Air stacking: effects of Pilates mat exercises on muscle strength and on pulmonary function in patients with cystic fibrosis.

OBJECTIVE: To analyze the effects of Pilates mat exercises in patients with cystic fibrosis (CF). METHODS: This was a clinical trial involving 19 CF patients recruited from either the CF Outpatient Clinic of the State University at Campinas Hospital de Clínicas or the Children's Institute of the University of São Paulo School of Medicine Hospital das Clínicas. All of the patients performed Pilates mat exercises for four months (one 60-min session per week). The variables studied (before and after the intervention) were respiratory muscle strength, MIP, MEP, FVC, and FEV1. RESULTS: After the intervention, MIP was significantly higher in the male patients (p = 0.017), as were MIP and MEP in the female patients (p = 0.005 and p = 0.007, respectively). There were no significant differences between the pre- and post-intervention values of FVC or FEV1, neither in the sample as a whole nor among the patients of either gender. CONCLUSIONS: Our results show that Pilates mat exercises have beneficial effects on respiratory muscle strength in CF patients.

Effects of Pilates mat exercises on muscle strength and on pulmonary function in patients with cystic fibrosis / Efeitos do método Pilates na força muscular e na função pulmonar de pacientes com fibrose cística

OBJECTIVE: To analyze the effects of Pilates mat exercises in patients with cystic fibrosis (CF). METHODS: This was a clinical trial involving 19 CF patients recruited from either the CF Outpatient Clinic of the State University at Campinas Hospital de Clínicas or the Children's Institute of the University of São Paulo School of Medicine Hospital das Clínicas. All of the patients performed Pilates mat exercises for four months (one 60-min session per week). The variables studied (before and after the intervention) were respiratory muscle strength, MIP, MEP, FVC, and FEV1. RESULTS: After the intervention, MIP was significantly higher in the male patients (p = 0.017), as were MIP and MEP in the female patients (p = 0.005 and p = 0.007, respectively). There were no significant differences between the pre- and post-intervention values of FVC or FEV1, neither in the sample as a whole nor among the patients of either gender. CONCLUSIONS: Our results show that Pilates mat exercises have beneficial effects on respiratory muscle strength in CF patients. .

OBJETIVO: Analisar os efeitos do método Pilates em pacientes com fibrose cística (FC). MÉTODOS: Foi realizado um ensaio clínico, casuística de 19 pacientes com FC. Os pacientes foram recrutados no Ambulatório de FC do Hospital de Clínicas da Universidade Estadual de Campinas e no Instituto da Criança do Hospital das Clínicas da Faculdade de Medicina da Universidade de São Paulo. Todos os pacientes foram submetidos a uma sessão semanal de Pilates de 60 min durante quatro meses. As variáveis estudadas, antes e após a intervenção, foram força muscular respiratória, PImáx, PEmáx, CVF e VEF1. RESULTADOS: Houve um aumento significativo na PImáx nos pacientes do sexo masculino (p = 0,017), enquanto houve aumentos significativos na PImáx e PEmáx nos pacientes do sexo feminino (p = 0,005 e p = 0,007, respectivamente) após a intervenção. Não houve diferenças significativas nos valores de CVF e VEF1 antes e após a intervenção no grupo total de participantes, nem nos subgrupos em relação ao gênero. CONCLUSÕES: Os resultados deste estudo mostraram os efeitos benéficos da aplicação do método Pilates na força muscular respiratória nos pacientes estudados. (Registro Brasileiro de Ensaios Clínicos - ReBEC; número de identificação RBR-86vp8x [http://www.ensaiosclinicos.gov.br]) .

A first-year dornase alfa treatment impact on clinical parameters of patients with cystic fibrosis: the Brazilian cystic fibrosis multicenter study / El impacto de primer ano de tratamiento con dornasa alfa en los parametros clinicos de pacientes con fibrosis quistica: resultado de estudio brasileno multicentrico / O impacto de primeiro ano de tratamento com dornase alfa nos parametros clinicos de pacientes com fibrose cistica: estudo multicentrico brasileiro

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. 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 ...

