Joaquim Carlos Rodrigues

Rhinovirus C and respiratory exacerbations in children with cystic fibrosis.

To investigate a possible role for human rhinovirus C in respiratory exacerbations of children with cystic fibrosis, we conducted microbiologic testing on respiratory specimens from 103 such patients in São Paulo, Brazil, during 2006–2007. A significant association was found between the presence of human rhinovirus C and respiratory exacerbations.

High rate of viral identification and coinfections in infants with acute bronchiolitis

OBJECTIVES: To determine the viruses and risk factors associated with hospital and intensive care unit (ICU) admissions in infants with acute bronchiolitis. INTRODUCTION: Bronchiolitis is a major cause of morbidity in infants. Widespread use of molecular-based methods has yielded new insights about its etiology, but the impact of viral etiologies on early outcomes is still unclear. METHODS: Seventy-seven infants with bronchiolitis who were under two years of age and visited an emergency unit were included. Using molecular-based methods, samples were tested for 12 different respiratory viruses. Logistic regression models were used to identify clinical and virological variables associated with the main endpoints: hospital admission and ICU admission. RESULTS: We identified at least one virus in 93.5% of patients, and coinfections were found in nearly 40% of patients. RSV was the most common pathogen (63.6%), followed by rhinovirus (39%). Identification of RSV was only associated with an increased risk of hospital admission in the univariate model. Younger age and enterovirus infection were associated with an increased risk of hospital admission, while atopy of a first-degree relative showed a protective effect. Prematurity was associated with an increased risk of admission to the ICU. Coinfections were not associated with worse outcomes. CONCLUSIONS: Molecular-based methods resulted in high rates of viral identification but did not change the significant role of RSV in acute bronchiolitis. Younger age and enterovirus infection were risk factors for hospital admission, while prematurity appeared to be a significant risk factor for admission to the ICU in acute viral bronchiolitis.

Nutritional follow-up of cystic fibrosis patients: the role of nutrition education

OBJECTIVE To evaluate the nutritional status of a group of cystic fibrosis patients and establish the role of nutrition education addressed to them in a comparative study before and after intervention. METHODS All cystic fibrosis patients in regular follow-up in the pulmonology clinic of Instituto da Crianca during 1996-99 were prospectively monitored for 3.5 years. Measurements of weight, height, mid upper arm circumference, skinfolds and calculations of weight/age, height/age, weight/height, mid upper arm circumference and triceps z scores, percentage of ideal weight for height, percentage of body fat, check of the use of enzymes with meals and of the use of nutritional supplements were performed at four points in time: initial (I), 7 (II), 13 (III) and 43 (IV) months after the first evaluation. Nutritional counseling was given both verbally and in writing (booklet) to all patients. RESULTS Seventy-four patients, 38F/36M, age range 6 months to 18.4 years were evaluated. At study entry the anthropometric data showed: percentage of ideal weight for height = 94+/-13, percentage of body fat = 15+/-7.1, z scores for weight/age = -1.13+/-1.3, z scores for height/age = -0.94+/-1.2, z scores for weight/height = -0.69+/-1.1, z scores for mid upper arm circumference = -1.35+/-1.3, triceps z scores = -0.74+/-0.9. Compliance with enzyme therapy and use of high-calorie supplements improved during the study period. There was a significant increase in weight/height and triceps z scores and percentage of body fat throughout the study period. After stratifying patients into three age groups the anthropometric improvement was only significant among children under 5 years of age CONCLUSIONS Mild malnutrition was present in this group of cystic fibrosis patients. The nutrition education led to an improvement in compliance with enzyme therapy, use of nutritional supplements and in nutritional status, mostly among the younger patients.

Avaliação do conhecimento sobre o uso de inaladores dosimetrados entre profissionais de saúde de um hospital pediátrico

OBJETIVO: Avaliar os conhecimentos sobre o uso e manejo de inaladores pressurizados dosimetrados entre profissionais de um hospital pediatrico terciario. METODOS: Foram realizadas avaliacoes sobre o conhecimento do uso de inaladores pressurizados dosimetrados atraves de questionarios teoricos e praticos, com medicos, fisioterapeutas, enfermeiras e auxiliares de enfermagem, atribuindo-se uma nota de 0 a 10 para cada avaliacao. Calcularam-se as medianas das notas obtidas pelos profissionais de cada categoria, as questoes com maiores e menores indices de erros, e foi feita a comparacao descritiva do desempenho das diversas categorias profissionais. A analise estatistica foi feita atraves do metodo de Kruskal-Wallis de comparacao de medianas. Tambem foi realizada a analise atraves de regressao logistica multipla sequencial. RESULTADOS: Foram avaliados 30 medicos residentes ou estagiarios de pediatria, 23 medicos assistentes, 29 fisioterapeutas, 33 enfermeiras e 31 auxiliares de enfermagem. Os medicos residentes, fisioterapeutas e medicos assistentes obtiveram desempenho significativamente superior aos dos enfermeiros e auxiliares de enfermagem. Apenas os medicos residentes obtiveram mediana superior a 6. CONCLUSOES: O conhecimento a respeito do uso de inaladores dosimetrados entre os profissionais de saude da instituicao e insatisfatorio, principalmente entre enfermeiros e auxiliares de enfermagem, diretamente envolvidos na aplicacao pratica dos inaladores dosimetrados.

