Successful treatment of postinfectious bronchiolitis obliterans with gamma globulin in a tertiary center: 10 years of experience.

INTRODUCTION: Bronchiolitis obliterans is characterized by partial or total occlusion of the bronchioles due to inflammation and fibrosis, and the most common form is postinfectious bronchiolitis obliterans (PIBO). This study aimed to retrospectively present our intravenous immunoglobulin (IVIG) treatment experience in PIBO patients with a clinically severe course despite receiving commonly used treatment protocols. MATERIALS AND METHODS: The study included patients aged 0-18 with subtle immunological abnormalities who were followed up in our center for PIBO between 2010 and 2021. Clinical evaluation, body mass index (BMI), computerized tomography (CT) image scoring, and immunological parameters were recorded before and after IVIG treatment. RESULTS: Of the 11 patients included in the study, 90% were male, the mean age at diagnosis was 27.1 months (range: 5-68 months) and the mean current age was 81.4 months (range: 15-188 months). The number of hospital visits due to infection and the frequency of hospitalizations decreased markedly in the patients who underwent IVIG therapy. Oxygen therapy was discontinued in all patients, and improvements in radiological severity scores were observed. BMI z-scores improved over the baseline values after IVIG therapy. CONCLUSION: Corticosteroids are considered the best first-line treatment to control inflammation in PIBO. In our study group, PIBO patients showed favorable clinical and radiological responses to regular IVIG treatment, possibly due to minor immune deficiency secondary to steroids or as a result of undetected adaptive and innate immune defects involved in the etiology of severe PIBO.

TGFß1 and POSTN as biomarkers of postinfectious bronchiolitis obliterans and asthma in children.

RATIONALE: Asthma and postinfectious bronchiolitis obliterans (PIBO) are common chronic lung diseases in company with wheezing in children. However, it is not clear what is common and unique mechanisms between the two diseases. Thus, we used proteomic analysis to compare differences in biomarkers between children with asthma and PIBO. METHODS: Overall, 30 healthy children without respiratory underlying diseases, 18 children with asthma and 15 with PIBO were included for this study. Sequential window acquisition of all theoretical mass spectra (SWATH)-mass spectrometry (MS) was used to measure proteins in plasma samples. To identify specific pathways of each groups, we used the ingenuity pathway analysis (IPA) software. RESULTS: We identified and quantified 354 proteins across all 63 samples in the SWATH-MS analysis. Forty eight proteins were significantly different among 3 groups. The upstream analysis of IPA suggested that inhibitor of nuclear factor kappa B kinase subunit beta (IKBKB) was the upstream inhibitor of 4 differentially expressed proteins (DEPs) in asthma, while the upstream activator in PIBO subjects. Among 4 DEPs, TGF-ß1 in PIBO and periostin in asthma were negatively correlated with forced expiratory volume in 1 s (FEV1), FEV1/forced vital capacity, maximal med-expiratory flow, and PC20, respectively. CONCLUSION: These findings demonstrate that transforming growth factor ß1 and periostin were unique biomarkers of PIBO and asthma in children, respectively. The mechanism regulated by IKBKB may be therapeutically relevant for PIBO and asthma.

Lactate dehydrogenase and the severity of adenoviral pneumonia in children: A meta-analysis.

Background: Children with severe adenoviral pneumonia (ADVP) have poor prognosis and high risk of mortality. We performed a meta-analysis to evaluate the association between pretreatment lactate dehydrogenase (LDH) and severity, postinfectious bronchiolitis obliterans (PIBO), and mortality in children with ADVP. Methods: Relevant observational studies were identified by search of PubMed, Embase, Web of Science, Wanfang, and CNKI databases from inception to August 3, 2022. A random effect model was used to pool the results by incorporating the potential between-study heterogeneity. Results: Overall, 23 studies with 4,481 children with ADVP were included in this meta-analysis. Results of meta-analysis showed that children with severe ADVP had a significantly higher level of pretreatment LDH as compared to those with non-severe ADVP (standard mean difference [SMD]: 0.51, 95% confidence interval [CI]: 0.36 to 0.66, p < 0.001; I 2 = 69%). Besides, pooled results also suggested that the pretreatment LDH was significantly higher in children who developed PIBO as compared to those who did not (SMD: 0.47, 95% CI: 0.09 to 0.84, p = 0.02, I 2 = 80%). Finally, results of the meta-analysis also confirmed that a higher pretreatment LDH (>500 IU/L) was a risk factor of increased mortality during hospitalization (odds ratio: 3.10, 95% CI: 1.62 to 5.92, p < 0.001, I 2 = 0%). Sensitivity analyses by excluding one dataset at a time showed consistent results. Conclusion: High pretreatment LDH may be associated with disease severity, development of PIBO, and increased risk of mortality in children with ADVP.

