LUIZ VICENTE RIBEIRO FERREIRA DA SILVA FILHO
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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bookPart Crise asmática(2023) SEVERINI, Rafael da Silva Giannasi; SILVA FILHO, Luiz Vicente Ribeiro Ferreira da- Use of elexacaftor plus tezacaftor plus ivacaftor in individuals with cystic fibrosis and at least one F508del allele: a systematic review and meta-analysis(2023) SILVA FILHO, Luiz Vicente Ribeiro Ferreira da; ATHANAZIO, Rodrigo Abensur; TONON, Carolina Rodrigues; FERREIRA, Juliana Carvalho; TANNI, Suzana EricoObjective: To evaluate the effect of treatment with the combination of three cystic fibrosis transmembrane conductance regulator (CFTR) modulators-elexacaftor+tezac aftor+ivacaftor (ETI)-on important clinical endpoints in individuals with cystic fibrosis. Methods: This was a systematic review and meta-analysis of randomized clinical trials that compared the use of ETI in individuals with CF and at least one F508del allele with that of placebo or with an active comparator such as other combinations of CFTR modulators, following the Preferred Reporting Items for Systematic Reviews and MetaAnalyses (PRISMA) recommendations and the Patients of interest, Intervention to be studied, Comparison of interventions, and Outcome of interest (PICO) methodology. We searched the following databases: MEDLINE, EMBASE, Cochrane Central Register of Controlled Trials, and ClinicalTrials.gov from their inception to December 26th, 2022. The risk of bias was assessed using the Cochrane risk-of-bias tool, and the quality of evidence was based on the Grading of Recommendations Assessment, Development and Evaluation (GRADE). Results: We retrieved 54 studies in the primary search. Of these, 6 met the inclusion criteria and were analyzed (1,127 patients; 577 and 550 in the intervention and control groups, respectively). The meta-analysis revealed that the use of ETI increased FEV 1% [risk difference (RD), +10.47%; 95% CI, 6.88-14.06], reduced the number of acute pulmonary exacerbations (RD, -0.16; 95% CI, -0.28 to -0.04), and improved quality of life (RD, +14.93; 95% CI, 9.98-19.89) and BMI (RD, +1.07 kg/m(2); 95% CI, 0.90-1.25). Adverse events did not differ between groups (RD, -0.03; 95% CI, -0.08 to 0.01), and none of the studies reported deaths. Conclusions: Our findings demonstrate that ETI treatment substantially improves clinically significant, patientcentered outcomes.
- Quality of anthropometric data measured in children and adolescents with cystic fibrosis: a scoping review(2023) ESCALDELAI, Fernanda Martins Dias; SILVA FILHO, Luiz Vicente Ribeiro Ferreira da; NERI, Lenycia de Cassya Lopes; BERGAMASCHI, Denise PimentelObjective: This study aimed to identify methodological aspects involved in determining anthropometric measurements among studies assessing the nutritional status of individuals with cystic Methods: A search of the literature was performed on MEDLINE via Pubmed, Embase, and Web of Science databases. The population comprised children and adolescents with CF. Observational studies and clinical trials using anthropometric and body composition measures and indices determined by dualenergy X-ray absorptiometry (DXA) and bioelectrical impedance assessment (BIA) were included. Use of a standardized procedure for data collection was defined when details on the instruments and their calibration were given, the measuring procedures were described, and when it was clear measures had been determined by a trained team, or the use of an anthropometric reference manual was cited. Data extracted were expressed as absolute and relative frequencies. Results: A total of 32 articles were included, and a total of 233 measures or indices were observed. The most frequently used measures were body mass index (kg/m2; 35%), weight (kg; 33%), and height (cm; 33%). Among the 28 studies that used anthropometric measures, 21 (75%) provided a complete or partial description of the measurement instruments used, 3 (11%) reported information on equipment calibration, 10 (36%) indicated the measurement procedures employed by assessors, and 2 (7%) stated a trained team had carried out the measurements. Conclusions: The poor description of measuring procedures precluded a meaningful evaluation of data quality. Scientific ensure quality in collecting and fully presenting data.
