FABIOLA VILLAC ADDE
Projetos de Pesquisa
Unidades Organizacionais
LIM/36 - Laboratório de Pediatria Clínica, Hospital das Clínicas, Faculdade de Medicina
10 resultados
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- A first-year dornase alfa treatment impact on clinical parameters of patients with cystic fibrosis: the Brazilian cystic fibrosis multicenter study(2013) ROZOV, Tatiana; SILVA, Fernando Antonio A. e; SANTANA, Maria Angelica; ADDE, Fabiola Villac; MENDES, Rita HeloisaOBJECTIVE: To describe the clinical impact of the first year treatment with dornase alfa, according to age groups, in a cohort of Brazilian Cystic Fibrosis (CF) patients. METHODS: The data on 152 eligible patients, from 16 CF reference centers, that answered the medical questionnaires and performed laboratory tests at baseline (T0), and at six (T2) and 12 (T4) months after dornase alfa initiation, were analyzed. Three age groups were assessed: six to 11, 12 to 13, and >14 years. Pulmonary tests, airway microbiology, emergency room visits, hospitalizations, emergency and routine treatments were evaluated. Student's t-test, chi-square test and analysis of variance were used when appropriated. RESULTS: Routine treatments were based on respiratory physical therapy, regular exercises, pancreatic enzymes, vitamins, bronchodilators, corticosteroids, and antibiotics. In the six months prior the study (T0 phase), hospitalizations for pulmonary exacerbations occurred in 38.0, 10.0 and 61.4% in the three age groups, respectively. After one year of intervention, there was a significant reduction in the number of emergency room visits in the six to 11 years group. There were no significant changes in forced expiratory volume in one second (VEF1), in forced vital capacity (FVC), in oxygen saturation (SpO2), and in Tiffenau index for all age groups. A significant improvement in Shwachman-Kulczychi score was observed in the older group. In the last six months of therapy, chronic or intermittent colonization by P. aeruginosa was detected in 75.0, 71.4 and 62.5% of the studied groups, respectively, while S. aureus colonization was identified in 68.6, 66.6 and 41.9% of the cases. CONCLUSIONS: The treatment with dornase alfa promoted the maintenance of pulmonary function parameters and was associated with a significant reduction of emergency room visits due to pulmonary exacerbations in the six to 11 years age group, with better clinical scores in the >14 age group, one year after the intervention.
- Sweat conductivity: An accurate diagnostic test for cystic fibrosis?(2014) MATTAR, Ana Claudia Veras; LEONE, Claudio; RODRIGUES, Joaquim Carlos; ADDE, Fabiola VillacBackground: Sweat chloride test is the gold standard test for cystic fibrosis (CF) diagnosis. Sweat conductivity is widely used although still considered a screening test. Methods: This was a prospective, cross-sectional, diagnostic research conducted at the laboratory of the Instituto da Crianca of the Hospital das Clinicas, Sao Paulo, Brazil. Sweat chloride (quantitative pilocarpine iontophoresis) and sweat conductivity tests were simultaneously performed in patients referred for a sweat test between March 2007 and October 2008. Conductivity and chloride cut-off values used to rule out or diagnose CF were <75 and >= 90 mmol/L and <60 and >= 60 mmol/L, respectively. The ROC curve method was used to calculate the sensitivity, specificity, positive (PPV) and negative predictive value (NPV), as well as the respective 95% confidence intervals and to calculate the area under the curve for both tests. The kappa coefficient was used to evaluate agreement between the tests. Results: Both tests were performed in 738 children, and CF was ruled out in 714 subjects; the median sweat chloride and conductivity values were 11 and 25 mmol/L in these populations, respectively. Twenty-four patients who had received a diagnosis of CF presented median sweat chloride and conductivity values of 87 and 103 mmol/L, respectively. Conductivity values' above 90 mmol/L had 83.3% sensitivity, 99.7% specificity, 90.9% PPV and 99.4% NPV to diagnose CF. The best conductivity cut-off value to exclude CF was <75 mmol/L. Good agreement was observed between the tests (kappa: 0.934). Conclusions: The sweat conductivity test yielded a high degree of diagnostic accuracy and it showed good agreement with sweat chloride. We suggest that it should play a role as a diagnostic test for CF in the near future.
