Abstract Objective To assess the prevalence of GERD exclusively by means of multichannel intraluminal impedanciometry associated with pH monitoring (MIIpH) and compare it with respiratory symptoms in children with CF. To compare MIIpH with pHmetry alone to perform GERD diagnosis. Methods An analytical cross-sectional study was conducted with children diagnosed with CF who underwent MIIpH. Clinical and laboratory markers, including respiratory and digestive symptoms, were used for comparative analyses. High-resolution chest computed tomography was performed on patients with symptoms of chronic lung disease. Severity was classified according to the Bhalla score. Results A total of 29 children < 10 yo (18 girls) were evaluated; 19 of whom with physiological GER and 10 with GERD. Of the children with GERD, seven had predominantly acid GER, two acid+non-acid GER, and one non-acid GER. Three patients had GERD diagnosed only by MIIpH. Bhalla scores ranged from seven to 17.75 with no significant relationship with GERD. The number of pulmonary exacerbations was associated with a decrease in esophageal clearance regardless of the position in pHmetry and MIIpH. Conclusions The prevalence of GERD was 34% in children with CF. There was no association between respiratory disease severity and GER types. MIIpH detected 30% more patients with GERD than pHmetry.

ABSTRACT BACKGROUND: Primary ciliary dyskinesia (PCD) is a rare and heterogeneous disease that is difficult to diagnose and requires complex and expensive diagnostic tools. The saccharin transit time test is a simple and inexpensive tool that may assist in screening patients with PCD. OBJECTIVES: This study aimed to compare changes in the electron microscopy findings with clinical variables and saccharin tests in individuals diagnosed with clinical PCD (cPCD) and a control group. DESIGN AND SETTING: An observational cross-sectional study was conducted in an otorhinolaryngology outpatient clinic from August 2012 to April 2021. METHOD: Patients with cPCD underwent clinical screening questionnaires, nasal endoscopy, the saccharin transit time test, and nasal biopsy for transmission electron microscopy. RESULTS: Thirty-four patients with cPCD were evaluated. The most prevalent clinical comorbidities in the cPCD group were recurrent pneumonia, bronchiectasis, and chronic rhinosinusitis. Electron microscopy confirmed the clinical diagnosis of PCD in 16 of the 34 (47.1%) patients. CONCLUSION: The saccharin test could assist in screening patients with PCD due to its association with clinical alterations related to PCD.

Abstract Objectives: To detect RSV or other thirteen respiratory viruses as possible causer agent of bronchiolitis in infants. Method: This is an epidemiological analytical study, conducted using a nasopharyngeal aspirate of 173 hospitalized children younger than two years old with severe bronchiolitis in three hospitals in the Campinas Metropolitan Region (CMR) during 2013-14. The data was statically evaluated by Pearson’s chi-squared test with statistical significance of 0.05 and 95% confidence level. Results: As expected, the most prevalent viruses detected were RSV A and B in 47% and 16% of the samples, respectively. However, almost a third of severe bronchiolitis cases there were no detection of RSV, and the viruses more commonly detected were rhinoviruses, which were identified in almost a quarter of all positive samples for at least a viral agent. Conclusions: Although nothing could be concluded from the disease severity and clinicalepidemiological data, the present study’s results indicate that severe bronchiolitis is not always related to RSV infections in children younger than two years old, and the rhinoviruses were more prevalent in these cases. These findings reinforce the need to carry out a

