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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Resultados de Busca
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- Albuterol via metered-dose inhaler in children: Lower doses are effective, and higher doses are safe(2016) MUCHAO, Fabio Pereira; SOUZA, Juliana Miguita e; TORRES, Helida Conceicao Cavalcante; LALIBERA, Isabella Batista De; SOUZA, Andrea Vieira de; RODRIGUES, Joaquim Carlos; SCHVARTSMAN, Claudio; SILVA FILHO, Luiz Vicente Ribeiro Ferreira daBackgroundThe ideal dosing of albuterol via metered-dose inhalers for acute childhood asthma is not well established. We hypothesized that greater doses of albuterol would result in less time in the hospital and lower admission rates. MethodsThis was a randomized controlled double-blind multicenter study, conducted in emergency rooms (ER). We included patients with 2-17 years old with moderate to severe acute asthma (Pediatric Respiratory Assessment Measure, PRAM, score 5). Dosages administered during the first hour included: 6 (up to 25kg) or 12 puffs (>25kg) in the control group and 9 (up to 15kg), 12 (>15-20kg), 15 (>20-25kg), or 18 puffs (>25kg) in the study group. Several efficacy (changes in PRAM score, pulse oximetry, and FEV1, length of stay, and admission rates) and safety (albuterol plasma levels, heart rate, serum potassium, glucose and bicarbonate levels, EKG, and tremor rates) outcome measures were assessed. ResultsWe included 119 patients with similar baseline conditions, and no significant differences were observed between groups in the length of stay (P=0.48) or admission rate (P=0.55). No significant differences were observed in FEV1, PRAM score, and pulse oximetry changes after 1hr and at discharge or admission. No significant differences were observed in safety outcomes between groups. ConclusionsHigher albuterol dosage regimens did not result in lower admission rate or shorter length of stay in the ER, but showed similar safety profile for children with moderate to severe acute asthma. Pediatr Pulmonol. 2016;51:1122-1130. (c) 2016 Wiley Periodicals, Inc.
- A new insight into CFTR allele frequency in Brazil through next generation sequencing(2017) NUNES, Luisa M.; RIBEIRO, Roberto; NIEWIADONSKI, Vivian D. T.; SABINO, Ester; YAMAMOTO, Guilherme L.; BERTOLA, Debora R.; GABURO, Nelson; SILVA FILHO, Luiz Vicente R. F. daBackgroundAs of 2013, fewer than 20% of patients in the Brazilian CF Registry had two CFTR mutations identified. The aim of this study was to sequence the coding region of the CFTR in Brazilian CF patients and determine the frequency of mutations in this cohort. MethodsPatients with CF and those with suspected atypical CF or CFTR-related disorders were invited to enroll. Total DNA was extracted from blood samples, quantified, and purified. Library preparation was performed using Ion Xpress Plus gDNA and Amplicon Library preparation kits (Life Technologies), as well as sequencing using the Ion Torrent platform (Life Technologies). ResultsA total of 141 patients were enrolled, and 45 mutations were identified. Among 126 CF patients, we identified mutations in 97.2% of alleles. The three most common mutations were F508del, G542X, and 3120+1G->A. Five novel pathogenic mutations were also identified. ConclusionsNext generation sequencing (NGS) allowed the identification of mutations in most CF alleles and confirmed allelic heterogeneity in our population.