Objective: To describe the pulmonary function in children and adolescents with postinfectious bronchiolitis oblite­rans (PIBO), as well as to evaluate potential risk factors for severe impairment of pulmonary function. Methods: The pulmonary function of 77 participants, aged 8-18 years, was assessed by spirometry and plethysmography. The following parameters were analyzed: FVC; FEV1; FEF25-75%; FEV1/FVC; RV; TLC; RV/TLC; intrathoracic gas volume; and specific airway resistance (sRaw). We used Poisson regression to investigate the following potential risk factors for severe impairment of pulmonary function: gender; age at first wheeze; age at diagnosis; family history of asthma; tobacco smoke exposure; length of hospital stay; and duration of mechanical ventilation. Results: The mean age was 13.5 years. There were pronounced decreases in FEV1 and FEF25-75%, as well as increases in RV and sRaw. These alterations are characteristic of obstructive airway disease. For the parameters that were the most affected, the mean values (percentage of predicted) were as follows: FEV1 = 45.9%; FEF25-75% = 21.5%; RV = 281.1%; RV/TLC = 236.2%; and sRaw = 665.3%. None of the potential risk factors studied showed a significant association with severely impaired pulmonary function. Conclusions: The patients with PIBO had a common pattern of severe pulmonary function impairment, characterized by marked airway obstruction and pronounced increases in RV and sRaw. The combination of spirometric and plethysmographic measurements can be more useful for assessing functional damage, as well as in the follow-up of these patients, than are either of these techniques used in isolation. Known risk factors for respiratory diseases do not seem to be associated with severely impaired pulmonary function in PIBO.

Keywords: Respiratory function tests; Airway obstruction; Bronchiolitis obliterans

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.

Keywords: Multidetector computed tomography; Respiratory function tests; Bronchiolitis obliterans.

In the present report, we describe an unusual presentation of post-infectious bronchiolitis obliterans accompanied by pulmonary hemosiderosis in a nine-year-old boy with persistent respiratory symptoms subsequent to an episode of acute bronchiolitis occurring at the age of seven months. After the episode, the persistent respiratory symptoms worsened significantly, and, by the age of seven, the patient began to have difficulty breathing after minimal exertion. Computed tomography of the chest presented findings consistent with bronchiolitis obliterans. Open lung biopsy revealed numerous hemosiderin-laden macrophages, as well as other findings consistent with bronchiolitis obliterans. Pulmonary hemosiderosis can occasionally be accompanied by bronchiolitis obliterans in children with severe sequelae after an episode of viral infection.

Keywords: Bronchoalveolar lavage; Bronchiolitis obliterans; Hemosiderosis; Case reports [Publication type]

BACKGROUND: The clinical evaluation of patients with post-infectious bronchiolitis obliterans (BO) is variable. OBJECTIVE: Substantiate the clinical characteristics, the evolution and the complementary tests of 48 patients with post-infectious bronchiolitis obliterans (BO). METHOD: Observational and retrospective study. Diagnosis of BO was based upon clinical criteria, CT scan findings and exclusion of other diseases. History prior to diagnosis and complementary tests were evaluated as well as initial and final values of oxygen saturation. RESULTS: Mean age of patients at the acute stage of the infectious disease was of 9.6. Thirty two of the patients were male. All were hospitalized during the acute stage, 14 of them (29%) in the ICU. Four patients died two years after onset of acute bronchiolitis. During evolution, all required emergency care due to exacerbation of the pulmonary condition and 24 (50%) were hospitalized, 2 of them in the ICU. The majority continued presenting cough, wheezing, crackles and hyperinflation, however to a lesser degree. Mean of initial arterial saturation was of 89% and final of 92%. The most common infectious agents in sputum samples were H. influenzae, S. pneumoniae and M. catarrhalis. Increased serum IgM and IgC were found in 9 and 7 patients, respectively. The most frequent findings at thorax CT scan were mosaic perfusion, bronchiectasis, atelectasis and bronchial wall thickening. CONCLUSION: Post infectious BO is a chronic and severe disease with persistent symptoms that in general affect infants. Positive serum cultures and increased serum immunoglobulins are suggestive of chronic infectious inflammatory condition.