RESUMO Objetivo: Verificar se leituras de continuous glucose monitoring (CGM, monitoramento contínuo da glicose) anormais (hipoglicemia/hiperglicemia) podem prever o aparecimento de diabetes relacionado à fibrose cística (DRFC) e/ou comprometimento clínico (declínio do IMC e/ou do VEF1) em pacientes pediátricos com fibrose cística (FC). Métodos: Estudo de coorte longitudinal prospectivo envolvendo pacientes com FC sem diabetes no início do estudo. O tempo médio de acompanhamento foi de 3,1 anos. Os pacientes foram submetidos a CGM de três dias, teste oral de tolerância à glicose (TOTG) e medida de VEF1 e IMC no início do estudo. TOTG, VEF1 e IMC foram reavaliados ao final do acompanhamento. Resultados: Trinta e nove pacientes com FC (10-19 anos de idade) apresentaram leituras de CGM válidas no início do estudo, e 34 completaram o acompanhamento (média = 3,1 ± 0,5 anos). Nenhuma das variáveis estudadas previu evolução para DRFC ou apresentou associação com eventos hipoglicêmicos. O CGM conseguiu detectar anormalidades glicêmicas não reveladas pelo TOTG. Pacientes com níveis de glicose ≥ 140 mg/dL no CGM, comparados àqueles com níveis menores, apresentaram valores de IMC e escores z de IMC menores no início do estudo - 17,30 ± 3,91 kg/m2 vs. 19,42 ± 2,07 kg/m2; p = 0,043; e −1,55 ± 1,68 vs. −0,17 ± 0,88; p = 0,02, respectivamente - e no final do acompanhamento - 17,88 ± 3,63 kg/m2 vs. 19,95 ± 2,56 kg/m2; p = 0,039; e −1,65 ± 1,55 vs. −0,42 ± 1,08; p = 0,039. Na comparação dos pacientes com e sem DRFC, os primeiros apresentaram pior VEF1 (em % do previsto) - 22,67 ± 5,03 vs. 59,58 ± 28,92; p = 0,041 - e maior declínio do VEF1 (−36,00 ± 23,52 vs. −8,13 ± 17,18; p = 0,041) no final do acompanhamento. Conclusões: O CGM foi capaz de identificar anormalidades glicêmicas não detectadas pelo TOTG que se relacionaram com reduções precoces do IMC. O CGM foi ineficaz na previsão do aparecimento de diabetes nesta população com FC. Diferentes critérios diagnósticos para diabetes podem ser necessários para indivíduos com FC.

ABSTRACT Objective: To determine whether abnormal continuous glucose monitoring (CGM) readings (hypoglycemia/hyperglycemia) can predict the onset of cystic fibrosis-related diabetes (CFRD) and/or clinical impairment (decline in BMI and/or FEV1) in pediatric patients with cystic fibrosis (CF). Methods: This was a longitudinal prospective cohort study involving CF patients without diabetes at baseline. The mean follow-up period was 3.1 years. The patients underwent 3-day CGM, performed oral glucose tolerance test (OGTT), and had FEV1 and BMI determined at baseline. OGTT, FEV1, and BMI were reassessed at the end of the follow-up period. Results: Thirty-nine CF patients (10-19 years of age) had valid CGM readings at baseline, and 34 completed the follow-up period (mean = 3.1 ± 0.5 years). None of the study variables predicted progression to CFRD or were associated with hypoglycemic events. CGM could detect glucose abnormalities not revealed by OGTT. Patients with glucose levels ≥ 140 mg/dL, as compared with those with lower levels, on CGM showed lower BMI values and z-scores at baseline-17.30 ± 3.91 kg/m2 vs. 19.42 ± 2.07 kg/m2; p = 0.043; and −1.55 ± 1.68 vs. −0.17 ± 0.88; p = 0.02, respectively-and at the end of follow-up-17.88 ± 3.63 kg/m2 vs. 19.95 ± 2.56 kg/m2; p = 0.039; and −1.65 ± 1.55 vs. −0.42 ± 1.08; p = 0.039. When comparing patients with and without CFRD, the former were found to have worse FEV1 (in % of predicted)-22.67 ± 5.03 vs. 59.58 ± 28.92; p = 0.041-and a greater decline in FEV1 (−36.00 ± 23.52 vs. −8.13 ± 17.18; p = 0.041) at the end of follow-up. Conclusions: CGM was able to identify glucose abnormalities not detected by OGTT that were related to early-stage decreases in BMI. CGM was ineffective in predicting the onset of diabetes in this CF population. Different diagnostic criteria for diabetes may be required for individuals with CF.