OBJETIVO: Relatar el impacto clínico del primer año de tratamiento con dornasa alfa conforme a la franja de edad, en una cohorte de pacientes brasileños con fibrosis quística (FC). MÉTODOS: El presente estudio analizó datos de 152 pacientes elegibles, de 16 centros de referencia para FC, los que contestaron a los cuestionarios clínicos y realizaron pruebas laboratoriales, al inicio del tratamiento con la dornasa alfa (T0) y después de 6 (T2) y 12 (T4) meses de la intervención. Se analizaron 3 grupos etarios: 6-11, 12-13 e >14 años de edad. Se evaluaron las pruebas pulmonares, la microbiología de vías aéreas, las atenciones de emergencia, hospitalizaciones y tratamientos emergenciales y de rutina. Las estadísticas descriptivas, pruebas t y chi-cuadrado y ANOVA fueron usadas cuando pertinentes. RESULTADOS: El tratamiento regular se basó en la fisioterapia respiratoria, ejercicios regulares, encimas pancreáticas, vitaminas, broncodilatadores, corticosteroides y antibióticos. En los 6 meses anteriores al estudio (fase T0), las hospitalizaciones por exacerbación pulmonar ocurrieron en 38, 10 y 61,4%, respectivamente, para las tres franjas de edad analizadas. En el grupo 6-11 años, hubo reducción significativa de atenciones de emergencia después de 1 año de tratamiento. No hubo modificaciones significativas de volumen espiratorio forzado en el 1er segundo (VEF1), capacidad vital forzada (CVF), saturación de oxígeno (SpO)2 e índice de Tiffeneau, en todos grupos. El escore de Schwachman-Kulczychi mejoró significativamente en el grupo de más edad. Los últimos 6 meses de tratamiento, la colonización crónica o intermitente por P. aeruginosa fue detectada en el 75, 71,4 y 62,5%, respectivamente, mientras que por S. aureus ocurrió en 68,6, 66,6 y 41,9% de los casos en cada grupo de ...

OBJETIVO: Relatar o impacto clínico do primeiro ano de tratamento com dornase alfa de acordo com a faixa etária, numa coorte de pacientes brasileiros com fibrose cística (FC). MÉTODOS: O presente estudo analisou dados de 152 pacientes elegíveis, de 16 centros de referência para FC, os quais responderam aos questionários clínicos e realizaram testes laboratoriais, ao início do tratamento com dornase alfa (T0) e após seis (T2) e 12 (T4) meses da intervenção. Analisaram-se três grupos etários: seis a 11, 12 a 13 e >14 anos de idade. Avaliaram-se os testes pulmonares, a microbiologia de vias aéreas, os atendimentos de emergência, as hospitalizações e os tratamentos emergenciais e rotineiros. O teste t de Student, o qui-quadrado e a análise de variância foram usados quando pertinentes. RESULTADOS: O tratamento baseou-se em fisioterapia respiratória, exercícios regulares, enzimas pancreáticas, vitaminas, broncodilatadores, corticosteroides e antibióticos. Nos seis meses anteriores ao estudo (fase T0), as hospitalizações por exacerbação pulmonar ocorreram em 38,0, 10,0 e 61,4%, respectivamente para as três faixas etárias analisadas. No grupo de seis a 11 anos, houve redução significativa de atendimentos de emergência após um ano de tratamento. Não houve modificações significativas de volume expiratório forçado no primeiro segundo (VEF1), capacidade vital forçada (CVF), saturação de oxigênio (SpO)2 e índice de Tiffeneau em todos os grupos. O escore de Shwachman-Kulczychi melhorou significativamente no grupo de mais idade. Nos últimos seis meses de tratamento, a colonização crônica ou intermitente por P.aeruginosa foi detectada em 75,0, 71,4 e 62,5%, respectivamente, enquanto a colonização ...

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

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. 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 (VEF(1)), in forced vital capacity (FVC), in oxygen saturation (SpO(2)), 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.

Six-minute walk test in healthy children: is the leg length important?