Keywords: Bronchiolitis obliterans. Obstructive lung diseases.

Objective: Asthma is the most common chronic pulmonary disease, characterized by bronchial inflammation. Some children with asthma have persistent pulmonary function impairment. The prevalence and etiology of this abnormality in children with asthma in developing countries remain unknown. The objective of this study was to estimate the proportion of patients with impaired pulmonary function who were unresponsive to treatment in a group of children and adolescents with asthma, and to describe the phenotypic characteristics of the sample. Methods: Using a standardized questionnaire, we selected outpatients (5-17 years of age) diagnosed with persistent asthma. These patients underwent spirometry and skin prick tests for sensitivity to common aeroallergens. Persistent pulmonary function impairment was defined as an FEV1/FVC ratio < 0.80, even after 10 days of treatment with bronchodilators and oral corticosteroids. We used the atopic index to differentiate between patients with little or no response to the skin prick test and those with a strong response (cut-off point: 4 allergens). Results: We included 96 patients with a mean age of 10.6 years. Of those, 52 (54.1%) were male, and 89 (92.7%) were atopic. Of the 96 patients, 8 (8.3%) had impaired pulmonary function even after the treatment. Among those patients, 8 (100%) were atopic, 7 (87.5%) had moderate or severe asthma, and 7 (87.5%) had a history of hospitalization for acute bronchiolitis. Conclusions: Children and adolescents with moderate or severe asthma can present with impaired pulmonary function and be unresponsive to treatment. This clinical situation has been little studied in developing countries, and its risk factors and etiology will be better understood only through birth cohort studies.

Keywords: Asthma; Respiratory function tests; Allergy and immunology.

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.

Keywords: Bronchiolitis obliterans; Infection/complications; Airway obstruction; Bronchodilator agents.

Objective: To evaluate the association between nutritional status measurements and pulmonary function in children and adolescents with cystic fibrosis. Methods: We evaluated the nutritional status of 48 children and adolescents (aged 6-18 years) with cystic fibrosis based on body mass index (BMI) and body composition measurements-mid-arm muscle circumference (MAMC) and triceps skinfold thickness (TST)-at a referral center in the city of Rio de Janeiro, Brazil. Pulmonary function was assessed by means of spirometry, using FEV1 to classify the severity of airway obstruction. We used Student's t-tests for comparisons between proportions and linear regression analysis for associations between continuous variables. The level of significance was set at p < 0.05. Results: The evaluation of nutritional status based on BMI identified a smaller number of malnourished patients than did that based on MAMC (14 vs. 25 patients, respectively). Most of the patients presented mild pulmonary disease. Mean FEV1 was 82.5% of predicted. Pulmonary function was found to correlate significantly with BMI, MAMC and TST (p = 0.001, p = 0.001 and p = 0.03, respectively). All subjects with moderate or severe pulmonary involvement were considered malnourished based on BMI and body composition parameters. Of the 25 patients considered malnourished based on body composition (MAMC), 19 were considered well-nourished based on their BMI. Conclusions: In the present study, all nutritional status measurements correlated directly with the pulmonary function of children and adolescents with cystic fibrosis. However, body composition measurements allowed earlier detection of nutritional deficiencies.

Keywords: Cystic fibrosis; Body composition; Spirometry; Nutrition assessment.