Abstract Objective The aim of this study was to analyze the physical activity level, using two tools, and the functional capacity of children and adolescents with asthma and with different levels of disease control, and to compare them to those of individuals without asthma. Methods Cross-sectional study with children and adolescents with (asthma group, AG) and without asthma (WAG), aged from 7 to 17 years. All participants performed the six-minute walk test (6MWT), the Glittre Activities of Daily Living test (Glittre-ADL), the International Physical Activity Questionnaire (IPAQ), and daily record of steps on a pedometer. Results The study included 145 individuals with asthma and 173 individuals without asthma. The WAG walked a greater distance in the 6MWT and performed the Glittre-ADL in less time than the AG. Individuals with uncontrolled, partially controlled, and controlled asthma presented the same functional capacity. A difference was observed in the IPAQ classification, with 13.9% of participants from the WAG being sedentary, compared with 26.2% in the AG. The mean quantity of steps measured by the pedometer was higher in the WAG. Conclusion There was a difference in the performance of individuals with and without asthma in the physical activity and functional capacity tests. The AG presented worse performance in the physical activity tests and, regardless of the level of asthma control, presented worse functional capacity.

RESUMO Objetivo: Determinar a prevalência de sibilância recorrente (SR) em crianças pré-termo que receberam profilaxia contra infecção grave pelo vírus sincicial respiratório (VSR) e identificar susceptibilidade genética (atopia ou asma) e fatores de risco para SR. Métodos: Estudo transversal envolvendo crianças pré-termo que receberam profilaxia com palivizumabe em um centro de referência no Brasil durante os primeiros dois anos de vida. Um questionário estruturado foi aplicado em entrevista presencial com os pais ou responsáveis. Resultados: O estudo incluiu 410 crianças pré-termo (mediana de idade = 9 meses [0-24 meses]). Na amostra total, 111 crianças (27,1%; IC95%: 22,9-31,5) apresentavam SR. A análise univariada entre os grupos com e sem SR não mostrou diferenças em relação às seguintes variáveis: sexo, etnia, escolaridade materna, idade gestacional, peso ao nascer, aleitamento materno, número de crianças no domicílio, frequência em creche, presença de animais de estimação no domicílio e cuidador tabagista. A prevalência de SR foi duas vezes maior entre crianças com displasia broncopulmonar (OR ajustada = 2,08; IC95%: 1,11-3,89; p = 0,022) e quase cinco vezes maior entre aquelas com história pessoal/familiar de atopia (OR ajustada = 4,96; IC95%: 2,62-9,39; p < 0,001) do que entre aquelas sem essas condições. Conclusões: Crianças pré-termo que receberam profilaxia com palivizumabe, mas apresentam história pessoal/familiar de atopia ou displasia broncopulmonar, têm maior probabilidade de apresentar SR do que aquelas sem essas condições.

ABSTRACT Objective: To determine the prevalence of recurrent wheezing (RW) in preterm infants who received prophylaxis against severe infection with respiratory syncytial virus (RSV) and to identify genetic susceptibility (atopy or asthma) and risk factors for RW. Methods: This was a cross-sectional study involving preterm infants who received prophylaxis with palivizumab at a referral center in Brazil during the first two years of age. A structured questionnaire was administered in a face-to-face interview with parents or legal guardians. Results: The study included 410 preterm infants (median age = 9 months [0-24 months]). In the sample as a whole, 111 children (27.1%; [95% CI, 22.9-31.5]) had RW. The univariate analysis between the groups with and without RW showed no differences regarding the following variables: sex, ethnicity, maternal level of education, gestational age, birth weight, breastfeeding, number of children in the household, day care center attendance, pets in the household, and smoking caregiver. The prevalence of RW was twice as high among children with bronchopulmonary dysplasia (adjusted OR = 2.08; 95% CI, 1.11-3.89; p = 0.022) and almost five times as high among those with a personal/family history of atopy (adjusted OR = 4.96; 95% CI, 2.62-9.39; p < 0.001) as among those without these conditions. Conclusions: Preterm infants who received prophylaxis with palivizumab but have a personal/family history of atopy or bronchopulmonary dysplasia are more likely to have RW than do those without these conditions.

