JULIANA FOLLONI FERNANDES

(Fonte: Lattes)
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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

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Agora exibindo 1 - 10 de 24
  • 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 6 Citação(ões) na Scopus
    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.
  • article 82 Citação(ões) na Scopus
    Hematopoietic cell transplantation in chronic granulomatous disease: a study of 712 children and adults
    (2020) CHIESA, Robert; WANG, Junfeng; BLOK, Henric-Jan; HAZELAAR, Sheree; NEVEN, Benedicte; MOSHOUS, Despina; SCHULZ, Ansgar; HOENIG, Manfred; HAUCK, Fabian; SERAIHY, Amal Al; GOZDZIK, Jolanta; LJUNGMAN, Per; LINDEMANS, Caroline A.; FERNANDES, Juliana F.; KALWAK, Krzysztof; STRAHM, Brigitte; SCHANZ, Urs; SEDLACEK, Petr; SYKORA, Karl-Walter; AKSOYLAR, Serap; LOCATELLI, Franco; STEPENSKY, Polina; WYNN, Robert; LURN, Su Han; ZECCA, Marco; PORTA, Fulvio; TASKINEN, Mervi; GIBSON, Brenda; MATTHES, Susanne; KARAKUKCU, Musa; HAURI-HOHL, Mathias; VEYS, Paul; GENNERY, Andrew R.; LUCCHINI, Giovanna; FELBER, Matthias; ALBERT, Michael H.; BALASHOV, Dmitry; LANKESTER, Arjan; GUNGOR, Tayfun; SLATTER, Mary A.
    Chronic granulomatous disease (CGD) is a primary immunodeficiency resulting in life-threatening infections and inflammatory complications. Allogeneic hematopoietic cell transplantation (allo-HCT) can cure the disease, but the indication to transplant remains controversial. We performed a retrospective multicenter study of 712 patients with CGD who underwent allo-HCT transplantation from March 1993 through December 2018. We studied 635 children (aged <18 years) and 77 adults. Median follow-up was 45 months. Median age at transplantation was 7 years (range, 0.1-48.6). Kaplan-Meier estimates of overall survival (OS) and event-free survival (EFS) at 3 years were 85.7% and 75.8%, respectively. In multivariate analysis, older age was associated with reduced survival and increased chronic graft-versus-host disease. Nevertheless, OS and EFS at 3 years for patients >= 18 years were 76% and 69%, respectively. Use of 1-antigen-mismatched donors was associated with reduced OS and EFS . No significant difference was found in OS, but a significantly reduced EFS was noted in the small group of patients who received a transplant from a donor with a >1 antigen mismatch. Choice of conditioning regimen did not influence OS or EFS. In summary, we report an excellent outcome after allo-HCT in CGD, with low incidence of graft failure and mortality in all ages. Older patients and recipients of 1-antigen-mismatched grafts had a less favorable outcome. Transplantation should be strongly considered at a younger age and particularly in the presence of a well-matched donor.
  • article 9 Citação(ões) na Scopus
    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, Gil
    Background 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.
