JULIANA FOLLONI FERNANDES
Projetos de Pesquisa
Unidades Organizacionais
Instituto da Criança, Hospital das Clínicas, Faculdade de Medicina - Médico
LIM/36 - Laboratório de Pediatria Clínica, Hospital das Clínicas, Faculdade de Medicina
LIM/36 - Laboratório de Pediatria Clínica, Hospital das Clínicas, Faculdade de Medicina
31 resultados
Resultados de Busca
Agora exibindo 1 - 10 de 31
conferenceObject NON-MYELOABLATIVE HAPLOIDENTICAL BMT WITH PTCY FOR CHILDREN AND ADULTS WITH SICKLE CELL DISEASES: THE BRAZILIAN EXPERIENCE(2023) BONFIM, Carmem; SIMOES, Belinda; GOUVEIA, Roseane V.; SILVA, Roberto Luiz; LEITE, Lauro Augusto C.; FERNANDES, Juliana Folloni; GARCIA, Julia Lopes; ZECCHIN, Victor Gottardello; LEE, Maria Lucia; KUWAHARA, Cilmara; GOMES, Alessandra Araujo; RODRIGUES, Celso Arrais; NICHELE, Samantha; LOTH, Gisele; GINANI, Valeria Cortze; FELICIANO, Joao Vitor; LIMA, Alberto Cardoso Martins; DARRIGO JR., Luiz Guilherme; FUENTE, Josu De La; KASSIM, Adetola; SEBER, AdrianaconferenceObject Hospital Length of Stay and Impact of Readmission in the First 100 Days of Allogeneic Stem Cell Transplantation: Comparison among Alternative Donor in Pediatric and Adult Population(2018) KERBAUY, Mariana Nassif; KERBAUY, Lucila Nassif; ESTEVES, Iracema; ROCHA, Juliana DallAgnol; STANZIONE, Renata Leati; RODRIGUES, Morgani; FERNANDES, Juliana Folloni; KUTNER, Jose Mauro; SOBRINHO, Jairo J. N.; MANTOVANI, Luiz Fernando Alves Lima; KERBAUY, Fabio R.; RIBEIRO, Andreza Feitosa; HAMERSCHLAK, NelsonconferenceObject The Brazilian Experience with Haploidentical Hematopoietic Cell Transplants (Haplo-HCT) with Post-Transplant Cyclophosphamide (PTCy) in Pediatric Patients with Hematological Malignancies(2020) BONFIM, Carmem; ARCURI, Leonardo J.; COLTURATO, Vergilio; ZECCHIN, Victor G.; KUWAHARA, Cilmara C.; RIBEIRO, Lisandro L.; GOUVEIA, Roseane; FERNANDES, Juliana F.; TAVARES, Rita; DAUDT, Liane E.; DARRIGO JR., Luiz G.; SOUZA, Mair P. de; ROCHA, Vanderson; VILLELA, Neysimelia C.; MARIANO, Livia C. B.; GINANI, Valeria C.; LOTH, Gisele; GOMES, Alessandra A.; ZANETTE, Antonella; HAMERSCHLAK, Nelson; FLOWERS, Mary E.- 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. deLomentospora 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.
- Multicenter Long-Term Follow-Up of Allogeneic Hematopoietic Cell Transplantation with Omidubicel: A Pooled Analysis of Five Prospective Clinical Trials(2023) LIN, Chenyu; SCHWARZBACH, Aurelie; SANZ, Jaime; MONTESINOS, Pau; STIFF, Patrick; PARIKHA, Suhag; BRUNSTEIN, Claudio; CUTLER, Corey; LINDEMANS, Caroline A.; HANNA, Rabi; KOH, Liang Piu; JAGASIA, Madan H.; VALCARCEL, David; MAZIARZ, Richard T.; KEATING, Amy K.; HWANG, William Y. K.; REZVANI, Andrew R.; KARRAS, Nicole A.; FERNANDES, Juliana F.; ROCHA, Vanderson; BADELL, Isabel; RAM, Ron; SCHILLER, Gary J.; VOLODIN, Leonid; WALTERS, Mark C.; HAMERSCHLAK, Nelson; CILLONI, Daniela; FRANKFURT, Olga; MCGUIRK, Joseph P.; KURTZBERG, Joanne; SANZ, Guillermo; SIMANTOV, Ronit; HORWITZ, Mitchell E.Omidubicel is an umbilical cord blood (UCB)-derived ex vivo-expanded cellular therapy product that has dem-onstrated faster engraftment and fewer infections compared with unmanipulated UCB in allogeneic hematopoietic cell transplantation. Although the early benefits of omidubicel have been established, long-term outcomes remain unknown. We report on a planned pooled analysis of 5 multicenter clinical trials including 105 patients with hematologic malignancies or sickle cell hemoglobinopathy who underwent omidubicel transplantation at 26 academic transplantation centers worldwide. With a median follow-up of 22 months (range, .3 to 122 months), the 3-year estimated overall survival and disease-free survival were 62.7% and 56.4%, respectively. With up to 10 years of follow-up, omidubicel showed durable trilineage hematopoiesis. Serial quantitative assessments of CD3(+), CD4(+), CD8(+), CD19(+), CD116(+)CD56(+), and CD123(+) immune subsets revealed median counts remaining within normal ranges through up to 8 years of follow-up. Secondary graft failure occurred in 5 patients (5%) in the first year, with no late cases reported. One case of donor-derived myeloid neoplasm was reported at 40 months post-transplantation. This was also observed in a control arm patient who received only unmanipulated UCB. Overall, omidubicel demonstrated stable trilineage hematopoiesis, immune competence, and graft durability in extended follow-up. (c) 2023 The American Society for Transplantation and Cellular Therapy.
