Fernando Antônio A. e Silva

Glucose Intolerance in Patients With Cystic Fibrosis: Sex-Based Differences in Clinical Score, Pulmonary Function, Radiograph Score, and 6-Minute Walk Test

OBJECTIVE: To determine the sex-specific relationships between glucose intolerance and Shwachman-Kulczycki clinical score, lung function, Brasfield chest-radiograph score, and exercise capacity in patients with cystic fibrosis (CF). METHODS: We used a cross-sectional study design and included CF patients ≥ 10 years old. All patients had clinical and nutritional evaluation, oral glucose tolerance test, spirometry, chest radiograph, and 6-min walk test (6MWT). Patients were classified as having normal glucose tolerance, impaired glucose tolerance, or CF-related diabetes mellitus. RESULTS: We included 88 patients: 59 with normal glucose tolerance, 15 with impaired glucose tolerance, and 14 with CF-related diabetes. Shwachman-Kulczycki clinical score (P = .04), at-rest SpO2 (P = .001), SpO2 difference before versus after 6MWT (P = .001), and Brasfield chest-radiograph score (P = .01) were significantly lower in the impaired-glucose-tolerance group than in the normal-glucose-tolerance group, but did not differ from the CF-related-diabetes group. In female patients only, percent-of-predicted FEV1 was significantly lower in the impaired-glucose-tolerance group than in the normal-glucose-tolerance group (P = .02), but did not differ from the CF-related-diabetes group (P = .10). There was a significant relationship between glucose intolerance and sex when clinical score, 6-min walk distance, FEV1, and radiograph score were combined in a multivariate analysis. CONCLUSIONS: In patients with CF, glucose intolerance was associated with poor clinical score, lower at-rest SpO2, greater SpO2 difference before versus after 6MWT, poor lung function, and lower radiograph score. Overall, multivariate analysis indicated poorer performance in the latter variables in female patients with glucose intolerance than in male patients with glucose intolerance.

A first-year dornase alfa treatment impact on clinical parameters of patients with cystic fibrosis: the Brazilian cystic fibrosis multicenter study

OBJETIVO: Relatar o impacto clinico do primeiro ano de tratamento com dornase alfa de acordo com a faixa etaria, numa coorte de pacientes brasileiros com fibrose cistica (FC). METODOS: O presente estudo analisou dados de 152 pacientes elegiveis, de 16 centros de referencia para FC, os quais responderam aos questionarios clinicos e realizaram testes laboratoriais, ao inicio do tratamento com dornase alfa (T0) e apos seis (T2) e 12 (T4) meses da intervencao. Analisaram-se tres grupos etarios: seis a 11, 12 a 13 e >14 anos de idade. Avaliaram-se os testes pulmonares, a microbiologia de vias aereas, os atendimentos de emergencia, as hospitalizacoes e os tratamentos emergenciais e rotineiros. O teste t de Student, o qui-quadrado e a analise de variância foram usados quando pertinentes. RESULTADOS: O tratamento baseou-se em fisioterapia respiratoria, exercicios regulares, enzimas pancreaticas, vitaminas, broncodilatadores, corticosteroides e antibioticos. Nos seis meses anteriores ao estudo (fase T0), as hospitalizacoes por exacerbacao pulmonar ocorreram em 38,0, 10,0 e 61,4%, respectivamente para as tres faixas etarias analisadas. No grupo de seis a 11 anos, houve reducao significativa de atendimentos de emergencia apos um ano de tratamento. Nao houve modificacoes significativas de volume expiratorio forcado no primeiro segundo (VEF1), capacidade vital forcada (CVF), saturacao de oxigenio (SpO)2 e indice de Tiffeneau em todos os grupos. O escore de Shwachman-Kulczychi melhorou significativamente no grupo de mais idade. Nos ultimos seis meses de tratamento, a colonizacao cronica ou intermitente por P.aeruginosa foi detectada em 75,0, 71,4 e 62,5%, respectivamente, enquanto a colonizacao por S.aureus ocorreu em 68,6, 66,6 e 41,9% dos casos em cada grupo etario. CONCLUSOES: A intervencao com dornase alfa resultou em manutencao dos parâmetros pulmonares e associou-se a reducao significativa de visitas a emergencia por exacerbacao pulmonar no grupo de seis a 11 anos de idade, com melhora do escore clinico no grupo >14 anos de idade ao final de um ano de estudo.OBJECTIVE: 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. Students 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.

Sleep-disordered breathing in patients with cystic fibrosis

Abstract Objective: To test the hypothesis that disease severity in patients with cystic fibrosis (CF) is correlated with an increased risk of sleep apnea. Methods: A total of 34 CF patients underwent clinical and functional evaluation, as well as portable polysomnography, spirometry, and determination of IL-1β levels. Results: Mean apnea-hypopnea index (AHI), SpO2 on room air, and Epworth Sleepiness Scale score were 4.8 ± 2.6, 95.9 ± 1.9%, and 7.6 ± 3.8 points, respectively. Of the 34 patients, 19 were well-nourished, 6 were at nutritional risk, and 9 were malnourished. In the multivariate model to predict the AHI, the following variables remained significant: nutritional status (β = −0.386; p = 0.014); SpO2 (β = −0.453; p = 0.005), and the Epworth Sleepiness Scale score (β = 0.429; p = 0.006). The model explained 51% of the variation in the AHI. Conclusions: The major determinants of sleep apnea were nutritional status, SpO2, and daytime sleepiness. This knowledge not only provides an opportunity to define the clinical risk of having sleep apnea but also creates an avenue for the treatment and prevention of the disease.