- Six minute walk test Z score: Correlations with cystic fibrosis severity markers(2012) STOLLAR, Fabiola; RODRIGUES, Joaquim C.; CUNHA, Maristela T.; LEONE, Claudio; ADDE, Fabiola VillacBackground: The six-minute-walk-test (6MWT) has been increasingly used in cystic fibrosis (CF) patients. However, few studies in children have correlated 6MWT with current parameters used to evaluate CF severity. Moreover, no study transformed the values of distance walked from meters into Z scores to avoid bias like age and gender, which are sources of 6MWT variability. Methods: A cross-sectional descriptive study was performed to analyze the correlations (Spearman) among forced expiratory volume in one second (FEV1), body mass index (BMI), chest radiography (CXR), chest tomography (CT), and 6MWT Z score (Z-6MWT). Clinically stable CF patients, aged 6-21 years, were included. Results: 34 patients, 14F/20M, mean age 12.1 +/- 4.0 years were studied. The mean Z-6MWT was -1.1 +/- 1.106. The following correlations versus Z-6MWT were found: FEV1 (r=0.59, r(2)=0.32, p=0.0002), BMI Z score (r=0.42, r(2)=0.17, p=0.013), CXR (r=0.34, r(2)=0.15, p=0.0472) and CT (r=-0.45, r(2)=0.23, p=0.0073). Conclusions: In conclusion there was a significant, but poor, correlation between the six minute walk test Z score and the cystic fibrosis severity markers currently in use.
- Effectiveness of a multistep Pseudomonas aeruginosa eradication treatment protocol in children with cystic fibrosis in Brazil(2020) RIQUENA, Barbara; SILVA FILHO, Luiz Vicente Ribeiro Ferreira da; NAKAIE, Cleyde Myriam Aversa; ALMEIDA, Marina Buarque de; RODRIGUES, Joaquim Carlos; ADDE, Fabiola VillacObjective: Although various strategies have been proposed for eradicating Pseudomonas aeruginosa in patients with cystic fibrosis (CF), only a few employ multistep treatment in children colonized by that pathogen for the first time. The aim of this study was to describe the effectiveness of a three-phase eradication protocol, initiated after the first isolation of P. aeruginosa, in children with CF in Brazil. Methods: This was a retrospective real-life study in which we reviewed the medical records of pediatric CF patients in whom the eradication protocol was applied between June of 2004 and December of 2012. The three-phase protocol was guided by positive cultures for P. aeruginosa in airway secretions, and the treatment consisted of inhaled colistimethate and oral ciprofloxacin. Success rates were assessed after each phase, as well as cumulatively. Results: During the study period, 47 episodes of P. aeruginosa colonization, in 29 patients, were eligible for eradication. Among the 29 patients, the median age was 2.7 years, 17 (59%) were male, and 19 (65%) had at least one F508del allele. All 29 patients completed the first phase of the protocol, whereas only 12 and 6 completed the second and third phases, respectively. Success rates for eradication in the three treatment phases were 58.6% (95% CI: 40.7-74.5), 50.0% (95% CI: 25.4-74.6), and 66.7% (95% CI: 30.0-90.3), respectively. The cumulative success rate was 93.1% (95% CI: 78.0-98.1). Treatment failure in all three phases occurred in only 2 patients. Conclusions: In this sample of patients, the multistep eradication protocol was effective and had a high success rate.
conferenceObject EARLY PSEUDOMONAS AERUGINOSA COLONIZATION IN CYSTIC FIBROSIS: LONG TERM RESULTS OF AN ERADICATION PROTOCOL(2016) ADDE, Fabiola Villac; RIQUENA, Barbara; SILVA FILHO, Luiz Vicente R. F.; NAKAIE, Cleyde M. A.; ALMEIDA, Marina B.; RODRIGUES, Joaquim C.- Brazilian guidelines for the diagnosis and treatment of cystic fibrosis(2017) ATHANAZIO, Rodrigo Abensur; SILVA FILHO, Luiz Vicente Ribeiro Ferreira da; VERGARA, Alberto Andrade; RIBEIRO, Antonio Fernando; RIEDI, Carlos Antonio; PROCIANOY, Elenara da Fonseca Andrade; ADDE, Fabiola Villac; REIS, Francisco Jose Caldeira; RIBEIRO, Jose Dirceu; TORRES, Lidia Alice; FUCCIO, Marcelo Bicalho de; EPIFANIO, Matias; FIRMIDA, Monica de Cassia; DAMACENO, Neiva; LUDWIG-NETO, Norberto; MAROSTICE, Paulo Jose Cauduro; RACHED, Sarnia Zahi; MELO, Suzana Fonseca de Oliveira
conferenceObject RARE CFTR GENE MUTATIONS: IS IT CF OR NOT?