- Hematopoietic Stem Cell Transplantation for Chronic Granulomatous Disease in a Single Institution in Brazil. Reproducing Good Results with a Reduced Toxicity Regimen(2017) FERNANDES, Juliana Folloni; MANTOVANI, Luiz Fernando Alves Lima; VENANCIO, Angela Mandelli; DORNA, Mayra; PASTORINO, Antonio Carlos; VASCONCELOS, Dewton; NETO, Antonio Condino; MOURA, Ana Carla Augusto; COLLASSANTI, Maria Dulce; ZANICHELLI, Maria Aparecida; CARNEIRO-SAMPAIO, Magda; ROCHA, Vanderson G.; ODONE FILHO, Vicente
- Age-associated phenotypic imbalance in TCD4 and TCD8 cell subsets: comparison between healthy aged, smokers, COPD patients and young adults(2022) FERNANDES, Juliana Ruiz; PINTO, Thalyta Nery Carvalho; ARRUDA, Lia Barbara; SILVA, Cibele Cristine Berto Marques da; CARVALHO, Celso Ricardo Fernandes de; PINTO, Regina Maria Carvalho; DUARTE, Alberto Jose da Silva; BENARD, GilBackground COPD is associated with an abnormal lung immune response that leads to tissue damage and remodeling of the lung, but also to systemic effects that compromise immune responses. Cigarette smoking also impacts on innate and adaptative immune responses, exerting dual, pro- and anti-inflammatory effects. Previously, we showed that COPD patients presented accelerated telomere shortening and decreased telomerase activity, while, paradoxically, cigarette-smokers exhibited preserved telomerase activity and slower rate of telomere shortening. Results Here, we evaluated the naive, CM, EM and T-EMRA subsets of TCD4 and TCD8 cells according to the expression of CCR7/CD45RA. We compared age-matched COPD patients, cigarette-smokers without clinical-laboratory evidence of pulmonary compromise, and healthy individuals. They were additionally compared with a group of young adults. For each subset we analysed the expression of markers associated with late differentiation, senescence and exhaustion (CD27/CD28/CD57/KLRG1/PD1). We show that COPD patients presented a drastically reduced naive cells pool, and, paradoxically, increased fractions of naive cells expressing late differentiation, senescence or exhaustion markers, likely impacting on their immunocompetence. Pronounced phenotypic alterations were also evidenced in their three memory T-cell subsets compared with the other aged and young groups, suggesting an also dysfunctional memory pool. Surprisingly, our smokers showed a profile closer to the Healthy aged than COPD patients. They exhibited the usual age-associated shift of naive to EM TCD4 and TCD8 cells, but not to CM or T-EMRA T-cells. Nonetheless, their naive T-cells phenotypes were in general similar to those of the Youngs and Healthy aged, suggesting a rather phenotypically preserved subset, while the memory T-cells exhibited increased proportions of cells with the late-differentiation or senescence/exhaustion markers as in the Healthy aged. Conclusion Our study extends previous findings by showing that COPD patients have cells expressing a full range of late differentiated, senescent or exhausted phenotypes encompassing all TCD4 and TCD8 subsets, consistent with a premature immunosenescence phenotype. Surprisingly, the smokers group's results suggest that moderate to heavy chronic cigarette smoking did not accelerate the pace of immunosenescence as compared with the Healthy aged.
