JULIANA FOLLONI FERNANDES

(Fonte: Lattes)
Índice h a partir de 2011
10
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 - 7 de 7
  • 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, Adriana
  • 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 14 Citação(ões) na Scopus
    Transplantation of Hematopoietic Stem Cells for Primary Immunodeficiencies in Brazil: Challenges in Treating Rare Diseases in Developing Countries
    (2018) FERNANDES, Juliana Folloni; NICHELE, Samantha; DAUDT, Liane E.; TAVARES, Rita B.; SEBER, Adriana; KERBAUY, Fabio R.; KOLISKI, Adriana; LOTH, Gisele; VIEIRA, Ana K.; DARRIGO-JUNIOR, Luiz G.; ROCHA, Vanderson; GOMES, Alessandra A.; COLTURATO, Vergilio; MANTOVANI, Luiz F.; RIBEIRO, Andreza F.; RIBEIRO, Lisandro L.; KUWAHARA, Cilmara; RODRIGUES, Ana L. M.; ZECCHIN, Victor G.; COSTA-CARVALHO, Beatriz T.; CARNEIRO-SAMPAIO, Magda; CONDINO-NETO, Antonio; FASTH, Anders; GENNERY, Andrew; PASQUINI, Ricardo; HAMERSCHLAK, Nelson; BONFIM, Carmem
    The results of hematopoietic stem cell transplant (HSCT) for primary immunodeficiency diseases (PID) have been improving over time. Unfortunately, developing countries do not experience the same results. This first report of Brazilian experience of HSCT for PID describes the development and results in the field. We included data from transplants in 221 patients, performed at 11 centers which participated in the Brazilian collaborative group, from July 1990 to December 2015. The majority of transplants were concentrated in one center (n=123). The median age at HSCT was 22months, and the most common diseases were severe combined immunodeficiency (SCID) (n=67) and Wiskott-Aldrich syndrome (WAS) (n=67). Only 15 patients received unconditioned transplants. Cumulative incidence of GVHD grades II to IV was 23%, and GVHD grades III to IV was 10%. The 5-year overall survival was 71.6%. WAS patients had better survival compared to other diseases. Most deaths (n=53) occurred in the first year after transplantation mainly due to infection (55%) and GVHD (13%). Although transplant for PID patients in Brazil has evolved since its beginning, we still face some challenges like delayed diagnosis and referral, severe infections before transplant, a limited number of transplant centers with expertise, and resources for more advanced techniques. Measures like newborn screening for SCID may hasten the diagnosis and ameliorate patients' conditions at the moment of transplant.
  • article 295 Citação(ões) na Scopus
    Reduced-intensity conditioning and HLA-matched haemopoietic stem-cell transplantation in patients with chronic granulomatous disease: a prospective multicentre study
    (2014) GUENGOER, Tayfun; TEIRA, Pierre; SLATTER, Mary; STUESSI, Georg; STEPENSKY, Polina; MOSHOUS, Despina; VERMONT, Clementien; AHMAD, Imran; SHAW, Peter J.; CUNHA, Jose Marcos Telles da; SCHLEGEL, Paul G.; HOUGH, Rachel; FASTH, Anders; KENTOUCHE, Karim; GRUHN, Bernd; FERNANDES, Juliana F.; LACHANCE, Silvy; BREDIUS, Robbert; RESNICK, Igor B.; BELOHRADSKY, Bernd H.; GENNERY, Andrew; FISCHER, Alain; GASPAR, H. Bobby; SCHANZ, Urs; SEGER, Reinhard; RENTSCH, Katharina; VEYS, Paul; HADDAD, Elie; ALBERT, Michael H.; HASSAN, Moustapha
    Background In chronic granulomatous disease allogeneic haemopoietic stem-cell transplantation (HSCT) in adolescents and young adults and patients with high-risk disease is complicated by graft-failure, graft-versus-host disease (GVHD), and transplant-related mortality. We examined the effect of a reduced-intensity conditioning regimen designed to enhance myeloid engraftment and reduce organ toxicity in these patients. Methods This prospective study was done at 16 centres in ten countries worldwide. Patients aged 0-40 years with chronic granulornatous disease were assessed and enrolled at the discretion of individual centres. Reduced-intensity conditioning consisted of high-dose fludarabine (30 mg/m2 [infants <9 kg 1-2 mg/kg]; one