Necrotizing Pneumonia In Children: A Review.

The objective of the review was to determine the long-term outcomes of necrotising pneumonia (NP). Studies published since 1990 in English, Portuguese, or Spanish, published on PubMed and Scielo were evaluated. Our findings showed ultrasound scanning is the diagnostic modality of choice. Despite prolonged hospitalisation (median 13-27 days) and fever (median 9-16 days), most patients recover completely. Empyema and bronchopleural fistulae are frequent in bacterial NP. Streptococcus pneumoniae is the most prevalent cause. Seventeen studies with 497 patients followed for 30 days to 8.75 years showed that most patients were clinically asymptomatic and had normal lung function. X-ray or CT chest imaging demonstrated that almost all lung lesions recovered within 4-6 months. We suggest that it is not necessary to request frequent chest X-rays during the treatment and recovery process. Chest CT scans should be reserved for specific cases not following the expected clinical course.

Surgical treatment of non-cystic fibrosis bronchiectasis in children and adolescents: A review.

OBJECTIVE: To discuss surgery for non-cystic fibrosis [CF] bronchiectasis in children and adolescents. SOURCES: Non-systematic review including articles in English, mainly from the last 5 years. SUMMARY OF FINDINGS: In this review, we present that in low- and middle-income countries [LMIC] clinical treatment fails in around 20% of cases due to low socioeconomic status and poor adherence. This causes the disease to progress and require surgery. We emphasize that the indications for surgery are not well defined and must be considered on an individual basis. The surgical treatment of bronchiectasis in children may be indicated in selected cases; especially in localized disease with frequent exacerbations despite an optimized clinical approach. Surgery can improve quality of life [QoL] and reduce exacerbations. It has few postoperative complications and low morbidity and mortality. Finally, we propose an algorithm for managing bronchiectasis, which takes into account LMIC settings with limited resources. CONCLUSION: We conclude that in LMICs, surgery is a treatment strategy for selected children/adolescents with bronchiectasis.

The wheezy infant: A viewpoint from low-middle income countries.

OBJECTIVE: To review the recent evidence in the literature of various aspects of recurrent/severe wheezing in children under 3 in low-middle income countries [LMICS]. SOURCES: A non-systematic review including articles in English. We mainly selected publications from the last 5 years. Studies on epidemiology, aetiology, diagnosis, treatment, and prevention were included in the search. We reviewed differential diagnoses of wheezing that focused on LMICS. We also reviewed aspects of prevention. SUMMARY OF THE FINDINGS: Many epidemiological studies have shown a variable but significant number of wheezy infants [WI] cases in LMICS when compared to other countries. The differential diagnosis of causes of wheezing in this age group is mandatory, taking into account local facilities. Few treatment options have been well studied for this age group. In LMICS, a pragmatic approach could be considered, as described in the article. It is difficult to study primary prevention for WI and secondary prevention (mainly environmental) may have some impact. A schematic approach for recurrent wheezers is presented, which takes into account settings with limited resources. CONCLUSION: Severely or recurrently wheezy children under 3 is a common clinical issue in LMICS. Studies on this age group are needed to reduce the significant morbidity. It may be possible to lower the high burden of wheezing in this age group by selecting the phenotype which may respond to inhaled steroids.

COVID-19 Prevention Policies Reduce Pediatric Hospital Mortality Due to Community-acquired Pneumonia

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Adverse early-life environmental exposures and their repercussions on adult respiratory health.

OBJECTIVE: To review in the literature the environmental problems in early life that impact the respiratory health of adults. SOURCES: Non-systematic review including articles in English. Search filters were not used in relation to the publication date, but the authors selected mainly publications from the last five years. SUMMARY OF THE FINDINGS: In this review, the authors present the exposure pathways and how the damage occurs depending on the child's stage of development; the authors describe the main environmental pollutants - tobacco smoke, particulate matter, air pollution associated with traffic, adverse childhood experiences and socioeconomic status; the authors present studies that evaluated the repercussions on the respiratory system of adults resulting from exposure to adverse environmental factors in childhood, such as increased incidence of Chronic Obstructive Pulmonary Disease (COPD), asthma and allergies; and, a decline in lung function. The authors emphasize that evidence demonstrates that adult respiratory diseases almost always have their origins in early life. Finally, the authors emphasize that health professionals must know, diagnose, monitor, and prevent toxic exposure among children and women. CONCLUSION: The authors conclude that it is necessary to recognize risk factors and intervene in the period of greatest vulnerability to the occurrence of harmful effects of environmental exposures, to prevent, delay the onset or modify the progression of lung disease throughout life and into adulthood.