  • article 2 Citação(ões) na Scopus
    Umbilical Cord Mesenchymal Stromal Cells for Steroid-Refractory Acute Graft-versus-Host Disease
    (2023) DONADEL, Camila Derminio; PIRES, Bruno Garcia; ANDRE, Nathalia Cristine; COSTA, Thalita Cristina Mello; ORELLANA, Maristela Delgado; CARUSO, Samia Rigotto; SEBER, Adriana; GINANI, Valeria Cortez; GOMES, Alessandra Araujo; NOVIS, Yana; BARROS, George Mauricio Navarro; VILELLA, Neysimelia Costa; MARTINHO, Glaucia Helena; VIEIRA, Ana Karine; KONDO, Andrea Tiemi; HAMERSCHLAK, Nelson; FILHO, Jayr Schmidt; XAVIER, Erick Menezes; FERNANDES, Juliana Folloni; ROCHA, Vanderson; COVAS, Dimas Tadeu; CALADO, Rodrigo Tocantins; GUERINO-CUNHA, Renato Luiz; SANTIS, Gil Cunha De
    Background: Steroid-refractory acute graft-vs.-host disease (SR-aGVHD) is a complication of allogeneic hematopoietic stem cell transplantation with a dismal prognosis and for which there is no consensus-based second-line therapy. Ruxolitinib is not easily accessible in many countries. A possible therapy is the administration of mesenchymal stromal cells (MSCs). Methods: In this retrospective study, 52 patients with severe SR-aGVHD were treated with MSCs from umbilical cord (UC-MSCs) in nine institutions. Results: The median (range) age was 12.5 (0.3-65) years and the mean +/- SD dose (x10(6)/kg) was 4.73 +/- 1.3 per infusion (median of four infusions). Overall (OR) and complete response (CR) rates on day 28 were 63.5% and 36.6%, respectively. Children (n = 35) had better OR (71.5% vs. 47.1%, p = 0.12), CR (48.6% vs. 11.8%, p = 0.03), overall survival (p = 0.0006), and relapse-free survival (p = 0.0014) than adults (n = 17). Acute adverse events (all of them mild or moderate) were detected in 32.7% of patients, with no significant difference in children and adult groups (p = 1.0). Conclusions: UC-MSCs are a feasible alternative therapy for SR-aGVHD, especially in children. The safety profile is favorable.
  • article 4 Citação(ões) na Scopus
    Hematopoietic cell transplantation for Diamond Blackfan anemia: A report from the Pediatric Group of the Brazilian Bone Marrow Transplantation Society
    (2020) DARRIGO JR., Luiz Guilherme; LOTH, Gisele; KUWAHARA, Cilmara; VIEIRA, Ana; COLTURATO, Vergilio; RODRIGUES, Ana Luiza; ARCURI, Leonardo; FERNANDES, Juliana; MACEDO, Antonio; TAVARES, Rita; GOMES, Alessandra; RIBEIRO, Lisandro; SEBER, Adriana; ZECCHIN, Victor; SOUZA, Mair de; CALIXTO, Rodolfo; PASQUINI, Ricardo; FLOWERS, Mary; ROCHA, Vanderson; BONFIM, Carmem
    Objectives The aim of this study was to analyze the outcomes of children with Diamond-Blackfan anemia (DBA) treated in Brazil with hematopoietic cell transplantation (HCT). Methods We performed a retrospective analysis of 44 pediatrics patients transplanted between 1990 and 2018. The median age of patients was 5 years, and 57% were male. Twenty-five received their first HCT from an HLA-matched sibling donor (MSD), 12 from a HLA matched unrelated bone marrow donor (MUD 10/10, n = 12) and 7 other HLA mismatched donors (MMD). Results After a median follow-up of 4 years, estimate 5-year overall survival (OS) for the entire cohort was 70%, 80% for MSD group, 73% for MUD, and 29% for MMD. Thirty-eight out of the 44 evaluable patients engrafted successfully. Primary and secondary graft failure was observed in five and three patients, respectively. Rates of grade II-IV and III-IV acute graft-versus-host disease (aGVHD) were 25% and 18%, respectively. Nine patients developed chronic GVHD (cGVHD). Conclusion Overall survival rates observed after HLA matched donors transplant for DBA were comparable to those reported from higher-income countries and international registries.