Resumo Objetivo: Avaliar sistematicamente estudos que analisaram propriedades psicométricas da impedância intraluminal multicanal associada à pHmetria (IIM-pH) no diagnóstico de refluxo gastroesofágico. Verificar sistematicamente estudos que analisaram as propriedades psicométricas de impedância intraluminal multicanal associada à pHmetria em relação ao diagnóstico da doença do refluxo gastroesofágico em pacientes pediátricos. Métodos: Revisão sistemática que considerou descritores específicos (crianças e adolescentes, doença do refluxo gastroesofágico e impedância intraluminal multicanal associada à pHmetria) e seus sinônimos. A busca sistemática foi realizada nas bases de dados Medline/Ovid, Scopus e Embase e considerou o período de outubro de 2017 a dezembro de 2018. Esta revisão foi realizada de acordo com o checklist Preferred Reporting Items for Systematic Reviews and Meta-Analyses (Prisma), usado para seleção de estudos. As propriedades psicométricas descritas nos estudos foram: sensibilidade, especificidade, acurácia e reprodutibilidade. Resultados: A sensibilidade foi discutida na maioria dos estudos, com um resultado de mais de 70% em quatro deles. A especificidade foi avaliada apenas em conjunto com a sensibilidade, com valores em torno de 23 a 25% em dois estudos, e alcançou 80% em um único estudo. A acurácia foi avaliada em mais de 76% em um dos artigos selecionados. A qualidade metodológica dos estudos demonstrou baixo risco de viés e nenhuma consideração significativa relacionada à aplicabilidade dos estudos. Conclusões: De acordo com os estudos selecionados, a impedância intraluminal multicanal associada à pHmetria foi considerada uma ferramenta segura e eficaz, apresentou valores de sensibilidade superiores à pHmetria no diagnóstico da doença do refluxo gastroesofágico.

Abstract Objective: Systematically assess studies that have analyzed the psychometric properties of multichannel intraluminal impedance-pH for the diagnosis of gastroesophageal reflux. Systematically verify studies that have analyzed the psychometric properties of multichannel intraluminal impedance-pH regarding the diagnosis of gastroesophageal reflux disease in pediatric patients. Methods: Systematic review considering specific descriptors (children and adolescents, gastroesophageal reflux disease and multichannel intraluminal impedance-pH) and their synonyms. The systematic search was carried out in the MEDLINE/Ovid, Scopus, and Embase databases, considering the period from October 2017 to December 2018. This review was conducted according to the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA), which was used for study selection. The psychometric properties described in the studies were: sensitivity, specificity, accuracy, and reproducibility. Results: Sensitivity was discussed in most studies, with a result of more than 70% in four of them. Specificity was assessed only in conjunction with sensitivity, with values from 23% to 25% in two studies, and it reached 80% in a single study. Accuracy was assessed higher than 76% in one of the selected articles. The methodological quality of the studies demonstrated low risk of bias, and there was no significant concern related to applicability of the studies. Conclusions: According to the selected studies, multichannel intraluminal impedance-pH was considered a safe and effective tool, presenting higher sensitivity values than pHmetry regarding the diagnosis of gastroesophageal reflux disease.

Resumo Objetivo Comparar os valores dos marcadores para capnografia volumétrica e espirometria e sua capacidade de classificar crianças e adolescentes com asma, fibrose cística (FC) e controles saudáveis. Métodos Foi realizado um estudo transversal que incluiu 103 pacientes com asma alérgica persistente controlada, 53 com FC e um grupo controle saudável com 40 voluntários (6 a 15 anos), de ambos os sexos. Os indivíduos foram submetidos a capnografia volumétrica e espirometria. Resultados O slope da fase III (SIII), SIII padronizada pelo volume tidal exalado (SIII/VT) e o índice capnográfico (SIII/SII) × 100 (KPIv) foram diferentes entre os três grupos avaliados, com maiores valores para o grupo FC. A relação entre o volume expiratório forçado no primeiro segundo e a capacidade vital forçada (VEF1/CVF) foi o único marcador de espirometria com diferenças nos três grupos. Nos indivíduos com espirometria normal, o KPIv e VEF1/CVF foram diferentes entre os três grupos. A curva ROC diferenciou os indivíduos com asma ou FC daqueles do grupo controle, ambos através da capnografia volumétrica (melhor para identificar a FC em relação aos controles pelo KPIv) e por meio da espirometria (melhor para identificar a asma em relação aos controles). O KPIv foi o melhor parâmetro para distinguir a asma da FC, mesmo em indivíduos com espirometria normal. Conclusão A capnografia volumétrica e a espirometria identificaram diferentes alterações de função pulmonar na asma, na FC e nos controles saudáveis, permitiram que os três grupos fossem diferenciados.