Ozone Is Associated With an Increased Risk of Respiratory Exacerbations in Patients With Cystic Fibrosis

Background Tropospheric oxidant pollutants may injure the respiratory tract. Cystic fibrosis (CF) respiratory disease involves significant inflammation and excessive oxidative stress, and exposure to air pollutants can magnify the lung damage. The objective of this study was to investigate the association between the short-term variation in the concentration of air pollutants in metropolitan São Paulo, Brazil, and the occurrence of respiratory exacerbations in children and adolescents with CF. Methods A longitudinal panel of repeated measurements was obtained from 103 patients attending the outpatient center of our institution from September 6, 2006 through September 4, 2007. Daily concentrations of inhaled particulate matter, sulfur dioxide, nitrogen dioxide, ozone (O3), carbon monoxide, and meteorologic variables, such as the minimum temperature and relative humidity, were evaluated. The generalized estimation equation model for binomial distribution was used to assess the impact of these measurements on the occurrence of acute respiratory exacerbations. Results In total, 103 patients with CF (median age, 8.9 years) made 408 visits, with a mean ± SD of 4 ± 1.74 visits per patient (range, 2-9). A respiratory disease exacerbation was diagnosed on 142 visits (38.4%). An interquartile range increase in the O3 concentration (45.62 μg/m3) had a positive, delayed (2 days after exposure) effect on the risk of a respiratory exacerbation (relative risk = 1.86; 95% CI, 1.14-3.02). Conclusions This study demonstrates that exposure to short-term air pollution in a large urban center increases the risk of a pulmonary exacerbation in patients with CF.

Primary ciliary dyskinesia: evaluation using cilia beat frequency assessment via spectral analysis of digital microscopy images

Ciliary beat frequency (CBF) measurements provide valuable information for diagnosing of primary ciliary dyskinesia (PCD). We developed a system for measuring CBF, used it in association with electron microscopy to diagnose PCD, and then analyzed characteristics of PCD patients. The CBF measurement system was based on power spectra measured through digital imaging. Twenty-four patients suspected of having PCD (age 1-19 yr) were selected from a group of 75 children and adolescents with pneumopathies of unknown causes. Ten healthy, nonsmoking volunteers (age ≥ 17 yr) served as a control group. Nasal brush samples were collected, and CBF and electron microscopy were performed. PCD was diagnosed in 12 patients: 5 had radial spoke defects, 3 showed absent central microtubule pairs with transposition, 2 had outer dynein arm defects, 1 had a shortened outer dynein arm, and 1 had a normal ultrastructure. Previous studies have reported that the most common cilia defects are in the dynein arm. As expected, the mean CBF was higher in the control group (P < 0.001) and patients with normal ultrastructure (P < 0.002), than in those diagnosed with cilia ultrastructural defects (i.e., PCD patients). An obstructive ventilatory pattern was observed in 70% of the PCD patients who underwent pulmonary function tests. All PCD patients presented bronchial wall thickening on chest computed tomography scans. The protocol and diagnostic techniques employed allowed us to diagnose PCD in 16% of patients in this study.

Etiological diagnosis of pneumonia: a critical view

OBJECTIVES To search literature related to the etiological diagnosis of acute pneumonia in children. SOURCES Systematic review of Medline and Lilacs databases. SUMMARY OF THE FINDINGS The use of new diagnostic methods such as immunological techniques and polymerase chain reaction has proven invaluable for specific diagnosis and epidemiological investigation, showing adequate sensitivity, specificity and promptness of results, with the aim of guiding therapy properly. Review of epidemiological studies of community acquired pneumonia showed that Streptococcus pneumoniae is still one of the most significant etiologic agents in all age groups, in developing and industrialized countries. Resistance of this agent to penicillin and cephalosporins is increasing in all continents and is worrisome. Atypical agents such as Mycoplasma pneumoniae and Chlamydia pneumoniae are common in community acquired pneumonia, mainly in children older than 4 years, representing one third of the cases in industrial countries. However, their prevalence in developing countries remain to be determined. Respiratory syncytial virus is also a very common etiology of community acquired pneumonia and may cause severe infections, mainly in infants and younger children. The introduction of new conjugated vaccines for Streptococcus pneumoniae and Haemophilus influenzae type b resulted in significant reduction of morbidity and mortality of pneumonia in children. CONCLUSIONS A significant impact on morbidity and mortality of acute pneumonia in children is likely to occur if microbiological and antimicrobial control is continuously and dynamically performed, thus allowing for the development of new vaccines, particularly against the respiratory syncytial virus.Objective: to search literature related to the etiological diagnosis of acute pneumonia in children. Sources: systematic review of Medline and Lilacs databases. Summary of the findings: the use of new diagnostic methods such as immunological techniques and polymerase chain reaction has proven invaluable for specific diagnosis and epidemiological investigation, showing adequate sensitivity, specificity and promptness of results, with the aim of guiding therapy properly. Review of epidemiological studies of community acquired pneumonia showed that Streptococcus pneumoniae is still one of the most significant etiologic agents in all age groups, in developing and industrialized countries. Resistance of this agent to penicillin and cephalosporins is increasing in all continents and is worrisome. Atypical agents such as Mycoplasma pneumoniae and Chlamydia pneumoniae are common in community acquired pneumonia, mainly in children older than 4 years, representing one third of the cases in industrial countries. However, their prevalence in developing countries remain to be determined. Respiratory syncytial virus is also a very common etiology of community acquired pneumonia and may cause severe infections, mainly in infants and younger children. The introduction of new conjugated vaccines for Streptococcus pneumoniae and Haemophilus influenzae type b resulted in significant reduction of morbidity and mortality of pneumonia in children. Conclusions: a significant impact on morbidity and mortality of acute pneumonia in children is likely to occur if microbiological and antimicrobial control is continuously and dynamically performed, thus allowing for the development of new vaccines, particularly against the respiratory syncytial virus.