- Brazilian guidelines for the pharmacological treatment of the pulmonary symptoms of cystic fibrosis. Official document of the Sociedade Brasileira de Pneumologia e Tisiologia (SBPT, Brazilian Thoracic Association)(2023) ATHANAZIO, Rodrigo Abensur; TANNI, Suzana Erico; FERREIRA, Juliana; DALCIN, Paulo de Tarso Roth; FUCCIO, Marcelo B. de; ESPOSITO, Concetta; CANAN, Mariane Goncalves Martynychen; COELHO, Liana Sousa; FIRMIDA, Monica de Cassia; ALMEIDA, Marina Buarque de; MAROSTICA, Paulo Jose Cauduro; MONTE, Luciana de Freitas Velloso; SOUZA, Edna Lucia; PINTO, Leonardo Araujo; RACHED, Samia Zahi; OLIVEIRA, Veronica Stasiak Bednarczuk de; RIEDI, Carlos Antonio; SILVA FILHO, Luiz Vicente Ribeiro Ferreira daCystic fibrosis (CF) is a genetic disease that results in dysfunction of the CF transmembrane conductance regulator (CFTR) protein, which is a chloride and bicarbonate channel expressed in the apical portion of epithelial cells of various organs. Dysfunction of that protein results in diverse clinical manifestations, primarily involving the respiratory and gastrointestinal systems, impairing quality of life and reducing life expectancy. Although CF is still an incurable pathology, the therapeutic and prognostic perspectives are now totally different and much more favorable. The purpose of these guidelines is to define evidence- based recommendations regarding the use of pharmacological agents in the treatment of the pulmonary symptoms of CF in Brazil. Questions in the Patients of interest, Intervention to be studied, Comparison of interventions, and Outcome of interest (PICO) format were employed to address aspects related to the use of modulators of this protein (ivacaftor, lumacaftor+ivacaftor, and tezacaftor+ivacaftor), use of dornase alfa, eradication therapy and chronic suppression of Pseudomonas aeruginosa, and eradication of methicillin-resistant Staphylococcus aureus and Burkholderia cepacia complex. To formulate the PICO questions, a group of Brazilian specialists was assembled and a systematic review was carried out on the themes, with meta-analysis when applicable. The results obtained were analyzed in terms of the strength of the evidence compiled, the recommendations being devised by employing the GRADE approach. We believe that these guidelines represent a major advance to be incorporated into the approach to patients with CF, mainly aiming to favor the management of the disease, and could become an auxiliary tool in the definition of public policies related to CF.
bookPart Teste do suor e outros recursos diagnósticos para a fibrose cística(2023) ADDE, Fabíola Villac; SILVA FILHO, Luiz Vicente Ribeiro Ferreira daconferenceObject The Impact of Neonatal Screening on the Pulmonary Function of School-aged Children with Cystic Fibrosis(2023) MUNOZ, T. A. Soria; AMORAS, A. Da Silva; SARUHASHI, R. Porto Avary de Campos; SILVA, R. Galdino Da; GOMES, I. M. Forti; PEREIRA, C. M. Lourinho; ARANHA NETTO, N.; TANAKA, S. Onoda Tomikawa; NAKAIE, C. M. Aversa; RODRIGUES, J. C.; V, L. Ribeiro Ferreira Silva FilhoconferenceObject Chest Computed Tomography for Infants and Preschoolers with Cystic Fibrosis: When to Indicate and What are the Therapeutic Implications?(2023) AMORAS, A. Da Silva; MUNOZ, T. A. Soria; SILVA, R. Galdino Da; CAMPOS, R. Porto Avary de; GOMES, I. M. Forti; PEREIRA, C. M. Lourinho; ARANHA NETTO, N.; TANAKA, S. Onoda Tomikawa; NAKAIE, C. M. Versa; RODRIGUES, J. C.; SILVA FILHO, L. V. Ribeiro Ferreira Da- Five-year performance analysis of a cystic fibrosis newborn screening program in northeastern Brazil(2023) GODOY, Carolina; PAIXAO, Danielle C.; BOA-SORTE, Ney C. A.; AMORIM, Tatiana; SILVA FILHO, Luiz Vicente R. F. da; SOUZA, Edna LuciaObjective: To analyze the performance of the cystic fibrosis (CF) newborn screening (NBS) pro-gram over its first five years in a Brazilian northeastern state. Method: A population-based study using a screening algorithm based on immunoreactive tryp-sinogen (IRT)/IRT. Data were retrieved from the state referral screening center registry. The pro-gram performance was evaluated using descriptive indicators such as the results of an active search, coverage, newborn's age at the time of blood sampling, the time between sample collec-tion and its arrival at the laboratory, and the child's age at diagnosis of disease. Results: The public CF screening program covered 82.6% of the 1,017,576 births that occurred, with an accumulated five-year incidence of 1:20,767 live births. The median (25th-75th) age at diagnosis was 3.5 (2.3-7.3) months. The sampling before 7 days of life for the first IRT (IRT1) increased between 2013 and 2017 from 42.2 to 48.3%. Around 5% of IRT1 samples and 30% of the second samples were collected after 30 days of life. In the first and second stages of screening, 23.6% and 19.9% of the infants, respectively, were lost to follow-up. In both stages of screening, the samples were retained at the health units for a median (25th -75th) of 9.0 (7.0-13.0) days. Conclusions: The coverage by the CF-NBS program was satisfactory as compared to other Brazil-ian state rates and the percentage of IRT1 samples collected within the first week of life increased progressively. However, time of samples retention at the health units, inappropriate sampling, inherent methodological problems, and loss of follow-up need to improve.(c) 2022 Sociedade Brasileira de Pediatria.