Adverse early-life environmental exposures and their repercussions on adult respiratory health

Abstract Objective: To review in the literature the environmental problems in early life that impact the respiratory health of adults. Sources: Non-systematic review including articles in English. Search filters were not used in relation to the publication date, but the authors selected mainly publications from the last five years. Summary of the findings: In this review, the authors present the exposure pathways and how the damage occurs depending on the child's stage of development; the authors describe the main environmental pollutants - tobacco smoke, particulate matter, air pollution associated with traffic, adverse childhood experiences and socioeconomic status; the authors present studies that evaluated the repercussions on the respiratory system of adults resulting from exposure to adverse environmental factors in childhood, such as increased incidence of Chronic Obstructive Pulmonary Disease (COPD), asthma and allergies; and, a decline in lung function. The authors emphasize that evidence demonstrates that adult respiratory diseases almost always have their origins in early life. Finally, the authors emphasize that health professionals must know, diagnose, monitor, and prevent toxic exposure among children and women. Conclusion: The authors conclude that it is necessary to recognize risk factors and intervene in the period of greatest vulnerability to the occurrence of harmful effects of environmental exposures, to prevent, delay the onset or modify the progression of lung disease throughout life and into adulthood.

Impact of non-pharmacological initiatives for COVID-19 on hospital admissions due to pediatric acute respiratory illnesses.

INTRODUCTION: Interventions to deal with the COVID-19 pandemic may impact the burden of other respiratory diseases. The aim of this study is to analyze the impact of non-pharmacological initiatives (NPI) against COVID-19 on the number of hospitalizations due to pediatric acute respiratory illnesses (ARIs). MATERIAL AND METHODS: This is a retrospective analysis of pediatric hospitalizations in Porto Alegre, Brazil. We analyzed the monthly incidence of hospital admissions from 2018 to 2020 due to ARIs included in the study. The time series was divided into the period before introducing NPI (2018 and 2019), and the period when NPI were running (2020). We compared means between the years with Student's t-test. The Dickey-Fuller test was used for secular trend analysis. For seasonality, Fischer's G test was performed. Dynamic linear univariate and multivariate models were used to estimate the association between the predictors (the introduction of NPI, secular trend, and seasonality) and outcome (the incidence of ARI admissions). For the statistical analysis, the cut-off probability for rejecting the null hypothesis was defined as <5%. RESULTS: From 2018 to 2020, 10,109 hospital admissions were due to the respiratory causes included in this study. There was a significant decrease in 2020 in the mean incidence of the ARIs studied compared with 2018 and 2019. The number of hospitalizations due to respiratory diseases in children decreased by 64% for asthma and 93% for bronchiolitis. A secular trend of monthly admissions rates due to ARIs was only observed in the laryngotracheitis data (p = 0.485), but seasonality was detected in all analyses. According to the univariate and multivariate analysis, the introduction of NPI was associated with a decrease in the incidence of ARI admissions. CONCLUSION: There was a significant reduction in hospital admissions due to ARIs in children. Our data suggest a significant impact of NPI on reducing the spread of viruses associated with ARIs in children. These results support respiratory illness prevention strategies.

Cystic Fibrosis: A Simple and Customized Strategy for Genetic Screening Able to Detect Over 90% of Identified Mutated Alleles in Brazilian Newborns.

INTRODUCTION: The incorporation of molecular genetic testing into cystic fibrosis (CF) screening programs increases the specificity of the diagnostic strategy and has the potential to decrease the rate of false- positive results. In this sense, our objective was to develop a genotyping assay that could detect 25 pathogenic variants in the cystic fibrosis transmembrane conductance regulator (CFTR) gene with high sensitivity and that could be incorporated into the routine of newborn screening, complementing the current existing protocol used in our public health institution. METHODS: A mini-sequencing assay was standardized using single-base extension in a previously genotyped control sample. This strategy was validated in a Brazilian cohort of CF patients by Sanger sequencing. RESULTS: The inclusion of the 25 variants in the current newborn screening program increased the identification rates of two alleles from 33 to 52.43% in CF patients. This new approach was able to detect a total of 37 variants, which represents 93.01% of all mutated alleles described in the last CF Brazilian Register. CONCLUSIONS: Mini-sequencing for the simultaneous detection of 25 CFTR gene variants improves the screening of Brazilian newborns and decreases the number of inconclusive cases. This method uses minimal hands-on time and is suited for rapid screening, which reduces sample processing costs.