(2016) ADDE, Fabiola Villac; RASKIN, Salmo- Shwachman-Kulczycki score still useful to monitor cystic fibrosis severity(2011) STOLLAR, Fabiola; ADDE, Fabiola Villac; CUNHA, Maristela T.; LEONE, Claudio; RODRIGUES, Joaquim C.INTRODUCTION: The Shwachman-Kulczycki score was the first scoring system used in cystic fibrosis to assess disease severity. Despite its subjectivity, it is still widely used. OBJECTIVE: To study correlations among forced expiratory volume in one second (FEV(1)), chest radiography, chest computed tomography, 6-minute walk test, and Shwachman-Kulczycki score in patients with cystic fibrosis and to test whether the Shwachman-Kulczycki score is still useful in monitoring the severity of the disease. METHODS: A cross-sectional prospective study was performed to analyze the correlations (Spearman). Patients with clinically stable cystic fibrosis, aged 3-21 years, were included. RESULTS: 43 patients, 19F/24M, mean age 10.5 +/- 4.7 years, with a median Shwachman-Kulczycki score of 70 were studied. The median Brasfield and Bhalla scores were 17 and 10, respectively. The mean Z score for the 6-minute walk test was -1.1 +/- 1.106 and the mean FEV(1) was 59 +/- 26 (as percentage of predicted values). The following significant correlations versus the Shwachman-Kulczycki score were found: FEV(1) (r = 0.76), 6-minute walk test (r = 0.71), chest radiography (r = 0.71) and chest computed tomography (r = -0.78). When patients were divided according to FEV(1), a statistically significantly correlation with the Shwachman-Kulczycki score was found only in patients with FEV(1) <70% (r = 0.67). CONCLUSIONS: The Shwachman-Kulczycki score remains an useful tool for monitoring the severity of cystic fibrosis, adequately reflecting the functional impairment and chest radiography and tomography changes, especially in patients with greater impairment of lung function. When assessing patients with mild lung disease its limitations should be considered and its usefulness in such patients should be evaluated in larger populations.
- Indications for lung resection surgery and lung transplant in South American children with cystic fibrosis(2018) ADDE, Fabiola Villac; CAMPOS, Silvia Vidal; TEIXEIRA, Ricardo Henrique de Oliveira Braga; RODRIGUES, Joaquim CarlosThe current available literature evaluating lung resection surgery and lung transplantation in children with cystic fibrosis (CF) was reviewed through a PubMed search and references from selected studies were additionally included. Pulmonary resections, i.e. lobectomy, segmentectomy, and pneumonectomy, are seldom performed in CF. The main indications, in patients with a forced expiratory volume in 1 second (FEV1) that is greater than 30% predicted, are localized bronchiectasis/atelectasis, severe hemoptysis, and bronchopleural fistula refractory to medical management. The potential benefits are decreased symptoms and pulmonary exacerbations, and an improved quality of life. Pre and postoperative intensive care is mandatory for surgical candidates. The risk of death should be taken into account when the procedure is considered. Selection for lung transplantation (LTx) candidates in children with CF in South America follows the International Society for Heart and Lung Transplantation (ISHLT) criteria. When compared to adults with CF, a poorer survival rate after LTx in children with CF has been observed in the literature, as well as in our LTx center in Brazil, reasons for which are still unknown. The main complications after LTx in children are early and late acute rejection, and infections. LTx is a therapeutic option for eligible children with CF, fulfilling the lung transplant candidacy criteria, as post-transplant survival rates are increasingly improving due to better management of the transplanted patient. (C) 2017 Published by Elsevier Ltd.
- Follow-up on pediatric patients with bronchiolitis obliterans treated with corticosteroid pulse therapy(2014) TOMIKAWA, Silvia Onoda; ADDE, Fabiola Villac; SILVA FILHO, Luiz Vicente Ribeiro Ferreira da; LEONE, Claudio; RODRIGUES, Joaquim CarlosBackground: Bronchiolitis obliterans (BO) is a rare but severe disease in children. Currently, there is no consensus on the treatment for BO with respect to the systemic use of corticosteroids. Here we report on the follow-up of children with a diagnosis of BO who were treated with corticosteroid pulse therapy. Methods: Forty patients fulfilling the BO diagnosis criteria were treated with methylprednisolone pulse therapy in monthly cycles until clinical improvement. After the pulse therapy began, we analyzed the clinical and laboratory data at intervals. Statistical analyses were performed using non-parametric tests to compare repeated measures (Friedman, Wilcoxon) or paired nominal data (McNemar) (a = 5%). Results: The frequency of wheezing exacerbations and hospitalizations was reduced (p = 0.0042 and p < 0.0001, respectively) and oxygen saturation improved (p = 0.0002) in the pulse therapy-treated patients. Prolonged oral corticosteroid therapy was discontinued in 83% of these patients. The mean Z-score length for age improved from -1.08 to -0.63, and the mean Z-score weight for age improved from -0.91 to -0.59. The adverse effects during the infusion were temporary and none were serious. Conclusions: Our data suggest that pulse corticotherapy could be a safe alternative to prolonged systemic oral corticotherapy in children with BO, thus minimizing the adverse effects of the oral therapy. New prospective controlled studies are required to confirm this proposition.