Comparison between classic Gibson and Cooke technique and sweat conductivity test in patients with and without cystic fibrosis

OBJECTIVE To compare sweat chloride values obtained by quantitative pilocarpine iontophoresis (classic test) with the sweat conductivity values obtained using Macroduct collection system in patients with and without cystic fibrosis (CF). The cost and time spent to carry out each test were also analyzed. METHODS The sweat test using both techniques was performed at the same time in patients with and without CF. Conductivity cutoff values to rule out or diagnose CF were < 75 and > or = 90 mmol/L, respectively, and for the classic test the chloride values were < 60 and > or = 60 mmol/L. RESULTS Fifty-two patients with CF (29 males and 23 females; aged from 1.5 to 18.2 years) underwent the sweat test using both techniques, showing median sweat chloride and conductivity values of 114 and 122 mmol/L, respectively. In all of them, conductivity was > or = 95 mmol/L, which provided the test with 100% sensitivity (95%CI 93.1-100). Fifty patients without CF (24 males and 26 females; aged from 0.5 to 12.5 years) had median sweat chloride and conductivity values of 15.5 and 30 mmol/L, respectively. In all cases, conductivity was < 70 mmol/L, which provided the test with 100% specificity (95%CI 92.9-100). Time spent to perform the tests was significantly shorter for the conductivity test, and its cost was also lower. CONCLUSIONS The conductivity test showed high sensitivity and specificity, and there was good correspondence between the tests. The time spent to carry out the conductivity test was shorter and the cost was lower in comparison with the classic test.

Shwachman-Kulczycki score still useful to monitor cystic fibrosis severity.

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 (FEV1), 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 FEV1 was 59 ± 26 (as percentage of predicted values). The following significant correlations versus the Shwachman-Kulczycki score were found: FEV1 (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 FEV1, a statistically significantly correlation with the Shwachman-Kulczycki score was found only in patients with FEV1 <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.

ABPA diagnosis in cystic fibrosis patients: the clinical utility of IgE specific to recombinant Aspergillus fumigatus allergens

OBJECTIVE Allergic bronchopulmonary aspergillosis (ABPA) is a complicating factor of cystic fibrosis which can result in a devastating combination as lung disease progresses. The overlap between the signs and symptoms of the two conditions makes diagnosis problematic, even if standardized criteria are used. The objective of this study was to identify, in a group of cystic fibrosis patients, cases of ABPA by assaying IgE specific to recombinant Aspergillus fumigatus antigens and to compare the method with the Cystic Fibrosis Foundation diagnostic criteria. METHODS Fifty-four patients, aged 2 to 20 years, presenting characteristics that could occur with ABPA in isolation, were systematically assessed based on the following: clinical data, a chest CT scan, immediate hypersensitivity skin test for A. fumigatus, total serum IgE assay, RAST for A. fumigatus and serum IgE specific for the recombinant allergens Asp f1, f2, f3, f4 and f6. RESULTS Thirty-nine patients were eligible for the study. Thirty-two of these were investigated. Sensitization to A. fumigatus was observed in 34%. Both the Cystic Fibrosis Foundation criteria and the recombinant antigen specific IgE assay defined three patients as suffering from ABPA; however, only two of these patients were diagnosed by both methods. CONCLUSIONS The detection of A. fumigatus recombinant antigen specific IgE was a useful tool for the early detection of sensitization and diagnosis of ABPA. Nevertheless, diagnostic confirmation cannot be divorced from clinical findings, and before this method can be used for ABPA diagnosis, for detecting relapses and for defining cure criteria, longitudinal studies with larger numbers of patients are required.