Health-related quality of life in post-infectious bronchiolitis obliterans: agreement between children and their proxy / Qualidade de vida relacionada à saúde na bronquiolite obliterante pós-infecciosa: concordância entre as crianças e seus cuidadores

Abstract Objective: To assess the level of agreement in health-related quality of life between children with Post-infectious Bronchiolitis Obliterans and their parent (so-called proxy). Methods: Participants aged between 8and 17 years who had been previously diagnosed with Post-infectious Bronchiolitis Obliterans were regularly followed up at a pediatric pulmonology outpatient clinic. Parents or legal guardians (caregivers) of these patients were also recruited for the study. A validated and age-appropriate version of the Pediatric Quality of Life Inventory 4.0 was used for the assessment of health-related quality of life. Caregivers completed the corresponding proxy versions of the questionnaire. The correlation between self and proxy reports of health-related quality of life was determined by intra-class correlation coefficient and dependent t-tests. Results: The majority of participants were males (79.4%), and the average age was 11.8 years. Intra-class correlations between each of the Pediatric Quality of Life Inventory 4.0 domains and the total score were all lower than 0.6, with a range between 0.267 (poor) and 0.530 (fair). When the means of each domain and the total score of the questionnaires were compared, caregivers were observed to have a significantly lower health-related quality of life score than children, with the exception of the social domain in which the difference was not significant. However, the differences in score exceeded the critical threshold difference of four points in all other domains. Conclusion: Proxies of children and adolescents with Post-infectious Bronchiolitis Obliterans appear to consistently perceive their children as having lower health-related quality of life than how the patients perceive themselves.

Resumo: Objetivo: Avaliar o nível de concordância a respeito da qualidade de vida relacionada à saúde entre crianças com bronquiolite obliterante pós-infecciosa e seus pais (responsáveis). Métodos: Os participantes entre 8-17 anos, anteriormente diagnosticados com bronquiolite obliterante pós-infecciosa, foram acompanhados regularmente no ambulatório de pneumologia pediátrica. Os pais ou responsáveis legais (cuidadores) desses pacientes também foram convidados a participar do estudo. Uma versão validada e adequada para a idade do Inventário Pediátrico de Qualidade de Vida, versão 4.0, foi utilizada para a avaliação da qualidade de vida relacionada à saúde. Os cuidadores concluíram as versões do questionário correspondentes aos responsáveis. A correlação entre os autorrelatos e os relatos dos responsáveis da qualidade de vida relacionada à saúde foi determinada pelo coeficiente de correlação intraclasse e pelos testes t dependentes. Resultados: A maior parte dos participantes era do sexo masculino (79,4%) e a idade média foi 11,8 anos. As correlações intraclasse entre cada um dos domínios do Inventário Pediátrico de Qualidade de Vida e o escore total foram todas inferiores a 0,6, com intervalo entre 0,267 (baixo) e 0,530 (justo). Quando as médias de cada domínio e o escore total dos questionários foram comparados, observamos que os cuidadores apresentaram qualidade de vida relacionada à saúde significativamente menor em comparação com as crianças, com exceção do domínio social, no qual a diferença não foi significativa. Contudo, as diferenças no escore ultrapassaram o limite de diferença essencial de 4 pontos em todos os outros domínios. Conclusão: Os responsáveis pelas crianças e adolescentes com bronquiolite obliterante pós-infecciosa parecem perceber de forma consistente que suas crianças possuem qualidade de vida relacionada à saúde menor que os próprios pacientes.

Health-related quality of life in post-infectious bronchiolitis obliterans: agreement between children and their proxy.