Abstract Objective: To compare the values of the markers for volumetric capnography and spirometry and their ability to classify children and adolescents with asthma, cystic fibrosis (CF), and healthy controls. Methods: This was a cross-sectional study that included 103 patients with controlled persistent allergic asthma, 53 with CF and a healthy control group with 40 volunteers (aged 6 to 15 years), of both sexes. The individuals underwent volumetric capnography and spirometry. Results: Phase III slope (SIII), SIII standardized by exhaled tidal volume (SIII/TV) and capnographic index (SIII/SII) × 100 (KPIv) were different among the three groups assessed, with highest values for CF. The relation between the forced expiratory volume in one second and the forced vital capacity (FEV1/FVC) was the only spirometric marker that presented difference on the three groups. On individuals with normal spirometry, KPIv and FEV1/FVC were different among the three groups. The ROC curve identified the individuals with asthma or CF from the control group, both through volumetric capnography (better to identify CF in relation to the control using KPIv) and through spirometry (better to identify asthma in relation to the control). KPIv was the best parameter to distinguish asthma from CF, even in individuals with normal spirometry. Conclusion: Volumetric capnography and spirometry identified different alterations in lung function on asthma, CF, and healthy controls, allowing the three groups to be distinguished.

RESUMO Objetivo Avaliar a concordância entre as medidas de controle da asma e a capacidade de exercício funcional em crianças e adolescentes com asma não controlada e controlada. Métodos Selecionamos crianças e adolescentes com asma de 7 a 17 anos, que foram atendidos no Ambulatório de Pneumologia Pediátrica da Universidade Estadual de Campinas. Todos os pacientes tiveram nível de controle da asma avaliado pelo questionário da Global Initiative for Asthma (GINAq), Teste de Controle da Asma (TCA), espirometria e teste de caminhada de seis minutos (TC6M). Os pacientes foram classificados como asma não controlada ou controlada em cada teste e a concordância entre as medidas foi avaliada pelas estatísticas do Kappa. A curva ROC foi calculada para o TC6M. O índice espirométrico da espirometria foi composto por FEV1, FEV1/FVC e FEF25-75%. Os resultados da espirometria e do TC6M foram comparados entre o grupo de asma não controlada e controlada pela GINAq. Resultados Dos 138 sujeitos incluídos, 78 (56,5%) eram do sexo masculino com idade média de 11,00 (7-17) anos. O GINAq detectou 68,8% dos pacientes com asma não controlada. Foi observada concordância moderada (p<0,001; k=0,56) e alta especificidade (100%) entre o GINAq e o TCA. No TC6M, o ponto de corte de 82,03% da distância prevista possibilitou a distinção de pacientes com asma controlada e não controlada. O índice espirométrico apresentou 73,4% de sensibilidade com o GINAq. Foram observados resultados piores no TC6M em pacientes com asma não controlada. Conclusões Este estudo destaca a importância de avaliar mais de uma medida para diferenciar o nível de controle da asma. O GINAq identificou mais pacientes com asma não controlada e apresentou concordância moderada com o TCA. O índice espirométrico foi associado à asma não controlada de acordo com o GINAq. O TC6M foi uma boa medida para distinguir pacientes com asma controlada e não controlada.