RATIONALE: Measures as height, age, and weight influence the six-minute walk test (6MWT). It was shown that children's true leg length (TLL) influence the 6MWT distance but so far it has never been evaluated how much this variable could predict the distance walked related to height. Our hypothesis is that there should not have any significant difference between models including height or TLL. OBJECTIVE: To establish and to compare the predicted walked distance of healthy children in the 6MWT by two distinct models, one including TLL and other including height. METHODS: Observational cross sectional study. A total of 161 healthy children (84 girls, 52.2%), 6-13 years old from three local primary and secondary schools. Two 6MWT were performed following ATS guidelines. Weight, height, and TLL were measured. The longer walked distance was selected for statistical analysis. RESULTS: Anthropometric data were similar for both genders into each group of age. Mean (±SD) walked distances in whole group were: boys 704.4 m (77.7); girls 681.6 m (67.9; P = 0.049). In the univariate regression to predict the distance walked, the adjusted coefficients to TLL and height were in boys: 0.46 and 0.39; and in girls: 0.35 and 0.29, respectively. After adjusting for age and weight, the residuals of the two equations were not significantly different (P = 0.998). CONCLUSION: The model including TLL to predict the walked distance by healthy children explained more of the variation on the walked distance in the 6MWT than the model using the height but without significant difference between the models.

Dornase alfa improves the health-related quality of life among Brazilian patients with cystic fibrosis--a one-year prospective study.

SUMMARY BACKGROUND: Health-related quality of life (HRQOL) measurements provide valuable information about the psychological and social impact of treatment on patients with cystic fibrosis (CF). This study evaluated the HRQOL of Brazilian patients with CF and assessed the changes in HRQOL domains over 1 year after dornase alfa (Pulmozyme) introduction. PATIENTS AND METHODS: One hundred fifty-six stable patients with CF and 89 caregivers answered the Portuguese-validated version of the Cystic Fibrosis Questionnaire-Revised (CFQ-R) at baseline (T(0)), and at 3 (T(1)), 6 (T(2)), 9 (T(3)), and 12 (T(4)) months of follow-up. Eighteen patients were excluded because they did not fulfill the inclusion criteria. The patients were analyzed in two groups: those aged 6-11 years and those aged 14 years and older. ANOVA for observed repeated results and the last observation carried forward (LOCF) method for missing data were used for the statistical analysis. RESULTS: After 1 year of follow-up, there was significant improvement in respiratory symptoms (T(4) - T(0) = 8.1; 95% confidence interval (95% CI) = [2.1;14.0]; effect size (ES) = 0.35; P < 0.001), Emotional Functioning (T(4) - T(0) = 5.6; 95% CI = [1.1;10.1]; ES = 0.31; P < 0.05), Social Functioning (T(4) - T(0) = 6.0; 95% CI = [1.3;11.7]; ES = 0.31; P < 0.05), Body Image (T(4) - T(0) = 11.9; 95% CI = [4.1;19.7]; ES = 0.42; P < 0.05), and Treatment Burden (T(4) - T(0) = 5.3; 95% CI = [0.3;10.3]; ES = 0.24; P < 0.05) domains in the younger group. A significant improvement in Role Functioning (T(4) - T(0) = 6.1; 95% CI = [1.1;11.1]; ES = 0.40; P < 0.05), Body Image (T(4) - T(0) = 12.6; 95% CI = [3.5;21.7]; ES = 0.46; P < 0.05), and Weight (T(4) - T(0) = 11.7; 95% CI = [1.8;21.6]; ES = 0.40; P < 0.05) was obtained in the older group. The caregivers' CFQ-R showed improvements in the Digestive Symptoms (T(4) - T(0) = 5.5; 95% CI = [1.5;9.4]; ES = 0.30; P < 0.05), Respiratory Symptoms (T(4) - T(0) = 7.6; 95% CI = [3.9;11.4]; ES = 0.48; P < 0.05), and Weight (T(4) - T(0) = 10.1; 95% CI = [1.6;18.6]; ES = 0.26; P < 0.05) domains. CONCLUSION: The introduction of dornase alfa improved the HRQL of the patients with CF during the first year of treatment.