OBJECTIVE: To assess the level of agreement in health-related quality of life between children with Post-infectious Bronchiolitis Obliterans and their parent (so-called proxy). METHODS: Participants aged between 8and 17 years who had been previously diagnosed with Post-infectious Bronchiolitis Obliterans were regularly followed up at a pediatric pulmonology outpatient clinic. Parents or legal guardians (caregivers) of these patients were also recruited for the study. A validated and age-appropriate version of the Pediatric Quality of Life Inventory 4.0 was used for the assessment of health-related quality of life. Caregivers completed the corresponding proxy versions of the questionnaire. The correlation between self and proxy reports of health-related quality of life was determined by intra-class correlation coefficient and dependent t-tests. RESULTS: The majority of participants were males (79.4%), and the average age was 11.8 years. Intra-class correlations between each of the Pediatric Quality of Life Inventory 4.0 domains and the total score were all lower than 0.6, with a range between 0.267 (poor) and 0.530 (fair). When the means of each domain and the total score of the questionnaires were compared, caregivers were observed to have a significantly lower health-related quality of life score than children, with the exception of the social domain in which the difference was not significant. However, the differences in score exceeded the critical threshold difference of four points in all other domains. CONCLUSION: Proxies of children and adolescents with Post-infectious Bronchiolitis Obliterans appear to consistently perceive their children as having lower health-related quality of life than how the patients perceive themselves.

Childhood asthma in low and middle-income countries: Where are we now?

Pediatric asthma has been increasing in LMICs (Low Middle-Income Countries), leading to an important burden for both children and national health systems. Implementing measures to achieve control are influenced by the degree of organization health systems have, the availability and affordability of essential asthma medications, and the effective implementation of asthma programs and asthma guidelines. In this review authors give an updated view of the current situation of these components of asthma management in LMICs.

Health-related quality of life in patients with bronchiolitis obliterans / Qualidade de vida relacionada à saúde em pacientes com bronquiolite obliterante

Abstract Objective: To evaluate the overall health-related quality of life in patients with bronchiolitis obliterans. Methods: Participants with a diagnosis of post-infectious bronchiolitis obliterans, who were being followed-up at two specialized outpatient clinics of Pediatric Pulmonology in Porto Alegre, Brazil, and controls aged between 8 and 17 years, of both genders, were included in the study. Controls were paired by gender, age, and socioeconomic level in relation to the group of participants with post-infectious bronchiolitis obliterans. The version of the Pediatric Quality of Life Inventory (PedsQ) tool validated for Brazil was applied for the assessment of Health-related Quality of Life, through an interview. The comparison of the Health-related Quality of Life means between the groups was performed using Student's t-test for independent samples and the chi-squared test, for categorical variables. Results: 34 patients diagnosed with post-infectious bronchiolitis obliterans and 34 controls participated in the study. The mean age of the children included in the study was 11.2 ± 2.5 years, and 49 (72%) of them were males. The groups showed no significant differences in relation to these variables. The quality of life score was significantly and clinically lower in the post-infectious bronchiolitis obliterans group when compared with controls in the health (72.36 ± 15.6, 81.06 ± 16.4, p = 0.031) and school domains (62.34 ± 20.7, 72.94 ± 21.3, p = 0.043), as well as in the total score (69.53 ± 14.9, 78.02 ± 14.8, p = 0.024), respectively. Conclusion: Patients with post-infectious bronchiolitis obliterans presented lower health-related quality of life scores when compared with healthy individuals in the total score and in the health and school domains.

Resumo Objetivo: Avaliar a qualidade de vida relacionada à saúde geral em participantes com bronquiolite obliterante. Métodos: Foram incluídos no estudo participantes com diagnóstico de bronquiolite obliterante pós-infecciosa que estavam em acompanhamento em dois ambulatórios especializados de pneumologia pediátrica em Porto Alegre, Brasil e controles, entre 8 e 17 anos, de ambos os sexos. Os controles foram pareados por sexo, idade e nível socioeconômico em relação ao grupo de participantes com bronquiolite obliterante pós-infecciosa. Para avaliação da Qualidade de Vida Relacionada à Saúde geral foi aplicado a versão validada para o Brasil do instrumento PedsQL (Pediatric Quality of Life Inventory), por meio de entrevista. A comparação entre as médias da Qualidade de Vida Relacionada à Saúde entre os grupos foi realizada mediante o teste t para amostras independentes e para as variáveis categóricas por teste qui-quadrado. Resultados: Participaram do estudo 34 pacientes com diagnóstico de bronquiolite obliterante pós-infecciosa e 34 controles. A média da idade das crianças incluídas foi de 11,2 ± 2,5 anos e 49 (72%) deles eram do sexo masculino. Os grupos não apresentaram diferenças significativas em relação a essas variáveis. O escore de qualidade de vida foi significativamente e clinicamente menor no grupo bronquiolite obliterante pós-infecciosa em comparação com o controle nos domínios saúde: (72,36 ± 15,6; 81,06 ± 16,4; p = 0,031); escolar: (62,34 ± 20,7; 72,94 ± 21,3; p = 0,043) e no escore total (69,53 ± 14,9; 78,02 ± 14,8, p = 0,024), respectivamente. Conclusão: Os pacientes com bronquiolite obliterante pós-infecciosa apresentam escores de qualidade de vida relacionados à saúde menor do que indivíduos saudáveis no escore total e nos domínios saúde e escolares.