conferenceObject T-CELL- AND ANTIBODY-MEDIATED AUTOIMMUNE MANIFESTATIONS IN SCID DUE TO IL7RA MUTATIONS(2012) ZAGO, C. A.; JACOB, C. M. A.; DINIZ, E. M. D. A.; ZERBINI, C.; DORNA, M.; FERNANDES, J.; ROCHA, V.; OLIVEIRA, J. B.; CARNEIRO-SAMPAIO, M.Introduction: B+ SCID due to IL7Ra deficiency represents around 10% of SCID cases, and has seldom been described among Brazilian patients. Objective: We present two unrelated SCID female infants with IL7RA mutations and distinct autoimmune manifestations. Case 1: This infant was born to non-consanguineous parents at 28 weeks of gestational age presenting characteristic clinical as pects of Omenn syndrome (OS). She died after 2 days due to meconium-aspiration pneumonitis and pancarditis (with eosinophil and histiocyte infiltration). She presented leukocytosis (19,500/mm3), eosinophilia (4,860cells/mm3), lymphocytes=2,925cells/mm3 (CD3+=684cells/mm3, CD4+=345cells/mm3, CD8+=6cells/mm3, without naive T-cells, CD25+Foxp3+=2.3%, CD19+=641cells/mm 3, CD3-CD16+CD56+=280cells/mm3), thrombocytopenia=49,000/mm3, IgG=468mg/dL, IgM=45mg/dL, IgA<22mg/dL, IgE=3,310UI/ml. She harbored a homozygous p.C118Y IL7RA mutation. She is the second IL7Ra deficient OS in literature; the first described case presented the same mutation and was also Brazilian. Case 2: An 8-month-old girl presented since 4 months-old with severe thrombocytopenic purpura, treated with high IVIg doses and corticosteroids. She presented lymphocytopenia=1,287cells/mm3 (CD3=147cells/mm3, CD4=36cells/mm3, CD8=72cells/mm3), normal B (184cells/mm3, 80% withs IgM+IgD+) and NK numbers (259cells/mm3), very low TRECs, IgM=235mg/dL, IgA=51mg/dL, IgE=5UI/ml, and positive anti-nuclear antibodies (1/320). A sister with an equivalent clinical picture died at 15 months of age. She had compound heterozygous p.C118Y and p.I121NfsX8 IL7RA mutations. She did not present serious infections, and was successfully transplanted with cord blood cells at 13-months-old. Conclusions: Autoimmune diseases associated to “leaky” SCIDs due to IL7RA mutations may be mediated by both autoreactive T lymphocytes (probably in case1, an intrauterine OS) and autoantibodies (probably the predominant pathogenic mechanism in case2).conferenceObject Allogeneic bone marrow transplantation for children and adolescents with severe aplastic anemia in Brazil: A multicenter study on behalf of the Brazil-Seattle Consortium Study Group(2018) DARRIGO JR., Luiz; COULTURATO, Vergilio; SOUZA, Mair; MATTOS, Ederson; LOTH, Gisele; CALIXTO, Rodolfo; SEBER, Adriana; ZECCHIN, Victor; DAUDT, Liane; PAZ, Alessandra; TAVARES, Rita Barbosa; ARCURI, Leonardo; MACEDO, Antonio; VIEIRA, Ana Karine; KUWAHARA, Cilmara; GOUVEIA, Roseane; RIBEIRO, Lisandro; FERNANDES, Juliana; FLOWERS, Mary; PASQUINI, Ricardo; BONFIM, CarmemconferenceObject Familial Hemophagocytic Lymphohistiocytosis Caused by Perforin Deficiency in Brazilian Twins(2012) JACOB, Cristina Miuki Abe; SANTOS, Cristiane de Jesus Nunes dos; SAINT-BASILE, Genevieve de; CASTRO, Ana Paula B. Moschione; PASTORINO, Antonio Carlos; FERNANDES, Juliana Folloni; ROCHA, Vanderson; CARNEIRO-SAMPAIO, Magda M. SalesTwo female monozygotic twins presented: Case1-at2mo presented fever and vomiting after vaccination with DTP, Haemophilus, Salk, Rotavirus. The initial evaluation showed: anemia, hepatosplenomegaly, pancytopenia, LDH=0760 U/L, ferritin=0622 ng/mL and triglycerides=0362 mg/dL. Hemophagocytosis was found in bone marrow. Case2: clinically asymptomatic, being detected anemia, LDH=0726 U/L, ferritin=0436 ng/mL, triglycerides=0166 mg/dL, without hemophagocytosis. Infections were excluded in both. Molecular testing identified two heterozygous mutations in the perforin gene, C46T leading to P16S and 50delT leading to L17 stop, making the diagnosis of FHL type 2. Both twins underwent to therapy based on HLH-2004 protocol followed by cord blood transplantation and after CMV infection with a good response to treatment. FHL should be suspected in all children with fever, visceromegaly and cytopenias for early treatment, including hematopoietic stem cell transplantation.