Incidence of cystic fibrosis in five different states of Brazil as determined by screening of p.F508del, mutation at the CFTR gene in newborns and patients.

Cystic Fibrosis (CF) is one of the most common single-gene defects in European descent populations with an incidence of about 1 in every 2500 live births and carrier frequency of approximately 1 in 25. The most common mutation at the CF transmembrane conductance regulator (CFTR) gene is a deletion (p.F508del) of the phenylalanine codon 508; its frequency, however, is not the same throughout the world. The purpose of this paper is to document an application of a two-tier survey design in different states of Brazil, from which regional differences of the incidence of CF and frequency of CF-causing mutation(s) carriers can be for the first time estimated. We present data on genotype distributions in reference to p.F508del mutation in samples of newborns, adult controls and CF patients from five Brazilian states, in which a total of 2683 newborns born to Brazilian white parents and 500 African-Brazilians adult controls were screened, as well as 300 CF patients (262 European descents and 38 African descents) were genotyped. Our results suggest that the CF-incidence in different parts of Brazil may differ by almost 20-fold. For the five different states as a whole, nearly 48% of the CF-alleles carry the p.F508del mutation, which places the estimates of disease incidence and carrier frequencies for the Brazilian European descents as 1 in 7576 live births and 2.3%, respectively. The implications for prevention of CF and other rare Mendelian diseases through such surveys of mutation screening are discussed.

Six-minute walk test in children and adolescents with cystic fibrosis.

The 6-min walk test is a simple, rapid, and low-cost method that determines tolerance to exercise. We examined the reproducibility of the 6-min walk test in 16 children with cystic fibrosis (11 female, 5 male; age range, 11.0 +/- 1.9 years). We related the distance walked and the work performed (distance walked x body weight) with nutritional (body mass index and respiratory muscle strength) and clinical (degree of bronchial obstruction and Shwachman score) status. Patients were asked to walk as far as possible upon verbal command on two occasions. There was no statistical difference between distances walked (582.3 +/- 60 and 598.2 +/- 56.8 m, P = 0.31), heart rate, respiratory rate, pulse oxygen saturation, arterial blood pressure, dyspnea, and percentage of maximal heart rate for age in the two tests. Distance walked correlated (Pearson) with maximal expiratory pressure (98.6 +/- 28.1 cmH2O, r = 0.60, P < 0.01), maximal heart rate (157.9 +/- 10.1 bpm, r = 0.59, P < 0.02), Borg dyspnea scale (1.7 +/- 2.4, r = 0.55, P < 0.03), and double product (blood pressure x heart rate; r = 0.59, P < 0.02). The product of distance walked and body weight (work) correlated (Pearson) with height (r = 0.83, P = 0.000), maximal expiratory pressure (r = 0.64, P < 0.01), systolic blood pressure (r = 0.56, P < 0.02), and diastolic blood pressure (r = 0.55, P < 0.03). We conclude that the 6-min walk test is reproducible and easy to perform in children and adolescents with cystic fibrosis. The distance walked was related to the clinical variables studied. Work in the 6-min walk test may be an additional parameter in the determination of physical capacity.

Linguistic validation of cystic fibrosis quality of life questionnaires.

OBJECTIVE: The purpose of this study was to validate the Portuguese translations of four cystic fibrosis quality of life questionnaires (CFQ). The first three were developed for patients with cystic fibrosis aged from 6 to 11 years, from 12 to 13 years and 14 years or more, while the fourth was developed for the parents of patients aged 6 to 13 years. MATERIAL AND METHODS: The four CFQ translations contained from 35 to 50 questions covering nine domains and were validated as follows: translation from English to Portuguese, pilot application, back translation and then approval by the author of the English versions. The four translations were applied to 90 stable patients (30 from each age group) and the parents of patients aged 6-13 years (n = 60), on two occasions with a 13 to 17 day interval. Intraclass Correlation Coefficients (ICC) were used to measure reproducibility. This study was approved by the Commission for Ethics in Research at the institution. RESULTS: Reproducibility was good (ICC = 0.62 to 0.99) for the four translations in all domains, with the exceptions of the Digestion domain for the 6 to 11 and 12 to 13 years age groups with ICC = 0.59 and 0.47, respectively and the Social Role domain for the 14 and over age group (ICC = -0.19 ). CONCLUSION: The translation and cultural adaptation for Brazil resulted in four CFQ versions that are easy to understand and offer good reproducibility.