When should parapneumonic pleural effusions be drained in children?

Pneumonia is an important health problem in children, and parapneumonic pleural effusion (PPE) is a frequent complication. There is no standard strategy for treating PPE, reflected in the few international guidelines that have been published on the issue. Compared to adults, there is no consensus on the utility of pleural fluid analysis in paediatric PPE. This is because of the lack of good evidence either in favour or against it and the risks of procedural sedation for acquiring pleural fluid for analysis to guide management. In this paper we provide a succinct review of the different approaches to the management of PPE, including diagnosis, pleural fluid analysis (Light's criteria) and treatment, both medical and surgical.

Health-related quality of life in patients with bronchiolitis obliterans.

OBJECTIVE: To evaluate the overall health-related quality of life in patients with bronchiolitis obliterans. METHODS: Participants with a diagnosis of post-infectious bronchiolitis obliterans, who were being followed-up at two specialized outpatient clinics of Pediatric Pulmonology in Porto Alegre, Brazil, and controls aged between 8 and 17 years, of both genders, were included in the study. Controls were paired by gender, age, and socioeconomic level in relation to the group of participants with post-infectious bronchiolitis obliterans. The version of the Pediatric Quality of Life Inventory (PedsQ) tool validated for Brazil was applied for the assessment of Health-related Quality of Life, through an interview. The comparison of the Health-related Quality of Life means between the groups was performed using Student's t-test for independent samples and the chi-squared test, for categorical variables. RESULTS: 34 patients diagnosed with post-infectious bronchiolitis obliterans and 34 controls participated in the study. The mean age of the children included in the study was 11.2±2.5 years, and 49 (72%) of them were males. The groups showed no significant differences in relation to these variables. The quality of life score was significantly and clinically lower in the post-infectious bronchiolitis obliterans group when compared with controls in the health (72.36±15.6, 81.06±16.4, p=0.031) and school domains (62.34±20.7, 72.94±21.3, p=0.043), as well as in the total score (69.53±14.9, 78.02±14.8, p=0.024), respectively. CONCLUSION: Patients with post-infectious bronchiolitis obliterans presented lower health-related quality of life scores when compared with healthy individuals in the total score and in the health and school domains.

Evaluating bronchodilator response in pediatric patients with post-infectious bronchiolitis obliterans: use of different criteria for identifying airway reversibility.

OBJECTIVE: Post-infectious bronchiolitis obliterans (PIBO) is a clinical entity that has been classified as constrictive, fixed obstruction of the lumen by fibrotic tissue. However, recent studies using impulse oscillometry have reported bronchodilator responses in PIBO patients. The objective of this study was to evaluate bronchodilator responses in pediatric PIBO patients, comparing different criteria to define the response. METHODS: We evaluated pediatric patients diagnosed with PIBO and treated at one of two pediatric pulmonology outpatient clinics in the city of Porto Alegre, Brazil. Spirometric parameters were measured in accordance with international recommendations. RESULTS: We included a total of 72 pediatric PIBO patients. The mean pre- and post-bronchodilator values were clearly lower than the reference values for all parameters, especially FEF25-75%. There were post-bronchodilator improvements. When measured as mean percent increases, FEV1 and FEF25-75%, improved by 11% and 20%, respectively. However, when the absolute values were calculated, the mean FEV1 and FEF25-75% both increased by only 0.1 L. We found that age at viral aggression, a family history of asthma, and allergy had no significant effects on bronchodilator responses. CONCLUSIONS: Pediatric patients with PIBO have peripheral airway obstruction that is responsive to treatment but is not completely reversible with a bronchodilator. The concept of PIBO as fixed, irreversible obstruction does not seem to apply to this population. Our data suggest that airway obstruction is variable in PIBO patients, a finding that could have major clinical implications. OBJETIVO: A bronquiolite obliterante pós-infecciosa (BOPI) é uma entidade clínica que tem sido classificada como obstrução fixa e constritiva do lúmen por tecido fibrótico. Entretanto, estudos recentes utilizando oscilometria de impulso relataram resposta ao broncodilatador em pacientes com BOPI. O objetivo deste estudo foi avaliar a resposta broncodilatadora em pacientes pediátricos com BOPI, comparando critérios diferentes para a definição da resposta. MÉTODOS: Foram avaliados pacientes pediátricos com diagnóstico de BOPI tratados em um de dois ambulatórios de pneumologia pediátrica na cidade de Porto Alegre (RS). Parâmetros espirométricos foram medidos de acordo com recomendações internacionais. RESULTADOS: Foram incluídos 72 pacientes pediátricos com BOPI no estudo. As médias dos valores pré- e pós-broncodilatador foram claramente inferiores aos valores de referência para todos os parâmetros, especialmente FEF25-75%. Houve uma melhora pós-broncodilatador. Quando medidos como aumentos percentuais médios, VEF1 e FEF25-75% melhoraram em 11% e 20%, respectivamente. Entretanto, quando os valores absolutos foram calculados, as médias de VEF1 e FEF25-75% aumentaram somente em 0,1 l. Verificamos que a idade da agressão viral, história familiar de asma e alergia não tiveram efeitos significativos na resposta ao broncodilatador. CONCLUSÕES: Pacientes pediátricos com BOPI têm uma obstrução das vias aéreas periféricas que responde ao tratamento, mas não uma reversão completa com o broncodilatador. O conceito de BOPI como obstrução fixa e irreversível parece não se aplicar a essa população. Nossos dados sugerem que a obstrução de vias aéreas em pacientes com BOPI é variável, e esse achado pode ter importantes implicações clínicas.

