JULIANA FOLLONI FERNANDES

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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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  • conferenceObject
    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.
  • 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 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.
  • conferenceObject
    Relapsed Acute Lymphoblastic Leukemia and Blinatumomab: Results From a Single Institution in Brazil Universidade de Sao Paulo
    (2020) ZAMPERLINI-NETTO, G.; FONSECA, M.; ALMEIDA, M.; ODONE-FILHO, V.; TEIXEIRA, R.; FERNANDES, J.; AZAMBUJA, A.; DUTRA, A.; BREVIGLIERI, C.; CRISTOFANI, L.
  • conferenceObject
    Outcomes and Immune Reconstitution After T-Cell Replete Haploidentical Stem Cell Transplantation With Post-Transplantation Cyclophosphamide (PTCY) for Pediatric Patients with Primary Immunodeficiencies
    (2020) FERNANDES, Juliana Folloni; NICHELE, Samantha; ARCURI, Leonardo Javier; RIBEIRO, Lisandro; NETTO, Gabriele Zamperlini; LOTH, Gisele; RODRIGUES, Ana Luiza Melo; KUWAHARA, Cilmara; KOLISKI, Adriana; GARCIA, Julia Lopes; DAUDT, Liane Esteves; SEBER, Adriana; GOMES, Alessandra Araujo; HAMERSCHLAK, Nelson; BONFIM, Carmem
  • article 33 Citação(ões) na Scopus
    Outcomes after Haploidentical Stem Cell Transplantation with Post-Transplantation Cyclophosphamide in Patients with Primary Immunodeficiency Diseases
    (2020) FERNANDES, Juliana Folloni; NICHELE, Samantha; ARCURI, Leonardo Javier; RIBEIRO, Lisandro; ZAMPERLINI-NETTO, Gabriele; LOTH, Gisele; RODRIGUES, Ana Luiza Melo; KUWAHARA, Cilmara; KOLISKI, Adriana; TRENNEPOHL, Joanna; GARCIA, Julia Lopes; DAUDT, Liane Esteves; SEBER, Adriana; GOMES, Alessandra Araujo; FASTH, Anders; PASQUINI, Ricardo; HAMERSCHLAK, Nelson; ROCHA, Vanderson; BONFIM, Carmem
    Allogeneic hematopoietic stem cell transplantation (HCT) can cure primary immunodeficiency diseases (PID). When a HLA-matched donor is not available, a haploidentical family donor may be considered. The use of T cell-replete haploidentical HCT with post-transplantation cyclophosphamide (haplo-PTCy) in children with PID has been reported in few case series. A donor is usually readily available, and haplo-PTCy can be used in urgent cases. We studied the outcomes of 73 patients with PID who underwent haplo-PTCy, including 55 patients who did so as a first transplantation and 18 who did so as a salvage transplantation after graft failure of previous HCT. The median patient age was 1.6 years. Most of the children were male (n = 54) and had active infection at the time of transplantation (n = 50); 10 children had severe organ damage. The diagnosis was severe combined immunodeficiency (SCID) in 34 patients and non-SCID in 39 (Wiskott-Aldrich syndrome; n = 14; chronic granulomatous disease, n = 10; other PID, n = 15). The median duration of follow-up of survivors was 2 years. The cumulative incidence of neutrophil recovery was 88% in the SCID group and 84% in non-SCID group and was 81% for first transplantations and 83% after a salvage graft. At 100 days, the cumulative incidence of acute GVHD grade II-IV and III-IV was 33% and 14%, respectively. The majority of patients reached 200/mu L CD4(+) and 1000/mu L CD3(+) cell counts between 3 and 6 months. The estimated 2-year overall survival was 66%; it was 64% for SCID patients and 65% for non-SCID patients and 63% for first HCT and 77% for salvage transplantations. Twenty-five patients died, most of them due to infection early after transplantation (before 100 days). In conclusion, haplo-PTCy is a feasible procedure, can cure two-thirds of children with PID, and can be used as rescue treatment for previous graft failure. (C) 2020 American Society for Transplantation and Cellular Therapy.
  • article 6 Citação(ões) na Scopus
    Targeted-dose of busulfan: Higher risk of sinusoidal obstructive syndrome observed with systemic exposure dose above 5000 mu Mol min. A historically controlled clinical trial
    (2020) ESTEVES, Iracema; SANTOS, Fabio Pires Souza; RIBEIRO, Andreza Alice Feitosa; SEBER, Adriana; SUGAWARA, Eduardo Kinio; SOBRINHO, Jairo Jose do Nascimento; BARROS, Jose Carlos; OLIVEIRA, Jose Salvador Rodrigues; FERNANDES, Juliana Folloni; HAMERSCHLAK, Nelson; ANDERSSON, Borje S.; LIMA, Marcos de; KERBAUY, Fabio Rodrigues
    Busulfan is given in the conditioning regimens preceding hematopoietic stem cell transplantation (HSCT), and plasma levels can be monitored. A targeted, individualized systemic exposure (SE) dose can be achieved by calculating the area under the plasma concentration versus time curve (AUC). The objective of this study was to determine a cutoff value for safety for the AUC for busulfan plasma levels in patients undergoing HSCT. A total of 149 consecutive HSCT patients were studied. After an oral test dose of busulfan, we set target doses of 4000, 5000, or 6000 mu Mol min/day, and analyzed the AUC of oral or intravenous Bu. These patients were compared with 53 historical control subjects who had received myeloablative conditioning regimen without busulfan pharmacokinetic monitoring. Using a test dose and the administration route had no impact on the sinusoidal obstructive syndrome (SOS) incidence, transplant-related mortality or 1-year overall survival. However, patients receiving busulfan at doses set up at AUC > 5000 had an increased risk to develop SOS after HSCT (hazard ratio 3.39,p= 0.034, 95% CI 1.09-10.52). Adjusting the busulfan dose according to SE levels target dose during conditioning is associated with lower rates of oral severe mucositis and SOS. A cutoff of 5000 mu Mol min is safe and does not impair survival.
  • conferenceObject
    Blinatumomab (BLINA) as Sole Therapy for Relapsed Acute Leukemia (ALL) after Allogenic Hematopoietic Stem Cell Transplantation (HSCT)
    (2020) PEREIRA, P.; VINCE, C.; BRUMATTI, M.; HALLEY, N.; AZAMBUJA, A.; FERNANDES, J.; HAMERSCHLAK, N.; ZAMPERLINI-NETTO, G.; PEREIRA, A.; KROHLING, D.; ODONE-FILHO, V.
  • conferenceObject
    T-Cell Replete Haploidentical Stem Cell Transplantation with Post-Transplantation Cyclophosphamide (PTCy) for Pediatric Patients with Primary Immunodeficiencies (PID) - a Survey of the Pediatric Transplantation Working Group of the Brazilian Society of Bone Marrow Transplantation (SBTMO)
    (2020) FERNANDES, Juliana Folloni; NICHELE, Samantha; RIBEIRO, Lisandro; LOTH, Gisele; NETTO, Gabriele Zamperlini; SEBER, Adriana; DAUDT, Liane Esteves; GOMES, Alessandra; KOLISKI, Adriana; RODRIGUES, Ana Luiza Melo; ARCURI, Leonardo Javier; HAMERSCHLAK, Nelson; BONFIM, Carmem