Longitudinal Assessment of Pulmonary Function and Bronchodilator Response in Pediatric Patients With Post-infectious Bronchiolitis Obliterans.

Backgroud: Postinfectious bronchiolitis obliterans (PIBO) is a rare respiratory disease. In recent years, the disease has been recognized and diagnosed increasingly in children. Pulmonary function is important for diagnosis, identifying the severity of the PIBO and monitoring progression. But there have been only a few studies that followed the evolution of PIBO on the basis of pulmonary function tests (PFTs). Objective: The study targeted the evolution of pulmonary function and bronchodilator response in a case series of Chinese children with PIBO. Methods: Twelve children between the ages of 6-99 months with PIBO were studied retrospectively from 2009 to 2019. Forced vital capacity (FVC), forced expiratory volume in 1 s (FEV1), the FEV1/FVC ratio, and maximal midexpiratory flow velocity 25-75% (MMEF25-75%) were collected at each PFT, and bronchodilator responses were evaluated. Spirometric parameters were monitored over time, and generalized linear mixed models were used to analyze longitudinal panel data. Results: The median baseline PFT values for FVC, FEV1, the FEV1/FVC ratio, and MMEF25-75% were 41.6, 39.75, 90.7, and 22.2%, respectively. At the initial PFTs, 10 (83.3%) patients demonstrated a significant bronchodilator response. FVC and FEV1 increased by 8.212%/year and 5.007%/year, respectively, and the FEV1/FVC ratio decreased by an average of 3.537%/year. MMEF25-75% showed improvement at an average rate of 1.583% every year. Overall, FEV1 and MMEF25-75% showed different degrees of improvement after the use of inhaled bronchodilators at each PFT session for 10 patients, and FEV1 measures demonstrated significant (>12%) ß2-bronchodilation in 56% of PFT sessions. Conclusions: Pediatric patients with PIBO showed an obstructive defect in pulmonary function. The FVC, FEV1, and MMEF25-75% improved as they grew older, while the FEV1/FVC ratio decreased. This may be due to the development of lung parenchyma more than airway growth. Airway obstruction in some patients improved with the use of ß2 agonists.

Long-term course of bronchial inflammation and pulmonary function testing in children with postinfectious bronchiolitis obliterans.

RATIONALE: Postinfectious bronchiolitis obliterans (PIBO) is a rare, chronic respiratory condition, which follows an acute insult due to a severe infection of the lower airways. OBJECTIVES: The objective of this study was to investigate the long-term course of bronchial inflammation and pulmonary function testing in children with PIBO. METHODS: Medical charts of 21 children with PIBO were analyzed retrospectively at the Children's University Hospital Frankfurt/Main Germany. Pulmonary function tests (PFTs) with an interval of at least 1 month were studied between 2002 and 2019. A total of 382 PFTs were analyzed retrospectively and per year, the two best PFTs, in total 217, were evaluated. Additionally, 56 sputum analysis were assessed and the sputum neutrophils were evaluated. RESULTS: The evaluation of the 217 PFTs showed a decrease in FEV1 with a loss of 1.07% and a loss in z score of -0.075 per year. FEV1/FVC decreased by 1.44 per year. FVC remained stable, showing a nonsignificant increase by 0.006 in z score per year. However, FEV1 and FVC in L increased significantly with FEV1 0.032 L per cm and FVC 0.048 L/cm in height. Sputum neutrophils showed a significant increase of 2.12% per year. CONCLUSION: Our results demonstrated that in patients with PIBO pulmonary function decreased significantly showing persistent obstruction over an average follow-up period of 8 years. However, persistent lung growth was revealed. In addition, pulmonary inflammation persisted clearly showing an increasing amount of neutrophils in induced sputum. Patients did not present with a general susceptibility to respiratory infections.

Clinical analysis of adenovirus postinfectious bronchiolitis obliterans and nonadenovirus postinfectious bronchiolitis obliterans in children.