dose per day on days 8 to 3), serotherapy (anti-thymocyte globulin [10 mg/kg, one dose per day on days 4 to 1; or thymoglobuline 2-5 mg/kg, one dose per day on days 5 to 3]; or low-dose alerntuzurnab [<1 mg/kg on days 8 to 6]), and low-dose (50-72% of myeloablative dose) or targeted busulfan administration (recommended cumulative area under the curve: 45-65 mg/L xh). Busulfan was administered mainly intravenously and exceptionally orally from days 5 to 3. Intravenous busulfan was dosed according to weight-based recommendations and was administered in most centres (ten) twice daily over 4 h. Unmanipulated bone marrow or peripheral blood stem cells from HLA-matched relateddonors or HLA-9/10 or HLA-10/10 matched unrelated-donors were infused. The primary endpoints were overall survival and event-free survival (EFS), probabilities of overall survival and EFS at 2 years, incidence of acute and chronic GVHD, achievement of at least 90% myeloid donor chimerisrn, and incidence of graft failure after at least 6 months of follow-up. Findings 56 patients (median age 12-7 years; IQR 6-8-17-3) with chronic granulornatous disease were enrolled from June 15,2003, to Dec 15,2012.42 patients (75%) had high-risk features (ie, intractable infections and autoinflammation), 25(45%) were adolescents and young adults (age 14-39 years). 21 HLA-matched related-donor and 35 HLA-rnatched unrelated-donor transplants were done. Median time to engraftrnent was 19 days (IQR 16-22) for neutrophils and 21 days (IQR 16-25) for platelets. At median follow-up of 21 months (IQR 13-35) overall survival was 93% (52 of 56) and EFS was 89% (50 of 56). The 2-year probability of overall survival was 96% (95% CI 86-46-99.09) and of EFS was 91% (79-78-96-17). Graft-failure occurred in 5% (three of 56) of patients. The cumulative incidence of acute GVHD of grade III IV was 4% (two of 56) and of chronic graft-versus-host disease was 7% (four of 56). Stable (90%) myeloid donor chimerism was documented in 52 (93%) surviving patients. Interpretation This reduced-intensity conditioning regimen is safe and efficacious in high-risk patients with chronic granulomatous disease.
  • article 224 Citação(ões) na Scopus
    Long-term follow-up of IPEX syndrome patients after different therapeutic strategies: An international multicenter retrospective study
    (2018) BARZAGHI, Federica; HERNANDEZ, Laura Cristina Amaya; NEVEN, Benedicte; RICCI, Silvia; KUCUK, Zeynep Yesim; BLEESING, Jack J.; NADEMI, Zohreh; SLATTER, Mary Anne; ULLOA, Erlinda Rose; SHCHERBINA, Anna; ROPPELT, Anna; WORTH, Austen; SILVA, Juliana; AIUTI, Alessandro; MURGUIA-FAVELA, Luis; SPECKMANN, Carsten; CARNEIRO-SAMPAIO, Magda; FERNANDES, Juliana Folloni; BARIS, Safa; OZEN, Ahmet; KARAKOC-AYDINER, Elif; KIYKIM, Ayca; SCHULZ, Ansgar; STEINMANN, Sandra; NOTARANGELO, Lucia Dora; GAMBINERI, Eleonora; LIONETTI, Paolo; SHEARER, William Thomas; FORBES, Lisa R.; MARTINEZ, Caridad; MOSHOUS, Despina; BLANCHE, Stephane; FISHER, Alain; RUEMMELE, Frank M.; TISSANDIER, Come; OUACHEE-CHARDIN, Marie; RIEUX-LAUCAT, Frederic; CAVAZZANA, Marina; QASIM, Waseem; LUCARELLI, Barbarella; ALBERT, Michael H.; KOBAYASHI, Ichiro; ALONSO, Laura; HEREDIA, Cristina Diaz De; KANEGANE, Hirokazu; LAWITSCHKA, Anita; SEO, Jong Jin; GONZALEZ-VICENT, Marta; DIAZ, Miguel Angel; GOYAL, Rakesh Kumar; SAUER, Martin G.; YESILIPEK, Akif; KIM, Minsoo; YILMAZ-DEMIRDAG, Yesim; BHATIA, Monica; KHLEVNER, Julie; PADILLA, Erick J. Richmond; MARTINO, Silvana; MONTIN, Davide; NETH, Olaf; MOLINOS-QUINTANA, Agueda; VALVERDE-FERNANDEZ, Justo; BROIDES, Arnon; PINSK, Vered; BALLAUF, Antje; HAERYNCK, Filomeen; BORDON, Victoria; DHOOGE, Catharina; GARCIA-LLORET, Maria Laura; BREDIUS, Robbert G.; KAWAK, Krzysztof; HADDAD, Elie; SEIDEL, Markus Gerhard; DUCKERS, Gregor; PAI, Sung-Yun; DVORAK, Christopher C.; EHL, Stephan; LOCATELLI, Franco; GOLDMAN, Frederick; GENNERY, Andrew Richard; COWAN, Mort J.; RONCAROLO, Maria-Grazia; BACCHETTA, Rosa