ABSTRACT Objective To assess the agreement among asthma control measures and functional exercise capacity in children and adolescents with uncontrolled and controlled asthma. Methods Children and adolescents with asthma from 7-17 years old were selected, and they were attended in the “Pediatric Pulmonology Outpatient Clinic of State University of Campinas”, in Brazil. All patients had asthma control level assessed by Global Initiative for Asthma questionnaire (GINAq), Asthma Control Test (ACT), spirometry and six-minute-walk-test (6MWT). Patients were classified as uncontrolled or controlled asthma in each test and agreement among measures was assessed by kappa statistics. The ROC curve was calculated for the 6MWT. The spirometric index obtained from spirometry was composed by FEV1, FEV1/FVC and FEF25-75%. Spirometry and 6MWT results were compared between uncontrolled and controlled asthma group by GINAq. Results Of the 138 subjects included, 78 (56.5%) were male with median age of 11 (7-17) years old. GINAq detected 68.8% of patients with uncontrolled asthma. Moderate agreement (p < 0.001; k = 0.56) and high specificity (100%) was observed between GINAq and ACT. In 6MWT, the cut-off point of 82.03% of predicted distance was able to distinguish patients with controlled and uncontrolled asthma. Spirometric index presented 73.4% of sensitivity according to GINAq. The results for 6MWT in patients with uncontrolled asthma were the worst of all. Conclusion This study highlights the importance of assessing more than one measure to differentiate asthma control level. GINAq identified more patients with uncontrolled asthma and presented moderate agreement with ACT. Spirometric index was associated with uncontrolled asthma according to GINAq. 6MWT was a suitable measure to distinguish patients with controlled and uncontrolled asthma.

Resumo Objetivo: O diagnóstico da fibrose cística depende do valor da concentração de íons de cloreto no teste do suor (≥ 60 mEq/mL - reconhecido como o indicador-padrão para o diagnóstico da doença). Além disso, as glândulas salivares expressam a proteína RTFC igualmente às glândulas sudoríparas. Nesse contexto, nosso objetivo foi verificar a correlação da concentração de cloreto na saliva e a concentração de cloreto no suor e entre a concentração de sódio na saliva e a concentração de sódio no suor em pacientes com fibrose cística e indivíduos controles saudáveis, como uma ferramenta para diagnóstico de fibrose cística. Métodos: Contamos com a participação de 160 indivíduos [57/160 (35,70%) com fibrose cística e duas mutações no gene RTFC conhecidas e 103/160 (64,40%) indivíduos controles saudáveis]. A concentração de íons na saliva foi analisada pelo equipamento ABL 835 da Radiometer® e a concentração de cloreto no suor e sódio no suor, respectivamente, por titulação manual utilizando o método mercurimétrico de Schales & Schales e fotometria de chama. A análise estatística foi realizada pelo teste qui-quadrado, pelo teste de Mann-Whitney e pela correlação de Spearman. Alpha = 0,05. Resultados: Os pacientes com fibrose cística apresentaram maiores valores na concentração de cloreto no suor, concentração de sódio no suor, concentração de cloreto na saliva e concentração de sódio na saliva do que os indivíduos-controle saudáveis (valor de p < 0,001). A correlação entre as concentrações de cloreto na saliva e cloreto no suor mostrou Rho de Spearman (coeficiente de correlação) positivo = 0,475 (IC de 95% = 0,346 a 0,587). Além disso, a correlação entre concentração de sódio na saliva e concentração de sódio no suor mostrou Rho de Spearman positivo = 0,306 (IC de 95% = 0,158 a 0,440). Conclusões: A concentração de cloreto na saliva e a concentração de sódio na saliva são candidatas a ser usadas como diagnóstico de fibrose cística, principalmente em casos em que é difícil atingir a quantidade correta de suor, e/ou o exame da mutação RTFC é difícil e/ou o método de referência para o teste do suor não se encontra disponível ou não é de fácil acesso ao público em geral.