BACKGROUND AND OBJECTIVE: Postinfectious bronchiolitis obliterans (PIBO) is an uncommon and severe form of chronic obstructive lung disease in children. Adenovirus (ADV) is the main pathogen of PIBO in children. However, the risk factors of ADV-PIBO are not fully understood. This study aims to analyze the clinical characteristics of PIBO caused by ADV and compared with non-ADV-PIBO. MATERIALS AND METHODS: A retrospective study of children under the age of 14 years diagnosed PIBO was performed in 56 ADV-PIBO cases, 29 non-ADV-PIBO, and 39 healthy controls to determine clinical characteristics of PIBO. RESULTS: There was no difference between ADV-PIBO and non-ADV-PIBO cases in gender, age, individual and family atopy or history of asthma, and previous history of wheezing and no difference in the clinical manifestations and signs between the two groups. However, the hospital stay, duration of ventilator use, and multifocal pneumonia incidence of ADV-PIBO group have a significant differences compared with non-ADV-PIBO. Notably, inflammatory markers lactic dehydrogenase (LDH), interleukin 8 (IL-8), and interferon-gamma (IFN-γ) were significantly increased in PIBO patients, and those expressed in ADV-PIBO patients were higher than those in non-ADV-PIBO patients. In addition, ADV-PIBO children required a longer duration of oral prednisone and azithromycin than non-ADV-PIBO cases. CONCLUSIONS: Compared with non-ADV-PIBO, ADV-PIBO has a longer hospital stay, longer duration of ventilator use, increased LDH, IL-8, and IFN-γ expressions, and longer duration of oral prednisone and azithromycin treatment. Further research is needed to determine why PIBO caused by ADVs are more severe than those caused by other pathogens.

Clinical and Genetic Spectrum of Children With Primary Ciliary Dyskinesia in China.

BACKGROUND: Primary ciliary dyskinesia (PCD) is a heterogeneous disease with a diverse clinical and genetic spectrum among populations worldwide. Few cases of pediatric PCD have been reported from China. RESEARCH QUESTION: What are the clinical and genotypic characteristics of children with PCD in China? STUDY DESIGN AND METHODS: Clinical characteristics, laboratory findings, and genetic results obtained for 75 patients with PCD were reviewed retrospectively at a single center in China. Genetic sequencing was conducted using whole-exome screening. RESULTS: Patient median age at diagnosis was 7.0 years (range, 2 months-14 years). Of 75 patients, 88% (66/75) had chronic wet cough, 77% (58/75) had recurrent sinusitis, 76% (57/75) had bronchiectasis, 40% (30/75) had neonatal respiratory distress, and 28% (21/75) had coexistent asthma. Notably, postinfectious bronchiolitis obliterans (PIBO) as first presentation was found in 8% of children (6/75). Genes with the highest incidence of mutations were DNAH11 (15/51), followed by DNAH5 (9/51), CCDC39 (5/51), DNAH1 (4/51), and CCNO (3/51). Four genes (DNAI1, HEATR2, RSPH9, and DNAAF3) each were respectively found in two patients, and seven genes (CCDC40, LRRC6, SPAG1, RSPH4A, ARMC4, CCDC114, and DNAH14, a novel gene) each were mutated once. No differences in classical clinical features were observed among patients with commonly observed PCD-associated genotypes. However, three of six PIBO patients carried DNAH1 mutations. INTERPRETATION: Besides typical clinical features, PIBO was observed as the first presentation of pediatric PCD in China. An association of the novel gene DNAH14 with PCD was observed, expanding the PCD genotypic spectrum.

Postinfectious bronchiolitis obliterans masked by misdiagnosis as asthma.

OBJECTIVES: Asthma and postinfectious bronchiolitis obliterans (PIBO) have similar clinical findings, and PIBO may be misdiagnosed with asthma. This study aimed to determine the clinical features of PIBO in children and the causes of delay in its diagnosis. METHODS: We retrospectively evaluated all patients diagnosed with PIBO in four pediatric pulmonology centers between 2007 and 2018. In total, 64 PIBO patients were retrospectively reviewed. We compared the clinical and laboratory differences between PIBO patients who had initially been misdiagnosed with asthma and correctly diagnosed with PIBO. RESULTS: Of the 64 patients, 22 (34.4%) had initially been misdiagnosed with asthma. Adenovirus was the most common infectious agent in children. The age upon diagnosis was older, and the symptom duration was significantly longer in patients misdiagnosed with asthma (P < .05). There were no statistical differences in terms of sex, history of prematurity, duration of hospitalization, treatment, history of oxygen or mechanical ventilation support, pulmonary function test (PFT) results and asthma-predisposing findings between the two groups (P > .05). CONCLUSIONS: Patients with PIBO who had initially been misdiagnosed with asthma were correctly diagnosed at older ages and had longer symptom duration. Asthma may mask PIBO diagnosis by the similarity of symptoms and the clinical response to inhaled ß2-agonist or steroid treatment. PFTs may not help clinicians because of the age of children. The delay in the diagnosis of PIBO is probably attributable to the fact that some clinicians fail to include PIBO in the differential diagnosis when there is no clinical response to asthma medication.