Evaluating bronchodilator response in pediatric patients with post-infectious bronchiolitis obliterans: use of different criteria for identifying airway reversibility / Avaliação da resposta ao broncodilatador em pacientes pediátricos com bronquiolite obliterante pós-infecciosa: uso de diferentes critérios de identificação de reversibilidade das vias aéreas

ABSTRACT Objective: Post-infectious bronchiolitis obliterans (PIBO) is a clinical entity that has been classified as constrictive, fixed obstruction of the lumen by fibrotic tissue. However, recent studies using impulse oscillometry have reported bronchodilator responses in PIBO patients. The objective of this study was to evaluate bronchodilator responses in pediatric PIBO patients, comparing different criteria to define the response. Methods: We evaluated pediatric patients diagnosed with PIBO and treated at one of two pediatric pulmonology outpatient clinics in the city of Porto Alegre, Brazil. Spirometric parameters were measured in accordance with international recommendations. Results: We included a total of 72 pediatric PIBO patients. The mean pre- and post-bronchodilator values were clearly lower than the reference values for all parameters, especially FEF25-75%. There were post-bronchodilator improvements. When measured as mean percent increases, FEV1 and FEF25-75%, improved by 11% and 20%, respectively. However, when the absolute values were calculated, the mean FEV1 and FEF25-75% both increased by only 0.1 L. We found that age at viral aggression, a family history of asthma, and allergy had no significant effects on bronchodilator responses. Conclusions: Pediatric patients with PIBO have peripheral airway obstruction that is responsive to treatment but is not completely reversible with a bronchodilator. The concept of PIBO as fixed, irreversible obstruction does not seem to apply to this population. Our data suggest that airway obstruction is variable in PIBO patients, a finding that could have major clinical implications.

RESUMO Objetivo: A bronquiolite obliterante pós-infecciosa (BOPI) é uma entidade clínica que tem sido classificada como obstrução fixa e constritiva do lúmen por tecido fibrótico. Entretanto, estudos recentes utilizando oscilometria de impulso relataram resposta ao broncodilatador em pacientes com BOPI. O objetivo deste estudo foi avaliar a resposta broncodilatadora em pacientes pediátricos com BOPI, comparando critérios diferentes para a definição da resposta. Métodos: Foram avaliados pacientes pediátricos com diagnóstico de BOPI tratados em um de dois ambulatórios de pneumologia pediátrica na cidade de Porto Alegre (RS). Parâmetros espirométricos foram medidos de acordo com recomendações internacionais. Resultados: Foram incluídos 72 pacientes pediátricos com BOPI no estudo. As médias dos valores pré- e pós-broncodilatador foram claramente inferiores aos valores de referência para todos os parâmetros, especialmente FEF25-75%. Houve uma melhora pós-broncodilatador. Quando medidos como aumentos percentuais médios, VEF1 e FEF25-75% melhoraram em 11% e 20%, respectivamente. Entretanto, quando os valores absolutos foram calculados, as médias de VEF1 e FEF25-75% aumentaram somente em 0,1 l. Verificamos que a idade da agressão viral, história familiar de asma e alergia não tiveram efeitos significativos na resposta ao broncodilatador. Conclusões: Pacientes pediátricos com BOPI têm uma obstrução das vias aéreas periféricas que responde ao tratamento, mas não uma reversão completa com o broncodilatador. O conceito de BOPI como obstrução fixa e irreversível parece não se aplicar a essa população. Nossos dados sugerem que a obstrução de vias aéreas em pacientes com BOPI é variável, e esse achado pode ter importantes implicações clínicas.