Linguistic validation of cystic fibrosis quality of life questionnaires

OBJECTIVE: The purpose of this study was to validate the Portuguese translations of four cystic fibrosis quality of life questionnaires (CFQ). The first three were developed for patients with cystic fibrosis aged from 6 to 11 years, from 12 to 13 years and 14 years or more, while the fourth was developed for the parents of patients aged 6 to 13 years.MATERIAL AND METHODS: The four CFQ translations contained from 35 to 50 questions covering nine domains and were validated as follows: translation from English to Portuguese, pilot application, back translation and then approval by the author of the English versions. The four translations were applied to 90 stable patients (30 from each age group) and the parents of patients aged 6-13 years (n = 60), on two occasions with a 13 to 17 day interval. Intraclass Correlation Coefficients (ICC) were used to measure reproducibility. This study was approved by the Commission for Ethics in Research at the institution. RESULTS: Reproducibility was good (ICC = 0.62 to 0.99) for the four translations in all domains, with the exceptions of the Digestion domain for the 6 to 11 and 12 to 13 years age groups with ICC = 0.59 and 0.47, respectively and the Social Role domain for the 14 and over age group (ICC = -0.19 ).CONCLUSION: The translation and cultural adaptation for Brazil resulted in four CFQ versions that are easy to understand and offer good reproducibility.

[Bronchopulmonary dysplasia]. / Displasia broncopulmonar.

OBJECTIVE: To present a wide-ranging review of the literature on broncopulmonary dysplasia, covering new definitions, pathophysiology, prevention, treatment, prognosis and progression. SOURCES OF DATA: The most relevant articles published on the subject since it was first described in 1967 were selected from MEDLINE search results. SUMMARY OF THE FINDINGS: Bronchopulmonary dysplasia is considered one of the primary causes of chronic lung disease among infants. It is associated with frequent and prolonged hospital admissions, in particular for pulmonary diseases, with high rates of mortality and alterations to neuropsychomotor development and pondero-statural growth. Pathogenesis is complex, being primarily influenced by prematurity, infection, supplementary oxygen and mechanical ventilation. Prevention involves appropriate prenatal care, the prevention of premature delivery, prenatal corticosteroids, surfactant replacement therapy and "protective" ventilatory strategies. Treatment of bronchopulmonary dysplasia patients demands a multidisciplinary team. When indicated, oxygen supplementation is extremely important. Despite increased risk of morbidity and mortality during the first years of life, long term progress is favorable in the majority of cases. CONCLUSIONS: Bronchopulmonary dysplasia has been and continues to be studied in great depth with the objective of identifying its causes and possible prevention and treatment strategies. Controversies remain with respect of these issues and also about the prognosis of these patients, in particular when the subject is long-term progress of "new" bronchopulmonary dysplasia patients.

Displasia broncopulmonar / Bronchopulmonary dysplasia

OBJETIVO: Apresentar uma ampla revisão da literatura sobre displasia broncopulmonar, abordando novas definições, fisiopatologia, prevenção, tratamento, prognóstico e evolução. FONTE DOS DADOS: Foram selecionados os artigos mais relevantes sobre o tema, desde a sua descrição inicial, em 1967, pesquisados na MEDLINE. SíNTESE DOS DADOS: A displasia broncopulmonar é considerada uma das principais causas de doença pulmonar crônica em lactentes. Está associada a hospitalizações freqüentes e prolongadas, especialmente por doenças pulmonares, altos índices de mortalidade e alterações no desenvolvimento neuropsicomotor e no crescimento pôndero-estatural. A patogênese é complexa e influenciada principalmente por prematuridade, infecção, oxigênio suplementar e ventilação mecânica. A prevenção envolve o acompanhamento pré-natal adequado, a prevenção do parto prematuro, o uso pré-natal do corticosteróide, a terapia de reposição de surfactante e o uso de estratégias ventilatórias "protetoras". O tratamento do paciente com displasia broncopulmonar demanda uma equipe multidisciplinar. Quando indicada, a suplementação de oxigênio é de extrema importância. Apesar de maior risco de morbimortalidade nos primeiros anos de vida, a evolução em longo prazo é favorável na maioria das vezes. CONCLUSÕES: A displasia broncopulmonar vem sendo profundamente estudada na tentativa de identificação das suas causas e possibilidades de prevenção e de tratamento. Ainda existem controvérsias quanto a esses assuntos e também em relação ao prognóstico desses pacientes, especialmente quando se trata da evolução tardia da "nova" displasia broncopulmonar.