Clinical profile and course of children with postinfectious bronchiolitis obliterans from a tertiary care hospital.

BACKGROUND: Postinfectious bronchiolitis obliterans (PIBO) is a chronic obstructive lung disease with scanty information in literature on etiology, clinical profile, treatment, and outcome. OBJECTIVE: The objective of the study is to describe the clinical profile and course of children diagnosed with PIBO. METHODS: A chart review of children below 18 years of age diagnosed as PIBO over the past 9 years was carried out. Details of clinical profile, laboratory investigations, imaging, treatment received, and outcome were recorded. RESULTS: Eight children (boys 4) with PIBO were identified. Median (interquartile range [IQR]) age at the first episode of acute severe bronchiolitis such as illness and diagnosis of PIBO was 15 (6, 23.5) and 30 (16.5, 60) months, respectively, indicating a delay in diagnosis. The most common symptoms were recurrent episodes of cough (100%), fast breathing (100%), wheezing (87.5%), and fever (62.5%). Median (IQR) number of hospitalizations and episodes of antibiotic use prior to diagnosis were 2.5 (2, 5.5) and 2 (2, 4), respectively. Three (37.5%) children received mechanical ventilation during previous hospitalizations. Chest computed tomography revealed mosaic attenuation in 8 (100%), ground-glass opacities in 2 (25%), and bronchial wall thickening in 2 (25%). After diagnosis, 7 received oral steroids, 7 received hydroxychloroquine, 5 received azithromycin, and 2 received azathioprine. The median (IQR) duration of follow-up (n = 6) was 6 (1.5, 9.5) months. Median (IQR) number of pulmonary exacerbations in follow-up was 2 (1, 5). CONCLUSION: PIBO is still an under-recognized entity with substantial delay in diagnosis and unnecessary use of antibiotics. Clinical course with imaging findings may help to diagnose and manage this entity.

Respiratory reactance in children aged three to five years with postinfectious bronchiolitis obliterans is higher than in those with asthma.

AIM: We investigated airway function in preschoolers with postinfectious bronchiolitis obliterans (PIBO) using impulse oscillometry (IOS). METHODS: This study enrolled 182 children aged three to five years: 12 with PIBO, 135 with asthma and 35 nonatopic controls. Respiratory resistance and reactance were assessed using IOS. RESULTS: The percentage predicted (% predicted) of prebronchodilator respiratory resistance at 5 Hz was significantly higher in children with PIBO (177.9 ± 118.4%) than the asthma (126.1 ± 30.5%, p = 0.013) or control (121.1 ± 21.8%, p = 0.014) groups. After bronchodilator use, children with PIBO did not reach the values of Rrs5% predicted in the asthma and control groups. Respiratory reactance (Xrs5% predicted) in children with PIBO (337.1 ± 478.5%) was significantly higher than both asthma (130.0 ± 80.0%, p = 0.004) and control (105.1 ± 30.8%, p < 0.001) groups before bronchodilator use and significantly higher than the two groups after bronchodilator use (p = 0.010 and p = 0.004, respectively). The changes in Rrs5 and Xrs5 were not significantly different between the children with PIBO and asthma. CONCLUSION: Measuring Rrs5 and Xrs5 before and after bronchodilator use may help to discriminate PIBO from asthma in children aged three to five years with chronic or recurrent respiratory symptoms.

Etiology and Outcome of Diffuse Acute Infectious Bronchiolitis in Adults.

