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
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conferenceObject 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, Nelson- 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.
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, Carmem- 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, NelsonAllogeneic 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.
conferenceObject Salvage Haploidentical Stem Cell Transplantation with Post-Transplant Cyclophosphamide for graft failure in children with non-malignant diseases(2018) FERNANDES, Juliana Folloni; RIBEIRO, Andreza Alice Feitosa; KERBAUY, Fabio Rodrigues; MANTOVANI, Luiz Fernando; NETTO, Gabriele Zamperlini; VENANCIO, Angela Mandelli; SILVA, Cinthya Correa; KONDO, Andrea Tiemi; COLLASSANTI, Maria Dulce Silveira; ZANICHELLI, Maria Aparecida; VINCE, Carolina Sgarioni Camargo; BRUMATTI, Melina; ODONE FILHO, Vicente; HAMERSCHLAK, Nelson- 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, CarmemThe 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.
- 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, RosaBackground: 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.
conferenceObject Outcomes after Hematopoietic Cell Transplantation (HCT) in Brazil for Children and Adolescents with Non-Malignant Diseases: A Multicenter Study on Behalf on the Brazil-Seattle Consortium Study Group(2018) BONFIM, Carmem; FRANCO, Simone; COLTURATO, Vergilio A. R.; FERNANDES, Juliana Folloni; ZECCHIN, Victor; SEBER, Adriana; DARRIGO, Luiz Guilherme; DAUDT, Liane Esteves; ARCURI, Leonardo Javier; FLOWERS, Mary E.