Abstract Objective: Cystic fibrosis diagnosis is dependent on the chloride ion concentration in the sweat test (≥ 60 mEq/mL - recognized as the gold standard indicator for cystic fibrosis diagnosis). Moreover, the salivary glands express the CFTR protein in the same manner as sweat glands. Given this context, the objective was to verify the correlation of saliva chloride concentration and sweat chloride concentration, and between saliva sodium concentration and sweat sodium concentration, in patients with cystic fibrosis and healthy control subjects, as a tool for cystic fibrosis diagnosis. Methods: There were 160 subjects enrolled: 57/160 (35.70%) patients with cystic fibrosis and two known CFTR mutations and 103/160 (64.40%) healthy controls subjects. Saliva ion concentration was analyzed by ABL 835 Radiometer® equipment and, sweat chloride concentration and sweat sodium concentration, respectively, by manual titration using the mercurimetric procedure of Schales & Schales and flame photometry. Statistical analysis was performed by the chi-squared test, the Mann -Whitney test, and Spearman's correlation. Alpha = 0.05. Results: Patients with cystic fibrosis showed higher values of sweat chloride concentration, sweat sodium concentration, saliva chloride concentration, and saliva sodium concentration than healthy controls subjects (p-value < 0.001). The correlation between saliva chloride concentration and sweat chloride concentration showed a positive Spearman's Rho (correlation coefficient) = 0.475 (95% CI = 0.346 to 0.587). Also, the correlation between saliva sodium concentration and sweat sodium concentration showed a positive Spearman's Rho = 0.306 (95% CI = 0.158 to 0.440). Conclusions: Saliva chloride concentration and saliva sodium concentration are candidates to be used in cystic fibrosis diagnosis, mainly in cases where it is difficult to achieve the correct sweat amount, and/or CFTR mutation screening is difficult, and/or reference methods for sweat test are unavailable to implement or are not easily accessible by the general population.

RESUMO Objetivo: Nebulizadores caseiros são usados rotineiramente no tratamento de pacientes com fibrose cística (FC). Este estudo objetiva avaliar a contaminação de nebulizadores utilizados por pacientes de FC que estão cronicamente colonizados por Pseudomonas aeruginosa e a associação da contaminação do nebulizador com a higienização, esterilização e método de secagem. Métodos: Um estudo transversal, observacional, multicêntrico foi conduzido em sete centros de referência de FC no Brasil para obter dados de registros médicos; foram feitas entrevistas estruturadas com os pacientes/cuidadores e partes de nebulizadores (máscara e copo) foram coletados para cultura microbiológica. Resultados: No geral, 77 pacientes com FC foram incluídos. A frequência da contaminação do nebulizador foi de 71,6%. Candida spp. (52,9%), Stenotrophomonas maltophilia (11,9%), P. aeruginosa não mucoide (4,8%), Staphylococcus aureus (4,8%) e complexo Burkholderia cepacia (2.4%) foram os patógenos isolados mais comuns. A frequência de higienização dos nebulizadores foi de 97,4%, e 70,3% dos pacientes relata higienização, esterilização e secagem dos aparelhos. A lavagem com água da torneira e secagem das partes no tempo, em espaço aberto, aumentou significativamente (9 a 10 vezes) a chance de contaminação dos nebulizadores. Conclusões: Apesar dos relatos de frequente higienização dos nebulizadores, os métodos de limpeza e esterilização usados eram inadequados. Uma proporção significativa de nebulizadores foi contaminada com microrganismos potencialmente patogênicos para pacientes com FC. Estes resultados apoiam a necessidade de inclusão dos pacientes/cuidadores em programas educacionais e/ou novas estratégias para fornecimento de antibióticos inalatórios.

ABSTRACT Objective: Home nebulizers are routinely used in the treatment of patients with cystic fibrosis (CF). This study aims to evaluate the contamination of nebulizers used for CF patients, that are chronically colonized by Pseudomonas aeruginosa, and the association of nebulizer contamination with cleaning, decontamination and drying practices. Methods: A cross-sectional, observational, multicenter study was conducted in seven CF reference centers in Brazil to obtain data from medical records, structured interviews with patients/caregivers were performed, and nebulizer’s parts (interface and cup) were collected for microbiological culture. Results: overall, 77 CF patients were included. The frequency of nebulizer contamination was 71.6%. Candida spp. (52.9%), Stenotrophomonas maltophilia (11.9%), non-mucoid P. aeruginosa (4.8%), Staphylococcus aureus (4.8%) and Burkholderia cepacia complex (2.4%) were the most common isolated pathogens. The frequency of nebulizers’ hygiene was 97.4%, and 70.3% of patients reported cleaning, disinfection and drying the nebulizers. The use of tap water in cleaning method and outdoor drying of the parts significantly increased (9.10 times) the chance of nebulizers’ contamination. Conclusion: Despite the high frequency hygiene of the nebulizers reported, the cleaning and disinfection methods used were often inadequate. A significant proportion of nebulizers was contaminated with potentially pathogenic microorganisms for CF patients. These findings support the need to include patients/caregivers in educational programs and / or new strategies for delivering inhaled antibiotics.