RATIONALE: The etiology and outcome of diffuse acute infectious bronchiolitis (DAIB) in adults is not well known. OBJECTIVES: To retrospectively review adult patients with DAIB without pneumonia, document the etiologies and outcomes, and assess the relation between DAIB and postinfectious bronchiolitis obliterans. METHODS: We retrospectively analyzed medical records of 1,664 patients with lower respiratory tract infections admitted to our institution in Saitama, Japan. DAIB was diagnosed when patients developed acute feverish lower respiratory tract infection and chest computed tomography demonstrated mainly multiple centrilobular nodules in four or more lobes. Pneumonia was diagnosed when patients developed acute feverish lower respiratory tract infection and chest computed tomography demonstrated consolidation and/or ground-glass opacities with or without centrilobular nodules. MEASUREMENTS AND MAIN RESULTS: Of the 1,664 patients, 20 (1.2%) and 1,644 (98.8%) patients were diagnosed as having DAIB and pneumonia, respectively. Of the 20 patients with DAIB, the etiology was determined in 16 (80%): 13 (65.0%) had a single pathogen and 3 (15.0%) had two pathogens. Detected organisms included Mycoplasma pneumoniae in eight (40.0%) patients, influenza virus in two (10.0%), influenza virus and Streptococcus pneumoniae in two (10.0%), Haemophilus influenzae in three (15.0%), and respiratory syncytial virus and rhinovirus in one (5.0%) patient. All patients improved and none developed postinfectious bronchiolitis obliterans. CONCLUSIONS: The three most common etiologies of DAIB in the studied adults were M. pneumoniae, influenza virus, and H. influenzae. None of the patients with DAIB developed postinfectious bronchiolitis obliterans.

Balanço energético em crianças e adolescentes com bronquiolite obliterante pós-infecciosa / Energy balance in children and adolescents with post-infectious bronchiolitis obliterans

OBJETIVOS: Quantificar o gasto e estimar a ingestão energética de crianças e adolescentes com bronquiolite obliterante pós-infecciosa e comparar com crianças e adolescentes hígidos. MÉTODOS: Estudo transversal com 72 crianças e adolescentes de 8 a 18 anos. Compararam-se dois grupos de 36 indivíduos - um com diagnóstico de bronquiolite obliterante e outro hígido -, os quais foram pareados pelo sexo, idade e classificação do índice de massa corporal. Para avaliação nutricional, utilizaram-se a antropometria e a composição corporal. O gasto energético foi medido pela calorimetria indireta; o fator atividade, pelo recordatório 24h de atividades físicas, e a ingestão energética, pelos inquéritos alimentares. RESULTADOS: O grupo com bronquiolite obliterante e o grupo-controle apresentaram respectivamente: índice de massa corporal de M=18,9, DP=4,0kg/m² e M=18,8, DP=3,4kg/m²; gasto energético de repouso de M=1717,6, DP=781,5 e M=2019,9, DP=819; gasto energético total de M=2677,5, DP=1514,0kcal/dia e M=3396,1, DP=1557,9kcal/dia; estimativa da ingestão energética de M=2294,1, DP=746,7kcal/dia e M=2116,5, DP=612,1kcal/dia. O gasto energético de repouso (p=0,102) e o gasto energético total (p=0,051) não foram diferentes entre os grupos, mesmo quando ajustados pela massa magra. Não houve diferenças estatisticamente significativas entre o o gasto energético total e o consumo energético no grupo com bronquiolite obliterante (p=0,202). O grupo-controle consumiu menos calorias do que o previsto pelo gasto energético total (p<0,001). CONCLUSÃO: O gasto energético de repouso e o gasto energético total foram semelhantes entre os grupos. A estimativa da ingestão energética dos hígidos foi menor que o gasto energético total. O grupo com bronquiolite obliterante apresentava um balanço energético adequado.

OBJECTIVE: The aim of the study was to determine the energy expenditure and estimate the energy intake of children and adolescents with post-infectious bronchiolitis obliterans and compare them with those of healthy children and adolescents. METHODS: This cross-sectional study included 36 children and adolescents with bronchiolitis obliterans aged 8 to 18 years, and a control group with 36 healthy individuals matched for sex, age and body mass index. Anthropometric data were collected from all individuals. Energy expenditure was determined by indirect calorimetry, activity factor was estimated by a 24-hour record of physical activities, and energy intake was estimated by the 24-hour food recall. RESULTS: Data for the bronchiolitis obliterans and control groups are, respectively: body mass index of M=18.9, SD=4.0kg/m² and M=18.8, SD=3.4kg/m²; resting energy expenditure of M=1717.6, SD=781.5kcal/day and M=2019.9, SD=819kcal/day; total energy expenditure of M=2677.5, SD=1514.0kcal/day and M=3396.1, SD=1557.9kcal/day; estimated energy intake of M=2294.1, SD=746.7kcal/day and M=2116.5, SD=612.1kcal/day. Resting energy expenditure (p=0.102) and total energy expenditure (p=0.051) did not differ between the groups, even when adjusted for lean mass. Total energy expenditure and intake of the bronchiolitis obliterans group did not differ significantly (p=0.202). Energy intake by the control group was lower than expected according to their total energy expenditure (p<0.001). CONCLUSION: The resting energy expenditure and total energy expenditure of the two groups were similar. The estimated energy intake of the control group was lower than their total energy expenditure. The bronchiolitis obliterans group had an appropriate energy balance.