Hábito de fumar das gestantes e parturientes de um hospital universitário e seus conhecimentos sobre os efeitos do fumo em fetos e lactentes / Smoking habits in pregnant and postpartum womwn at universitary hospital and their knowledge about the effects of tabaco smoke on fetus and children

Por meio de um questionário específico foram entrevistadas 255 parturientes com os seguintes objetivos: 1) Avaliar seus conhecimentos a respeito das repercussões do fumo sobre a gestante, o feto e o lactente. 2) Investigar as atitudes que seriam adotadas no domicílio, para evitar os danos do cigarro sobre o lactente. 3) Investigar a prevalência de fumantes numa maternidade. 4) Quantificar o peso e a estatura do recém nascido de mães fumantes versus mães não-fumantes. A maioria das parturientes não era casada, com idade média de 26,3 anos, raça branca (53,1%), grau de escolaridade e nível socioeconômico baixos. Verificou-se que 33,3% eram fumantes em gestações anteriores e 20,6% fumaram na gestação atual. As médias de peso e estatura ao nascimento foram significativamente menores nas crianças de mães fumantes. No domicílio, em 33,3% dos casos o marido era fumante e em 5,9% havia outros fumantes. A análise combinada dos fumantes, no domicílio, revelou que 61% dos RN estavam no risco de se tornarem fumantes passivos. Em relação à proteção do RN, 50% das mães fumantes tinham a intenção de adotar a atitude de fumar fora de casa e 73,8% pediriam para outros fumantes saírem de casa para fumar. Apesar de aparentemente bem informadas a respeito dos malefícios do cigarro, faltou às parturientes deste estudo uma conscientização maior e uma atitude adequada para que o índice de tabagistas fosse menor. Os autores ressaltam a importância das orientações quanto ao hábito de fumar a todas as gestantes e puérperas por parte dos profissionais de saúde.

Trough a specific questionnaire we have interviewed 255 post partum women. The objectives of this study were: 1) Estimate their knowledge about the result of the smoking habit of pregnant women on fetus and child; 2) Investigate positive behaviors that mothers could have at home to avoid damage caused by tabacco in their infants and children; 3) Investigatethe prevalence of smokers; 4) Quantify the newborn’s weight and height in smoking and no smoking mothers. Most of them weren’t married and their average age was 26.3 years. About 53% of the interviewed mothers were white and had low education and social economic level. We have observedthat 33.3% of these women used to smoke at previous pregnancies and 20.6% smoked at actual pregnancy. Theaverage birth height and weight of the newborns were significantly lower in the group of mothers who smoke. Athome, in 33.3%, the husband had smoked and in 5.9% there were others smokers. The statistical analysis of all smokers have showed that 61% of the newborns were at risk to become passive smokers. About 50% of smoking mothers have an intention to smoke out of the house and 73.8% will ask thesmoking persons to go out to smoke. Despite of being well informed about the risks, the post partum women had noconscient attitude and positive behavior to reduce their smoking habit. It is important to emphasize that healthprofessionals should provide all the pregnant and post partum women with proper information and orientation about the smoking habit and it’s consequences.

Cystic fibrosis with normal sweat chloride concentration--case report.