  • article 3 Citação(ões) na Scopus
    Allogeneic Hematopoietic Stem Cell Transplantation for Children and Adolescents with Acute Myeloid Leukemia in Brazil: A Multicentric Retrospective Study
    (2020) RODRIGUES, Ana Luiza de Melo; BONFIM, Carmem; SEBER, Adriana; COLTURATO, Vergilio Antonio Rensi; ZECCHIN, Victor Gottardello; NICHELE, Samantha; DAUDT, Liane Esteves; FERNANDES, Juliana Folloni; VIEIRA, Ana Karine; DARRIGO JUNIOR, Luiz Guilherme; GOMES, Alessandra Araujo; ARCURI, Leonardo; LENZI, Luana; PICHARSKI, Gledson Luiz; RIBEIRO, Raul Correa; FIGUEIREDO, Bonald Cavalcante de
    The survival rates of children with high-risk acute myeloid leukemia (AML) treated with hematopoietic stem cell transplant (HSCT) range from 60% to 70% in high-income countries. The corresponding rate for Brazilian children with AML who undergo HSCT is unknown. We conducted a retrospective analysis of 114 children with AML who underwent HSCT between 2008 and 2012 at institutions participating in the Brazilian Pediatric Bone Marrow Transplant Working Group. At transplant, 38% of the children were in first complete remission (CR1), 37% were in CR2, and 25% were in CR3+ or had persistent disease. The donors included 49 matched-related, 59 matched-unrelated, and six haploidentical donors. The most frequent source of cells was bone marrow (69%), followed by the umbilical cord (19%) and peripheral blood (12%). The 4-year overall survival was 47% (95% confidence interval [CI] 30%-57%), and the 4-year progression-free survival was 40% (95% CI 30%-49%). Relapse occurred in 49 patients, at a median of 122 days after HSCT. There were 65 deaths: 40 related to AML, 19 to infection, and six to graft versus host disease. In conclusion, our study suggests that HSCT outcomes for children with AML in CR1 or CR2 are acceptable and that this should be considered in the overall treatment planning for children with AML in Brazil. Therapeutic standardization through the adoption of multicentric protocols and appropriate supportive care treatment will have a significant impact on the results of HSCT for AML in Brazil and possibly in other countries with limited resources.
  • article 13 Citação(ões) na Scopus
    Haploidentical bone marrow transplantation with post transplant cyclophosphamide for patients with X-linked adrenoleukodystrophy: a suitable choice in an urgent situation
    (2018) FERNANDES, Juliana Folloni; BONFIM, Carmem; KERBAUY, Fabio Rodrigues; RODRIGUES, Morgani; ESTEVES, Iracema; SILVA, Nathalia Halley; AZAMBUJA, Alessandra Prandini; MANTOVANI, Luiz Fernando; KUTNER, Jose Mauro; LOTH, Gisele; KUWAHARA, Cilmara Cristina; BUENO, Clarissa; KONDO, Andrea Tiemi; RIBEIRO, Andreza Alice Feitosa; KOK, Fernando; HAMERSCHLAK, Nelson
    Allogeneic hematopoietic stem cell transplantation (HSCT) is the only treatment that enhances survival and stabilizes neurologic symptoms in X-linked adrenoleukodystrophy (X-ALD) with cerebral involvement, a severe demyelinating disease of childhood. Patients with X-ALD who lack a well-matched HLA donor need a rapid alternative. Haploidentical HSCT using post transplant cyclophosphamide (PT/Cy) has been performed in patients with malignant and nonmalignant diseases showing similar outcomes compared to other alternative sources. We describe the outcomes of transplants performed for nine X-ALD patients using haploidentical donors and PT/Cy. Patients received conditioning regimen with fludarabine 150 mg/m(2) , cyclophosphamide 29 mg/kg and 2 Gy total body irradiation (TBI) with or without antithymocyte globulin. Graft-vs.-host disease prophylaxis consisted of cyclophosphamide 50 mg/kg/day on days +3 and +4, tacrolimus or cyclosporine A and mycophenolate mofetil. One patient had a primary graft failure and was not eligible for a second transplant. Three patients had secondary graft failure and were successfully rescued with second haploidentical transplants. Trying to improve engraftment, conditioning regimen was changed, substituting 2 Gy TBI for 4 Gy total lymphoid irradiation. Eight patients are alive and engrafted (17-37 months after transplant). Haploidentical HSCT with PT/Cy is a feasible alternative for X-ALD patients lacking a suitable matched donor. Graft failure has to be addressed in further studies.