Profile of cystic fibrosis in two reference centers in southern Brazil.

OBJECTIVE: to compare the characteristics of cystic fibrosis patients treated in two reference centers in southern Brazil in order to observe trends in the treatment and clinical outcomes that may produce changes in clinical conduct. METHODS: cross-sectional, retrospective study with 83 patients diagnosed with cystic fibrosis, aged one month to eighteen years. The variables analyzed were obtained through review of medical records, including: demographic and clinical characteristics, socioeconomic status, pulmonary function test, bacterial colonization profile, medication and physiotherapy. RESULTS: between the two centers there was significant difference in the variables, including admissions in the previous year (p<0.001), lifetime hospital admissions (p<0.001), use of Dornase alpha (p=0.003) and inhaled antibiotic therapy (p=0.006), which were higher at the Santo Antônio Children's Hospital (HCSA), while age at first colonization with Staphylococcus aureus (p=0.008), maternal age (p=0.030), clinical score (p=0.001), socioeconomic score (p=0.021) and use of hypertonic saline (p<0.001) were lower at HCSA compared to São Lucas Hospital (HSL). CONCLUSION: the study centers seem to receive a different population of patients, both in socioeconomic terms, as well as disease severity, which interferes with the choice of medication treatment. At the HCSA, preventive actions against infection due to the high incidence of Burkholderia cepacia Complex and careful research into early lung changes will be encouraged.

Profile of cystic fibrosis in two reference centers in southern Brazil / Perfil da fibrose cística em dois centros de referência do sul do Brasil

Summary Objective: to compare the characteristics of cystic fibrosis patients treated in two reference centers in southern Brazil in order to observe trends in the treatment and clinical outcomes that may produce changes in clinical conduct. Methods: cross-sectional, retrospective study with 83 patients diagnosed with cystic fibrosis, aged one month to eighteen years. The variables analyzed were obtained through review of medical records, including: demographic and clinical characteristics, socioeconomic status, pulmonary function test, bacterial colonization profile, medication and physiotherapy. Results: between the two centers there was significant difference in the variables, including admissions in the previous year (p<0.001), lifetime hospital admissions (p<0.001), use of Dornase alpha (p=0.003) and inhaled antibiotic therapy (p=0.006), which were higher at the Santo Antônio Children’s Hospital (HCSA), while age at first colonization with Staphylococcus aureus (p=0.008), maternal age (p=0.030), clinical score (p=0.001), socioeconomic score (p=0.021) and use of hypertonic saline (p<0.001) were lower at HCSA compared to São Lucas Hospital (HSL). Conclusion: the study centers seem to receive a different population of patients, both in socioeconomic terms, as well as disease severity, which interferes with the choice of medication treatment. At the HCSA, preventive actions against infection due to the high incidence of Burkholderia cepacia Complex and careful research into early lung changes will be encouraged. .

Resumo Objetivo: comparar o perfil de pacientes com fibrose cística em dois centros de referência do Sul do Brasil – Hospital da Criança Santo Antônio (HCSA) e Hospital São Lucas – para observar tendências no tratamento e desfechos clínicos que produzirão possíveis modificações de conduta. Métodos: estudo transversal, retrospectivo, com 83 pacientes com fibrose cística, de idade entre 1 mês e 18 anos. As variáveis analisadas, obtidas por meio da revisão de prontuários, foram: características demográficas, clínicas e socioeconômicas, teste de função pulmonar, perfil de infecção, tratamento medicamentoso e fisioterápico. Resultados: entre os dois centros, houve diferença estatisticamente significativa nas variáveis a seguir: internações no último ano (p<0,001), internações na vida (p<0,001), uso de dornase α (p=0,003) e uso de antibioticoterapia inalatória (p=0,006) foram maiores no HCSA enquanto idade da primeira colonização por Staphylococcus aureus (p=0,008), idade da mãe (p=0,030), escore clínico (p=0,001), escore socioeconômico (p=0,021) e uso de solução salina hipertônica (p<0,001) foram maiores no Hospital São Lucas. Conclusão: os centros estudados parecem receber uma população distinta de pacientes, tanto do ponto de vista socioeconômico quanto em relação à gravidade da doença, o que interfere na escolha da terapia medicamentosa utilizada. No HCSA, serão estimuladas ações preventivas de infecção, por causa da alta incidência do complexo Burkholderia cepacia, e uma investigação atenta para alterações pulmonares mais precoces. .