RESUMO Objetivo: Verificar as repercussões do teste de esforço submáximo na mecânica respiratória e na função pulmonar de escolares. Métodos: Estudo transversal com escolares de 7 a 14 anos submetidos à avaliação da mecânica respiratória por sistema de oscilometria de impulso (IOS) e da função pulmonar pela espirometria. Realizou-se também o teste de caminhada de seis minutos (TC6), todos segundo os padrões da Sociedade Torácica Americana. O TC6 foi executado duas vezes com intervalo de 30 minutos entre cada teste. O IOS e a espirometria foram feitos antes do primeiro TC6 (pré-TC6) e repetidos imediatamente após o primeiro TC6 (pós-TC61) e após o segundo TC6 (pós-TC62). A comparação dos resultados nos três tempos do estudo se deu por análise de variância para medidas repetidas (teste post-hoc de Bonferroni) ou teste de Friedman, sendo significante p≤0,05. Resultados: Participaram 21 sujeitos; 53% masculinos e idade média de 10,9±2,3 anos. Encontraram-se diferenças entre resistência total (R5) e resistência central das vias aéreas (R20) nos 3 tempos do estudo (p=0,025 e p=0,041, respectivamente). A análise post-hoc indicou aumento de resistência R5 entre pré-TC6 e pós-TC61 (R5=0,540±0,100 versus 0,590±0,150 kPa/L/s, p=0,013; e R20=0,440±0,800 versus 0,470±0,100 kPa/L/s, p=0,038). A única variável espirométrica com alteração no decorrer do tempo foi o fluxo expiratório forçado 25-75% (FEF25-75%) (p=0,003). Conclusões: As repercussões encontradas foram: aumento da resistência total e da resistência central das vias aéreas e redução do FEF25-75% após o TC6 em escolares, sugerindo a necessidade de mais atenção na realização de testes submáximos em crianças com alguma predisposição a alterações das vias aéreas.

ABSTRACT Objective: To verify repercussions of submaximal exercise testing on respiratory mechanics and pulmonary function in schoolchildren. Methods: Cross-sectional study, with children aged 7 to 14 years, who had their respiratory mechanics assessed by impulse oscillometry (IOS), and pulmonary function by spirometry. They performed the six-minute walk test (6MWT), as per the standards by the American Thoracic Society. The 6MWT was performed twice with a 30-minute interval. IOS and spirometry were performed before the first 6MWT (Pre-6MWT) and immediately after the first (Post-6MWT1) and second walking tests (Post-6MWT2). The results in these three phases were compared by analysis of variance for repeated measures (post-hoc Bonferroni test) or by the Friedman’s test, with p≤0.05 considered significant. Results: Twenty-one subjects participated in the study: 53% were males and mean age was 10.9±2.3 years. There were differences between total resistance (R5) and central airway resistance (R20) at the three phases of assessment (p=0.025 and p=0.041, respectively). Post-hoc analysis indicated increase in R5 when Pre-6MWT and Post-6MWT1 were compared (R5=0.540±0.100 versus 0.590±0.150 kPa/L/s, p=0.013; and R20=0.440±0.800 versus 0.470±0.100 kPa/L/s, p=0.038). Forced expiratory flow 25-75% (FEF25-75%) changed over time (p=0.003). Conclusions: Repercussions were: increase in central and total airway resistance and reduction of FEF25-75% after 6MWT in schoolchildren, suggesting that greater attention should be given to submaximal tests in children with predisposition to airways alterations.