  • article 54 Citação(ões) na Scopus
    Outcome of SARS-CoV-2 Infection in 121 Patients with Inborn Errors of Immunity: A Cross-Sectional Study
    (2021) GOUDOURIS, Ekaterini Simoes; PINTO-MARIZ, Fernanda; MENDONCA, Leonardo Oliveira; ARANDA, Carolina Sanchez; GUIMARAES, Rafaela Rolla; KOKRON, Cristina; BARROS, Myrthes Toledo; ANISIO, Flavia; ALONSO, Maria Luiza Oliva; MARCELINO, Fernanda; VALLE, Solange Oliveira Rodrigues; DORTAS JUNIOR, Sergio; BARRETO, Irma Douglas Paes; FERREIRA, Janaira Fernandes Severo; ROXO-JUNIOR, Persio; SILVA, Almerinda Maria do Rego; CAMPINHOS, Fernanda Lugao; BONFIM, Carmem; LOTH, Gisele; FERNANDES, Juliana Folloni; GARCIA, Julia Lopes; CAPELO, Albertina; TAKANO, Olga Akiko; NADAF, Maria Isabel Valdomir; TOLEDO, Eliana C.; CUNHA, Luciana Araujo Oliveira; GESU, Regina Sumiko Watanabe Di; SCHIDLOWSKI, Laire; FILLIPO, Priscila; BICHUETTI-SILVA, Danielli C.; SOLDATELI, Gustavo; FERRARONI, Natasha Reboucas; DANTAS, Ellen de Oliveira; PESTANA, Simone; MANSOUR, Eli; ULAF, Raisa Gusso; PRANDO, Carolina; CONDINO-NETO, Antonio; GRUMACH, Anete Sevciovic
    Purpose There is still scarce data on SARS-CoV-2 infection in patients with Inborn Errors of Immunity (IEI) and many unresolved questions. We aimed to describe the clinical outcome of SARS-CoV-2 infection in Brazilian IEI patients and identify factors influencing the infection. Methods We did a cross-sectional, multicenter study that included patients of any age affected by IEI and SARS-CoV-2 infection. The variables studied were sex, age, type of IEI, comorbidities (number and type), treatment in use for IEI, clinical manifestations and severity of SARS-CoV-2 infection. Results 121 patients were included: 55.4% female, ages from six months to 74 yo (median age = 25.1 yo). Most patients had predominantly antibody deficiency (n = 53). The infection was mostly asymptomatic (n = 21) and mild (n = 66), and one child had multisystem inflammatory syndrome (MIS-C). We could not observe sex-related susceptibility, and there was a weak correlation between age and severity of infection. The number of comorbidities was higher in severe cases, particularly bronchiectasis and cardiopathy. There were no severe cases in hereditary angioedema patients. Six patients aged 2 to 74 years died, three of them with antibody deficiency. Conclusion The outcome was mild in most patients, but the Case Fatality Ratio was higher than in the general population. However, the type of IEI was not a determining factor for severity, except for complement deficiencies linked to milder COVID-19. The severity of SARS-CoV-2 infection seems to be more related to older age, a higher number of comorbidities and type of comorbidities (bronchiectasis and cardiopathy).
  • article 23 Citação(ões) na Scopus
    Autoimmune manifestations in SCID due to IL7R mutations: Omenn syndrome and cytopenias
    (2014) ZAGO, Claudia Augusta; JACOB, Cristina Miuki Abe; DINIZ, Edna Maria de Albuquerque; LOVISOLO, Silvana Maria; ZERBINI, Maria Claudia Nogueira; DORNA, Mayra; WATANABE, Leticia; FERNANDES, Juliana Folloni; ROCHA, Vanderson; OLIVEIRA, Joao Bosco; CARNEIRO-SAMPAIO, Magda
    B+NK+SCID (severe combined immunodeficiency) due to IL7R alpha deficiency represents approximately 10% of American SCID cases. To better understand the spectrum of autoimmune disorders associated with IL7R alpha deficiency, we describe two unrelated IL7R alpha-deficient female SCID infants whose clinical picture was dominated by autoimmune manifestations: one with intrauterine Omenn syndrome (OS) and another with persistent thrombocytopenic purpura since 4 months of age. The OS baby harbored a homozygous p.C118Y mutation in IL7R. She presented dense eosinophilic infiltrates in several organs, including pancarditis, which may have contributed to her death (on the 2nd day of life). B cells were observed in lymph nodes, spleen, bone marrow and thymus. The second patient harbored compound heterozygous p.01 8Y and p.I121NfsX8 mutations. She underwent a successful unrelated cord blood transplant. In conclusion, early OS can be observed in patients with IL7R mutations, and autoimmune cytopenias could also complicate the clinical course of SCID babies with this type of defect. (C) 2014 Published by Elsevier Inc. on behalf of American Society for Histocompatibility and Immunogenetics.