Objective: To evaluate pulmonary function and functional capacity in children and adolescents with sickle cell disease. Methods: This was a cross-sectional study involving 70 children and adolescents (8-15 years of age) with sickle cell disease who underwent pulmonary function tests (spirometry) and functional capacity testing (six-minute walk test). The results of the pulmonary function tests were compared with variables related to the severity of sickle cell disease and history of asthma and of acute chest syndrome. Results: Of the 64 patients who underwent spirometry, 15 (23.4%) showed abnormal results: restrictive lung disease, in 8 (12.5%); and obstructive lung disease, in 7 (10.9%). Of the 69 patients who underwent the six-minute walk test, 18 (26.1%) showed abnormal results regarding the six-minute walk distance as a percentage of the predicted value for age, and there was a ≥ 3% decrease in SpO2 in 36 patients (52.2%). Abnormal pulmonary function was not significantly associated with any of the other variables studied, except for hypoxemia and restrictive lung disease. Conclusions: In this sample of children and adolescents with sickle cell disease, there was a significant prevalence of abnormal pulmonary function. The high prevalence of respiratory disorders suggests the need for a closer look at the lung function of this population, in childhood and thereafter.

Keywords: Anemia, sickle cell; Respiratory function tests; Exercise test.

Post-infectious bronchiolitis obliterans (PIBO) is a small airways disease characterized by fixed airflow limitation. Therefore, inhaled bronchodilators and corticosteroids are not recommended as maintenance therapy options. The management of PIBO currently consists only of close monitoring of affected patients, aimed at the prevention and early treatment of pulmonary infections. In recent years, there has been an increase in the incidence of PIBO in the pediatric population. Patients with PIBO are characterized by a progressive decline in lung function, accompanied by a decrease in overall functional capacity. Here, we report the case of a relatively young man diagnosed with PIBO and followed for three years. After short- and long-term therapy with an inhaled corticosteroid/long-acting β2 agonist combination, together with an inhaled long-acting antimuscarinic, the patient showed relevant improvement of airway obstruction that had been irreversible at the time of the bronchodilator test. The lung function of the patient worsened when he interrupted the triple inhaled therapy. In addition, a 3-week pulmonary rehabilitation program markedly improved his physical performance.

Keywords: Bronchiolitis obliterans/therapy; Infection/complications; Adrenergic beta-2 receptor antagonists/therapeutic use; Administration, inhalation; Anti-inflammatory agents/therapeutic use; Muscarinic antagonists/therapeutic use; Lung diseases/rehabilitation.

Background: Spirometry is widely used in the diagnosis and quantification of respiratory disorders such as asthma. A microspirometer is a portable and easily used apparatus that can substitute for a regular spirometer, although there is little evidence of its accuracy. Objective: To compare the microspirometer to a regular spirometer for use in asthmatic children and adolescents. Methods: The instruments used were a Micro Spirometer, manufactured by Micro Medical, and a conventional Cosmed Pony Graphic 3.5 spirometer, both with turbinometers (flow sensors). The study sample consisted of 62 children and adolescents, of both genders, clinically diagnosed with asthma and under treatment at a pulmonology clinic. Ages ranged from 5 and 16 years. All spirometric tests were carried out according to the guidelines established by the American Thoracic Society and by the Associacão Brasileira de Normas Técnicas (Brazilian Technical Standards Association). For each patient, microspirometry was performed first, followed by conventional spirometry. The parameters analyzed in both devices were forced vital capacity, forced expiratory volume in one second and peak expiratory flow. Data were analyzed using the Student's t-test and Pearson's correlation test. Results: Strong correlations were found between the two devices in the parameters analyzed: forced expiratory volume in one second: r = 0.97; forced vital capacity: r = 0.97; and peak expiratory flow: r = 0.91. Conclusion: These results demonstrate that the microspirometer is a useful diagnostic tool that can be used when a conventional spirometer is unavailable.

Keywords: Key words: Asthma. Spirometry. Micro spirometer. Puomonary ventilation.

There are few reports in the medical literature about association between bronchiolitis obliterans with organizing pneumonia (BOOP) and aspergilloma. This report shows the presence of both BOOP and pulmonary aspergilloma in a patient with adult T cell leukemia-lymphoma. It is suggested that these findings represent a random association rather than the expression of a nosologic unity.