Cystic fibrosis is a genetic disease usually diagnosed by abnormal sweat testing. We report a case of an 18-year-old female with bronchiectasis, chronic P. aeruginosa infection, and normal sweat chloride concentrations who experienced rapid decrease of lung function and clinical deterioration despite treatment. Given the high suspicion of cystic fibrosis, broad genotyping testing was performed, showing a compound heterozygous with deltaF508 and 3849+10 kb C-->T mutations, therefore confirming cystic fibrosis diagnosis. Although the sweat chloride test remains the gold standard for the diagnosis of cystic fibrosis, alternative diagnostic tests such as genotyping and electrophysiologic measurements must be performed if there is suspicion of cystic fibrosis, despite normal or borderline sweat chloride levels.

Infeccoes virais em criancas portadoras de doenca respiratoria aguda, atendidas em um Centro de Saude Escola, em Belem, Para, Brasil / Viral etiology in children with acute respiratory disease treated at a University Primary Care Center, in Belem, Para, Brazil

Avaliar a clinica e a etiologia viral em criancas abaixo de 2 anos de idade, portadoras de doenca respiratoria aguda...

Fibrose cística (Mucoviscidose) / Cystic fibrosis

A fibrose cística é a mais frequente doença genética letal da população caucasiana. A doença, descrita em 1938, compromete diferentes sistemas do organismo e evolui de forma crônica e progressiva. Há um transporte anormal de íons através da membrana apical das células epiteliais exócrinas. A apresentação clínica compreende a tríade clássica de: doença pulmonar crônica, insufiência pancreática exócrina e níveis elevados de eletrólitos no suor. Desde a identificação da gen de FC em 1989, avanços têm sido feitos não só na patogênese como no diagnóstico e na terapêutica. Os autores discutem o diagnóstico genético, as formas de apresentação clínica e terapêutica atualizada desta patologia fatal, cuja sobrevida tem aumentado significativamente nos últimos 30 anos.

Cystic Fibrosis (CF) is the most frequently seen lethal genetic disease of Caucasian populations. It was first described in 1938. The disease involves different systems with a chronic and progressive outcome. There is an abnormal transporte of ions trough the apical membrane of exocrine epithelial cells. The common clinical presentation is characterized by the classical triad of chronic obstructive pulmonary disease tippically associated withStaphylococcus aureus and/or Pseudomonas aeruginosa infections; exocrine pancreatic insufficiency and its nutritional consequences; and abnormal sweat chloride level. Diagnosis confirmation requires a positive sweat teste (CI > 60,0mEq/l). Since identification of the gene and cystic fibrosis transmembrane conductance regulator (CFTR) in 1989, research has made advances and changed on the pathogenesis, diagnosis and therapeutic approaches. DNA analyses, with demonstration of two mutations known to be associated with CF, is another diagnostic criterion (genetic diagnosis). With this new diagnostic criterion it is possible to found out CF cases with sweat test bordeline or normal results and patients lacking some diagnostic critera. CF remains a fatal disease, however, the prognosis for survival has improved dramatically over the past 30 years, with a progressively better life quality. Full recovery is the goal of genetic therapy to be achieved in the future.

Tuberculose de coluna cervical baixa em crianca: relato de caso / Tuberculosis of the lower cervical spine in a child: a case report

Os autores apresentam um caso de crianca de oito anos com queixa de dor e deformidade em regiao cervical com duracao de tres meses, alem de sinais de compressao medular...

PCR identification of Pseudomonas aeruginosa and direct detection in clinical samples from cystic fibrosis patients.

This report describes a PCR primer pair that targets the algD GDP mannose gene of Pseudomonas aeruginosa and produces a specific 520-bp PCR product useful for P. aeruginosa identification. This PCR assay was tested with 182 isolates of P. aeruginosa and 20 isolates of other bacterial species, and demonstrated 100% specificity and sensitivity. The test was also able to detect P. aeruginosa directly in clinical samples such as sputum or throat swabs obtained from cystic fibrosis patients. The combination of this primer with a universal bacterial primer, acting as a control to assess DNA quality in the sample, resulted in a robust PCR method that can be used for rapid P. aeruginosa detection.