    Background: Immunodysregulation polyendocrinopathy enteropathy x-linked(IPEX) syndrome is a monogenic autoimmune disease caused by FOXP3 mutations. Because it is a rare disease, the natural history and response to treatments, including allogeneic hematopoietic stem cell transplantation (HSCT) and immunosuppression (IS), have not been thoroughly examined. Objective: This analysis sought to evaluate disease onset, progression, and long-term outcome of the 2 main treatments in long-term IPEX survivors. Methods: Clinical histories of 96 patients with a genetically proven IPEX syndrome were collected from 38 institutions worldwide and retrospectively analyzed. To investigate possible factors suitable to predict the outcome, an organ involvement (OI) scoring system was developed. Results: We confirm neonatal onset with enteropathy, type 1 diabetes, and eczema. In addition, we found less common manifestations in delayed onset patients or during disease evolution. There is no correlation between the site of mutation and the disease course or outcome, and the same genotype can present with variable phenotypes. HSCT patients (n = 58) had a median follow-up of 2.7 years (range, 1 week-15 years). Patients receiving chronic IS (n 5 34) had a median follow-up of 4 years (range, 2 months-25 years). The overall survival after HSCT was 73.2% (95% CI, 59.4-83.0) and after IS was 65.1% (95% CI, 62.8-95.8). The pretreatment OI score was the only significant predictor of overall survival after transplant (P = .035) but not under IS. Conclusions: Patients receiving chronic IS were hampered by disease recurrence or complications, impacting long-term.disease-free survival. When performed in patients with a low OI score, HSCT resulted in disease resolution with better quality of life, independent of age, donor source, or conditioning regimen.
  • article 93 Citação(ões) na Scopus
    Transplantation in patients with SCID: mismatched related stem cells or unrelated cord blood?
    (2012) FERNANDES, Juliana F.; ROCHA, Vanderson; LABOPIN, Myriam; NEVEN, Benedicte; MOSHOUS, Despina; GENNERY, Andrew R.; FRIEDRICH, Wilhelm; PORTA, Fulvio; HEREDIA, Cristina Diaz de; WALL, Donna; BERTRAND, Yves; VEYS, Paul; SLATTER, Mary; SCHULZ, Ansgar; CHAN, Ka Wah; GRIMLEY, Michael; AYAS, Mouhab; GUNGOR, Tayfun; EBELL, Wolfram; BONFIM, Carmem; KALWAK, Krzysztof; TAUPIN, Pierre; BLANCHE, Stephane; GASPAR, H. Bobby; LANDAIS, Paul; FISCHER, Alain; GLUCKMAN, Eliane; CAVAZZANA-CALVO, Marina
    Pediatric patients with SCID constitute medical emergencies. In the absence of an HLA-identical hematopoietic stem cell (HSC) donor, mismatched related-donor transplantation (MMRDT) or unrelated-donor umbilical cord blood transplantation (UCBT) are valuable treatment options. To help transplantation centers choose the best treatment option, we retrospectively compared outcomes after 175 MMRDTs and 74 UCBTs in patients with SCID or Omenn syndrome. Median follow-up time was 83 months and 58 months for UCBT and MMRDT, respectively. Most UCB recipients received a myeloablative conditioning regimen; most MMRDT recipients did not. UCB recipients presented a higher frequency of complete donor chimerism (P = .04) and faster total lymphocyte count recovery (P = .04) without any statistically significance with the preparative regimen they received. The MMRDT and UCBT groups did not differ in terms of T-cell engraftment, CD4(+) and CD3(+) cell recoveries, while Ig replacement therapy was discontinued sooner after UCBT (adjusted P = .02). There was a trend toward a greater incidence of grades II-IV acute GVHD (P = .06) and more chronic GVHD (P = .03) after UCBT. The estimated 5-year overall survival rates were 62% +/- 4% after MMRDT and 57% +/- 6% after UCBT. For children with SCID and no HLA-identical sibling donor, both UCBT and MMRDT represent available HSC sources for transplantation with quite similar outcomes. (Blood. 2012;119(12):2949-2955)