Keywords: bronchiolitis obliterans; aspergillosis; T cell lymphoma; pneumonia

A 54 year old female patient, public servant, ex-smoker, after two weeks of a common cold complained of cough with yellow sputum, nasal obstruction and facial pain. A chest X-ray showed a solitary nodular lesion in the upper left lobe. The patient was submitted to a left thoracotomy, which established the diagnosis of bronchiolitis obliterans organizing pneumonia (BOOP).

Objective: To assess respiratory system impedance (Zrs) and spirometric parameters in children and adolescents with and without a history of preterm birth. Methods: We evaluated a sample of 51 subjects between 11 and 14 years of age: 35 who had a history of preterm birth (preterm group) and 16 who had been born at term (full-term group). Lung function was measured by spirometry, spectral oscillometry, and intra-breath oscillometry. Results: Neither spirometry nor spectral oscillometry revealed any statistically significant differences between the preterm and full-term groups. However, intra-breath oscillometry demonstrated significant differences between the two groups in terms of the change in resistance, reactance at end-inspiration, and the change in reactance (p < 0.05 for all). Conclusions: Our findings suggest that abnormalities in Zrs persist in children and adolescents with a history of preterm birth and that intra-breath oscillometry is more sensitive than is spectral oscillometry. Larger studies are needed in order to validate these findings and to explore the impact that birth weight and gestational age at birth have on Zrs later in life.

Keywords: Oscillometry; Premature birth; Respiratory function tests; Respiratory mechanics; Spirometry.

Objective: To evaluate quality of sleep and pulmonary function in clinically stable adolescents with sickle cell anemia (SCA). Methods: A cross-sectional descriptive study involving 50 patients with SCA submitted to nocturnal polysomnography and spirometry at the Brasília University Hospital. Anthropometric, polysomnographic and pulmonary function data were analyzed. Patients were divided into two groups according to oxygen saturation by pulse oximetry (SpO2) during rapid eye movement (REM) sleep: SpO2 ≤ 93%; and SpO2 > 93%. Descriptive statistics, Student's t-test, chi-square test and Pearson's correlation coefficient were used. Results: Mean age was 13.9 ± 2.5 years. Total sleep time and REM sleep percentage were lower, whereas REM sleep latency, the number of awakenings, movement during sleep, changes in sleep stage, sleep-disordered breathing index and obstructive apnea index were higher. Two patients (4%) did not present REM. There were statistically significant differences between the groups in most of the polysomnographic variables. The SpO2 in REM sleep presented a strong positive correlation with waking SpO2 and with SpO2 in non-REM sleep, whereas it presented a strong negative correlation with the percentage of total sleep time during which SPO2 was < 90%. Mean spirometric values were within normal ranges. Residual volume and the residual volume/total lung capacity/functional residual capacity ratio were elevated. Conclusion: Sleep impairment in clinically stable patients with SCA is probably due to hemoglobin desaturation and not to individual alterations in pulmonary function.

Keywords: Sleep disorders; Polysomnography; Adolescent; Anemia, sickle cell; Spirometry.

Most interstitial lung diseases (ILD) are indolent in nature, producing subacute or chronic symptoms that progress at various rates and are usually present for months to years before a diagnosis is established. This report describes a case of a fulminating and life-threatening variant of idiopathic bronchiolitis obliterans with organizing pneumonia (BOOP). Early suspicion, histologic diagnosis, and prompt initiation of corticosteroid therapy is important to prevent mortality.

Keywords: Bronchiolitis obliterans with organizing pneumonia (BOOP). Interstitial lung disease.

