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.

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 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.

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.

Introduction: Congenital tracheal stenosis (CTS) is an uncommon but a very important diagnosis because it has an impact in patient's evolution. Objectives: To present the different clinical evolution of 4 patienis with CTS, followed at the Hospital de Niños de San Antonio de Porto Alegre, Brasil. Resulsts: Four cases were studied, 2 with vascular ring, one with asthma and one with respiratory distress during the first year of life. Conclusion: This tracheal malformation is one alternative diagnosis in infants with respiratory disorders. The diagnosis and therapy will depend on the clinical manifestations of the patient

Introducción: La estenosis congénita de tráquea (ECT) es un diagnóstico poco frecuente pero de una gran importancia clínica por el manejo de estos pacientes. Objetivos: Discutir la variabilidad de la presentación clínica y la investigación en ECT, basado en el relato de cuatro casos seguidos en el Servicio de Neumología pediátrica del Hospital de Niños de San Antonio de Porto Alegre. Resultados: Cuatro casos de ECT, siendo dos asociados a anillo vascular; uno con asma de difícil control y otro con disfunción respiratoria en el primer año de vida. Conclusión: La malformación traqueal debe ser recordada en pacientes con síntomas respiratorios poco frecuentes y persistentes. El abordaje diagnóstico y terapéutico debe ser individualizado y el pronóstico dependerá de la forma de presentación anatómica y asociación con otras malformaciones

Objective: To describe clinical characteristics and lung function tests in children with Bronchiolitis Obliterans (B0). Methods: We studied clinical findings by questionnaires, lung function by spirometry and walking test, following published ATS standards. Results: Nineteen patients were studied (7-15 y.o), 73% boys. Ten (53%) had cough, 9 (48%) wheezing, 8 (42‰) bronchial secretions and 26% shortness of breath. Wheezing after exercise was found in 47% of cases. On physical exam we found wheezing in 95% cases, crackles in 90% and digital clubbing in 21%. Spirometry findings were obstructive pattern in 14 cases, and only in 5 patients we found a significant response to bronchodilator. On walking test, total distance was 90-705 m (552 ± 30 m). Oxygen saturation during exercise was 83-99%. We found a drop in SaO2 greater than 4% in 7 patients (37%). Conclusions: A majority of our patients with Bronchiolitis Obliterans had clinical and functional findings that showed a significant obstructive pattem with symptoms, after exercise

Objetivo: Describir el cuadro clínico y funcional de niños con bronquiolitis obliterante (BO). Material y Métodos: Estudio transversal en niños con BO. Datos clínicos obtenidos por cuestionarios. Espirometría y test de caminata siguiendo las normas de la ATS. Resultados: Estudiamos 19 pacientes, 7 a 15 años, 73,7%. Diez (53%) presentaban tos, 9 (48%) sibilancias, 8 (42%) espectoración y 26% "dificultad para respirar". Sibilancias post-ejercicio, 47%, 40% dificultad para realizar ejercicio. Examen físico: Sibilancias 95%, crepitaciones 90% e hipocratismo digital 21%. En la espirometría se encontró alteración ventilatoria obstructiva en 14 pacientes y sólo en 5 hubo respuesta al broncodilatador. En el test de caminata, la distancia recorrida fue de 90 a 705 mts (media 552 ± 30 m). La saturación durante ejercicio fue de 83 a 99%. La caída en la saturación mayor a 4% se presentó en 7 pacientes (37%). Conclusiones: La mayoría de los niños con BO presenta un cuadro clínico y funcional de enfermedad obstructiva y limitaciones al ejercicio