El impacto de primer año de tratamiento con dornasa alfa en los parámetros clínicos de pacientes con fibrosis quística: resultado de estudio brasileño multicéntrico

OBJETIVO: Relatar o impacto clinico do primeiro ano de tratamento com dornase alfa de acordo com a faixa etaria, numa coorte de pacientes brasileiros com fibrose cistica (FC). METODOS: O presente estudo analisou dados de 152 pacientes elegiveis, de 16 centros de referencia para FC, os quais responderam aos questionarios clinicos e realizaram testes laboratoriais, ao inicio do tratamento com dornase alfa (T0) e apos seis (T2) e 12 (T4) meses da intervencao. Analisaram-se tres grupos etarios: seis a 11, 12 a 13 e >14 anos de idade. Avaliaram-se os testes pulmonares, a microbiologia de vias aereas, os atendimentos de emergencia, as hospitalizacoes e os tratamentos emergenciais e rotineiros. O teste t de Student, o qui-quadrado e a analise de variância foram usados quando pertinentes. RESULTADOS: O tratamento baseou-se em fisioterapia respiratoria, exercicios regulares, enzimas pancreaticas, vitaminas, broncodilatadores, corticosteroides e antibioticos. Nos seis meses anteriores ao estudo (fase T0), as hospitalizacoes por exacerbacao pulmonar ocorreram em 38,0, 10,0 e 61,4%, respectivamente para as tres faixas etarias analisadas. No grupo de seis a 11 anos, houve reducao significativa de atendimentos de emergencia apos um ano de tratamento. Nao houve modificacoes significativas de volume expiratorio forcado no primeiro segundo (VEF1), capacidade vital forcada (CVF), saturacao de oxigenio (SpO)2 e indice de Tiffeneau em todos os grupos. O escore de Shwachman-Kulczychi melhorou significativamente no grupo de mais idade. Nos ultimos seis meses de tratamento, a colonizacao cronica ou intermitente por P.aeruginosa foi detectada em 75,0, 71,4 e 62,5%, respectivamente, enquanto a colonizacao por S.aureus ocorreu em 68,6, 66,6 e 41,9% dos casos em cada grupo etario. CONCLUSOES: A intervencao com dornase alfa resultou em manutencao dos parâmetros pulmonares e associou-se a reducao significativa de visitas a emergencia por exacerbacao pulmonar no grupo de seis a 11 anos de idade, com melhora do escore clinico no grupo >14 anos de idade ao final de um ano de estudo.OBJECTIVE: 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. Students 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.

O impacto de primeiro ano de tratamento com dornase alfa nos parâmetros clínicos de pacientes com fibrose cística: estudo multicêntrico brasileiro

OBJETIVO: Relatar o impacto clinico do primeiro ano de tratamento com dornase alfa de acordo com a faixa etaria, numa coorte de pacientes brasileiros com fibrose cistica (FC). METODOS: O presente estudo analisou dados de 152 pacientes elegiveis, de 16 centros de referencia para FC, os quais responderam aos questionarios clinicos e realizaram testes laboratoriais, ao inicio do tratamento com dornase alfa (T0) e apos seis (T2) e 12 (T4) meses da intervencao. Analisaram-se tres grupos etarios: seis a 11, 12 a 13 e >14 anos de idade. Avaliaram-se os testes pulmonares, a microbiologia de vias aereas, os atendimentos de emergencia, as hospitalizacoes e os tratamentos emergenciais e rotineiros. O teste t de Student, o qui-quadrado e a analise de variância foram usados quando pertinentes. RESULTADOS: O tratamento baseou-se em fisioterapia respiratoria, exercicios regulares, enzimas pancreaticas, vitaminas, broncodilatadores, corticosteroides e antibioticos. Nos seis meses anteriores ao estudo (fase T0), as hospitalizacoes por exacerbacao pulmonar ocorreram em 38,0, 10,0 e 61,4%, respectivamente para as tres faixas etarias analisadas. No grupo de seis a 11 anos, houve reducao significativa de atendimentos de emergencia apos um ano de tratamento. Nao houve modificacoes significativas de volume expiratorio forcado no primeiro segundo (VEF1), capacidade vital forcada (CVF), saturacao de oxigenio (SpO)2 e indice de Tiffeneau em todos os grupos. O escore de Shwachman-Kulczychi melhorou significativamente no grupo de mais idade. Nos ultimos seis meses de tratamento, a colonizacao cronica ou intermitente por P.aeruginosa foi detectada em 75,0, 71,4 e 62,5%, respectivamente, enquanto a colonizacao por S.aureus ocorreu em 68,6, 66,6 e 41,9% dos casos em cada grupo etario. CONCLUSOES: A intervencao com dornase alfa resultou em manutencao dos parâmetros pulmonares e associou-se a reducao significativa de visitas a emergencia por exacerbacao pulmonar no grupo de seis a 11 anos de idade, com melhora do escore clinico no grupo >14 anos de idade ao final de um ano de estudo.OBJECTIVE: 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. Students 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.