Objective: To describe lung function findings in overweight children and adolescents without respiratory disease. Methods: This was a cross-sectional study involving male and female overweight children and adolescents in the 8-18 year age bracket, without respiratory disease. All of the participants underwent anthropometric assessment, chest X-ray, pulse oximetry, spirometry, and lung volume measurements. Individuals with respiratory disease were excluded, as were those who were smokers, those with abnormal chest X-rays, and those with an SpO2 ≤ 92%. Waist circumference was measured in centimeters. The body mass index-for-age Z score for boys and girls was used in order to classify the individuals as overweight, obese, or severely obese. Lung function variables were expressed in percentage of the predicted value and were correlated with the anthropometric indices. Results: We included 59 individuals (30 males and 29 females). The mean age was 11.7 ± 2.7 years. Lung function was normal in 21 individuals (35.6%). Of the 38 remaining individuals, 19 (32.2%), 15 (25.4%), and 4 (6.7%) presented with obstructive, restrictive, and mixed ventilatory disorder, respectively. The bronchodilator response was positive in 15 individuals (25.4%), and TLC measurements revealed that all of the individuals with reduced VC had restrictive ventilatory disorder. There were significant negative correlations between the anthropometric indices and the Tiffeneau index in the individuals with mixed ventilatory disorder. Conclusions: Lung function was abnormal in approximately 65% of the individuals evaluated here, all of whom were overweight. Obstructive ventilatory disorder and positive bronchodilator response predominated.

Keywords: Obesity/complications; Respiratory function tests; Lung diseases/etiology.

Background: Several factors influence the pulmonary function values considered normal. In children of short stature, there are difficulties in interpreting the pulmonary function. Objective: To assess spirometric values in children and adolescents with short stature and to identify a correction factor to adequately predict the expected values for this population. Method: A prospective selection of 77 patients was made, all with short stature and no respiratory disease. These patients were submitted to spirometry, transcutaneous hemoglobin oxygen saturation, chest perimeter measurement, and immediate hypersensitivity testing. Bone age was assessed by wrist X-rays. The data obtained by spirometry (FVC, FEV1, and FEF25-75%) were compared with those of Polgar and Promadhat (1971), predicted in three ways: a) by actual height; b) by height estimated at the 50th percentile for chronological age (CA); c) by height estimated at the 50th percentile for bone age (BA). Results: The mean height was 133.3 ± 13.2 cm, and the deficit in relation to the third percentile was 5.4 ± 6.0 cm. The values obtained for FVC, FEV1, FEF25-75%, were significantly higher than those predicted by actual height. The mean FEV1 obtained was 2.42 ± 0.71 L, and the predicted (actual height) was 2.10 ± 0.64 L; according to the height estimated by BA and CA, the values were 2.27 and 2.86 L, respectively. The mean FVC1 was 2.20 ± 0.6 L, and the predicted was 1.90 ± 0.55 L. With the height estimated for bone age and chronologic age, the predicted values were 2.10 and 2.60 L, respectively. Conclusion: Children and adolescents with short stature have higher spirometric values than predicted for their actual height. These findings suggest that the height estimated at the 50th percentile for bone age can be used to evaluate pulmonary function.

Keywords: Lung function. Short stature.

Objective: To determine the prevalence of nocturnal hypoxemia and its association with pulmonary function, nutritional status, sleep macrostructure, and obstructive respiratory events during sleep in a population of clinically stable children and adolescents with cystic fibrosis (CF). Methods: This was a cross-sectional study involving 67 children and adolescents with CF between 2 and 14 years of age. All of the participants underwent polysomnography, and SpO2 was measured by pulse oximetry. We also evaluated the Shwachman-Kulczycki (S-K) scores, spirometry findings, and nutritional status of the patients. Results: The study involved 67 patients. The mean age of the patients was 8 years. The S-K scores differed significantly between the patients with and without nocturnal hypoxemia, which was defined as an SpO2 < 90% for more than 5% of the total sleep time (73.75  6.29 vs. 86.38  8.70; p < 0.01). Nocturnal hypoxemia correlated with the severity of lung disease, FEV1 (rs = −0.42; p = 0.01), FVC (rs = −0.46; p = 0.01), microarousal index (rs = 0.32; p = 0.01), and apnea-hypopnea index (rs = 0.56; p = 0.01). Conclusions: In this sample of patients with CF and mild-to-moderate lung disease, nocturnal oxygenation correlated with the S-K score, spirometry variables, sleep macrostructure variables, and the apnea-hypopnea index.

Keywords: Cystic fibrosis; Sleep; Oximetry.