CT densitovolumetry in children with obliterative bronchiolitis: correlation with clinical scores and pulmonary function test results / Densitovolumetria pulmonar por TC em criancas com bronquiolite obliterante: correlacao com escores clinicos e testes de funcao pulmonar

OBJECTIVE: To determine whether air trapping (expressed as the percentage of air trapping relative to total lung volume [AT%]) correlates with clinical and functional parameters in children with obliterative bronchiolitis (OB). METHODS: CT scans of 19 children with OB were post-processed for AT% quantification with the use of a fixed threshold of −950 HU (AT%950) and of thresholds selected with the aid of density masks (AT%DM). Patients were divided into three groups by AT% severity. We examined AT% correlations with oxygen saturation (SO2) at rest, six-minute walk distance (6MWD), minimum SO2 during the six-minute walk test (6MWT_SO2), FVC, FEV1, FEV1/FVC, and clinical parameters. RESULTS: The 6MWD was longer in the patients with larger normal lung volumes (r = 0.53). We found that AT%950 showed significant correlations (before and after the exclusion of outliers, respectively) with the clinical score (r = 0.72; 0.80), FVC (r = 0.24; 0.59), FEV1 (r = −0.58; −0.67), and FEV1/FVC (r = −0.53; r = −0.62), as did AT%DM with the clinical score (r = 0.58; r = 0.63), SO2 at rest (r = −0.40; r = −0.61), 6MWT_SO2 (r = −0.24; r = −0.55), FVC (r = −0.44; r = −0.80), FEV1 (r = −0.65; r = −0.71), and FEV1/FVC (r = −0.41; r = −0.52). CONCLUSIONS: Our results show that AT% correlates significantly with clinical scores and pulmonary function test results in children with OB. .

OBJETIVO: Determinar as correlações entre o volume de aprisionamento aéreo em relação ao volume pulmonar total (AA%) e parâmetros clínicos e funcionais em crianças com bronquiolite obliterante (BO). MÉTODOS: Técnicas de pós-processamento de imagem foram usadas em imagens de TC de 19 crianças com BO para quantificar AA% por meio de um limiar fixo de −950 UH (AA%950) e de limiares selecionados por meio de máscaras de densidade (AA%MD). Os pacientes foram divididos em três grupos, de acordo com a gravidade de AA%. Foram examinadas as correlações entre AA% e a saturação de oxigênio (SO2) em repouso, a distância percorrida no teste de caminhada de seis minutos (DTC6), a SO2 mínima durante o teste de caminhada de seis minutos (SO2_TC6), a CVF, o VEF1, a relação VEF1/CVF e parâmetros clínicos. RESULTADOS: A DTC6 foi maior nos pacientes com maiores volumes pulmonares normais (r = 0,53). Na amostra como um todo, encontramos (antes e depois da exclusão de valores extremos, respectivamente), correlações estatisticamente significativas entre AA%950 e o escore clínico (r = 0,72; 0,80), a CVF (r = 0,24; 0,59), o VEF1 (r = −0,58; −0,67) e a relação VEF1/CVF (r = −0,53; r = −0,62), bem como entre AA%MD e o escore clínico (r = 0,58; r = 0,63), a SO2 em repouso (r = −0,40; r = −0,61), a SO2_TC6 (r = −0,24; r = −0,55), a CVF (r = −0,44; r = −0,80), o VEF1 (r = −0,65; r = −0,71) e a relação VEF1/CVF (r = −0,41; r = −0,52). CONCLUSÕES: Os resultados deste estudo mostram que AA% correlaciona-se significativamente com escores clínicos e testes de função pulmonar em crianças com BO. .