Función pulmonar y calidad de vida en niños y adolescentes con bronquiolitis obliterante por adenovirus / Pulmonary function and quality of life in children and adolescents with bronchiolitis obliterans post-adenoviral infection

Introduction: Adenovirus infection is an important cause of pneumonia in Chilean children. Postinfectious Bronchiolitis Obliterans (PIBO) is the most important complication. There are few studies assessing pulmonary function and quality of life in PIBO patients. Objective: The aim of this study is to assess the pulmonary function and the quality of life of patients with PIBO and the correlation between both variables. Methods: 14 children with PIBO in follow up at the pediatric pulmonology outpatient clinic of a public children hospital were included in this study. Study period: April 2009 - April 2010. Pulmonary function was assessed in a medical visit by spirometry, flow/volume curve and intrathoracic gas volume measurement. The following indices were analyzed FVC, FEV1, FEV25-75, FEV1/FVC, RV, TLC, RV and RV/TLC. A survey of a self-administered Quality of Life (PedsQL, version 4.0, Spanish for Chile) was applied at the visit to investigate their global, physical and psychosocial quality of life. The Pearson linear correlation between quality of life and the pulmonary function test parameters was assessed, in the statistical analysis ap value < 0.05 was considered significant. Results: The mean age of our patients was 12.4 (range: 9-19 years-old). The functional alterations were characteristics of an obstructive respiratory disorder in 64.3 percent of the patients. Average pulmonary function test indices showed decreases in FEV1 (75 percent), FEV25-75 (48.8 percent), FEV1/FVC (67.6 percent) with normal value of FVC (93,7 percent) as well as pronounced increases in RV (333.5 percent), TLC (156 percent) and RV/TLC (51.8 percent). Mean change of FEV1 and FEV25-75post-bronchodilator was 11.3 percent and 26.4 percent respectively. Only 14.3 percent of our patients showed normal pulmonary function. The quality of life parameters were in average 58.2 percent of overall quality of life, 58.6 percent of physical quality of life and 57.9 percent of psychosocial ...

Introducción: La infección por adenovirus es una causa importante de neumonía en niños chilenos. La bronquiolitis obliterante (BOPI) es la complicación más importante. Existen pocos estudios donde evalúen la función pulmonar y la calidad de vida de los pacientes con BOPI. Objetivo: Evaluar la función pulmonar y la calidad de vida de los pacientes con BOPI y la correlación entre ambas variables. Métodos: Se incluyeron 14 niños con BOPI en el policlínico de la unidad de broncopulmonar de un hospital público de niños. Período de estudio: abril de 2009-abril de 2010. La función pulmonar se evaluó en una visita médica por espirometría, curva flujo / volumen y la determinación de volumen de gas intratorácico. Los índices analizados fueron los siguientes CVF, VEF1, FEV25-75, VEF1/CVF, VR,CPT, VR y VR / CPT. En la misma visita se realizó una encuesta auto-administrada de Calidad de Vida (PedsQL, versión 4.0, español de Chile) para investigar la calidad de vida global, física y psi-cosocial. Se aplicó la correlación lineal de Pearson entre calidad de vida y los parámetros de función pulmonar. Se consideró estadísticamente significativo unp <0,05. Resultados: La edad media de nuestros pacientes fue de 12,4 (rango: 9-19 años). La alteración funcional característica fue la obstructiva en el 64,3 por ciento de los pacientes. El promedio de los índices de pruebas de función pulmonar mostraron una disminución en VEF1 (75 por ciento), FEV25-75 (48,8 por ciento), VEF1/CVF (67,6 por ciento) con un valor normal de la CVF (93,7 por ciento), así como aumentos pronunciados en VR (333,5 por ciento), CPT (156 por ciento) y VR/CPT (51,8 por ciento). La media del cambio del VEF1 y FEV25-75post-broncodilatador fue 11,3 por ciento y 26,4 por ciento respectivamente. Sólo el 14,3 por ciento mostró una función pulmonar normal. Los parámetros de calidad de vida fueron en promedio 58,2 por ciento de calidad de vida general, 58,6 por ciento de calidad de vida física y el 57,9 por ciento...