IFN-gamma production by mycobacterium-specific memory T(H)1(star) cells.
  • article 7 Citação(ões) na Scopus
    Case report of myeloperoxidase deficiency associated with disseminated paracoccidioidomycosis and peritoneal tuberculosis
    (2017) DOMINGUES-FERREIRA, Mauricio; LEVY, Ariel; BARROS, Noac Chuffi; BERTOLINI, Dalton Luis; VASCONCELOS, Dewton de Moraes
    Myeloperoxidase (MOP) is present in monocyte and neutrophil lysosomes, catalyzing hydrogen peroxide and chloride ion conversion to hypochlorous acid. MOP seems to destroy pathogens during phagocytosis by neutrophils and is considered an important defense against innumerous bacteria. We present a patient who had MOP deficiency, who presented with a subacute form of paracoccidioidomycosis and later with peritoneal tuberculosis. MOP deficiency leads to the diminished destruction of phagocytized pathogens. This case gives important evidence of an association between MOP deficiency and increased susceptibility to infection by Paracoccidioides brasiliensis and Mycobacterium tuberculosis.
  • article 1 Citação(ões) na Scopus
    Chronic mucocutaneous candidiasis associated with paracoccidioidomycosis in a patient with mannose receptor deficiency: First case reported in the literature
    (2021) VASCONCELOS, Dewton de Moraes; BERTOLINI, Dalton Luis; FERREIRA, Mauricio Domingues
    We describe the first report of a patient with chronic mucocutaneous candidiasis associated with disseminated and recurrent paracoccidioidomycosis. The investigation demonstrated that the patient had a mannose receptor deficiency, which would explain the patient's susceptibility to chronic infection by Candida spp. and systemic infection by paracoccidioidomycosis. Mannose receptors are responsible for an important link between macrophages and fungal cells during phagocytosis. Deficiency of this receptor could explain the susceptibility to both fungal species, suggesting the impediment of the phagocytosis of these fungi in our patient.
  • article 3 Citação(ões) na Scopus
    Chronic mucocutaneous candidiasis and systemic lupus erythematosus: a new variant of chronic mucocutaneous candidiasis?
    (2012) MORAES-VASCONCELOS, Dewton de; DOMINGUES-FERREIRA, Mauricio; PIERI, Patricia de Campos; DUARTE, Alberto Jose da Silva
    Chronic mucocutaneous candidiasis (CMC) is characterized by susceptibility to Candida infection of skin, nails, and mucous membranes. Autoimmune endocrinopathies are common in CMC patients, but there are no reports of the involvement of systemic autoimmune disorders. We present here the first case of this kind of association in a patient with an autosomal dominant variant of CMC. The individual had had this disorder since childhood and systemic lupus erythematosus with secondary antiphospholipid syndrome, as well as renal, articular and hepatic manifestations without thymoma.
  • conferenceObject
    MUTATIONAL CHARACTERISTICS OF PATIENTS WITH X-LINKED AGAMMAGLOBULINEMIA WITH THE CARRIER IDENTIFIED BY GENETIC ANALYSIS
    (2012) RAMALHO, V. D.; VASCONCELOS, D.; ROXO, P.; SILVA, M. T. N. da; VILELA, M. M. S.
    Background: X-linked agammaglobulinemia (XLA) is a humoral primary immunodeficiency in which affected patients have very low levels of peripheral B cells and a profound deficiency of all immunoglobulin isotypes. The mutational screening of Bruton ́s tyrosine kinase (BTK) gene could detect XLA as well as its carrier. Materials and methods: In this work, we describe 13 patients with BTK mutations and the carrier status of their family members. DNA was prepared from the patients' peripheral blood cells and RNA from the patients' peripheral blood mononuclear cells. BTK gene analysis was carried out using PCR or RT-PCR followed by sequencing. Results: We identified five patients with missense mutations, five premature stop codons caused by substitutions or frameshifts, one deletion of 23 nucleotides and two with splice site defects. Most of these mutations were located at the kinase domain of BTK and, less frequently, they were found in PH and 271 SH2 domains. BTK mutated alleles was identified in nine women, defining them as XLA carriers. Conclusions: Characterization of the mutations responsible for XLA allowed us to diagnose the disease conclusively and enables subsequent genetic counseling of patients' relatives.
  • article 6 Citação(ões) na Scopus
    CD18 deficiency evolving to megakaryocytic (M7) acute myeloid leukemia: Case report
    (2014) VASCONCELOS, Dewton de Moraes; BEITLER, Beatriz; MARTINEZ, Gracia A.; PEREIRA, Juliana; AMIGO FILHO, Jose Ulysses; KLAUTAU, Giselle Burlamaqui; LIAN, Yu Cheng; NEGRA, Marinella Della; DUARTE, Alberto Jose da Silva
    Leukocyte adhesion deficiency type 1 (LAD 1 - CD18 deficiency) is a rare disease characterized by disturbance of phagocyte function associated with less severe cellular and humoral dysfunction. The main features are bacterial and fungal infections predominantly in the skin and mucosal surfaces, impaired wound healing and delayed umbilical cord separation. The infections are indolent, necrotic and recurrent. In contrast to the striking difficulties in defense against bacterial and fungal microorganisms, LAD 1 patients do not exhibit susceptibility to viral infections and neoplasias. The severity of clinical manifestations is directly related to the degree of CD18 deficiency. Here, a 20 year-old female presenting a partial CD18 deficiency that developed a megakaryocytic (M7) acute myeloid leukemia is described for the first time. The clinical features of the patient included relapsing oral thrush due to Candida, cutaneous infections and upper and lower respiratory tract infections, followed by a locally severe necrotic genital herpetic lesion. The patient's clinical features improved for a period of approximately two years, followed by severe bacterial infections. At that time, the investigation showed a megakaryocytic acute myeloid leukemia, treated with MEC without clinical improvement. The highly aggressive evolution of the leukemia in this patient suggests that adhesion molecules could be involved in the protection against the spread of neoplastic cells. (C) 2014 Published by Elsevier Inc.