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.

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

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

Nocardiosis is a localized or disseminated infection caused by gram-positive bacteria of the genus Nocardia. The infection most commonly affects the lungs, skin and central nervous system. Nocardiosis principally occurs in individuals with cellular immunodeficiency and should be considered in the differential diagnosis when such individuals present respiratory, cutaneous or neurological alterations. Herein, we report a case of pulmonary and cutaneous nocardiosis in a patient receiving oral corticosteroids to treat bronchiolitis obliterans accompanied by organizing pneumonia of unknown origin. After long-term treatment with sulfamethoxazole-trimethoprim, the clinical and radiological profile improved.

Keywords: Nocardia infections; Lung diseases; Bronchiolitis obliterans; Adrenal cortex hormones; Case reports [Publication type]

Objetivo: Analisar as características funcionais pulmonares, a resposta farmacodinâmica a um broncodilatador e sua prescrição em pacientes com diagnóstico de fibrose cística (FC). Métodos: Estudo de coorte retrospectivo de pacientes (6-18 anos) com diagnóstico de FC acompanhados em um centro de referência, capazes de realizar testes de função pulmonar (TFP) entre 2008 e 2010. Foram analisados CVF, VEF1 e FEF25-75%, em percentual do previsto, antes e após prova broncodilatadora (pré-BD e pós-BD, respectivamente) de 312 TFP. Foram realizadas ANOVA para medidas repetidas e comparações múltiplas. Resultados: Foram incluídos no estudo 56 pacientes. Desses, 37 e 19, respectivamente, tinham resultados de TFP entre 2008 e 2010 e apenas em 2009-2010, formando dois grupos. No grupo com TFP nos três anos estudados, houve redução significativa em VEF1 pós-BD em 2008-2010 (p = 0,028) e 2009-2010 (p = 0,036) e em FEF25-75% pré-BD e pós-BD em todas as comparações múltiplas (2008 vs. 2009; 2008 vs. 2010; e 2009 vs. 2010). No grupo com TFP apenas em 2009-2010, não houve diferenças significativas em nenhuma das comparações das variáveis estudadas. Dos 312 TFP, somente 24 (7,7%) apresentaram resposta significativa ao broncodilatador e pertenciam a pacientes sem prescrição de broncodilatador durante o período estudado. Conclusões: Houve perda funcional, com indicação de doença pulmonar progressiva, nos pacientes com FC estudados. Houve maiores alterações no FEF25-75%, sugerindo o comprometimento inicial de vias aéreas menores.

Keywords: Fibrose cística; Testes de função respiratória; Broncodilatadores.

Objective: To evaluate the impact of thoracic radiotherapy on respiratory function and exercise capacity in patients with breast cancer. Methods: Breast cancer patients in whom thoracic radiotherapy was indicated after surgical treatment and chemotherapy were submitted to HRCT, respiratory evaluation, and exercise capacity evaluation before radiotherapy and at three months after treatment completion. Respiratory muscle strength testing, measurement of chest wall mobility, and complete pulmonary function testing were performed for respiratory evaluation; cardiopulmonary exercise testing was performed to evaluate exercise capacity. The total radiotherapy dose was 50.4 Gy (1.8 Gy/fraction) to the breast or chest wall, including supraclavicular lymph nodes (SCLN) or not. Dose-volume histograms were calculated for each patient with special attention to the ipsilateral lung volume receiving 25 Gy (V25), in absolute and relative values, and mean lung dose. Results: The study comprised 37 patients. After radiotherapy, significant decreases were observed in respiratory muscle strength, chest wall mobility, exercise capacity, and pulmonary function test results (p < 0.05). DLCO was unchanged. HRCT showed changes related to radiotherapy in 87% of the patients, which was more evident in the patients submitted to SCLN irradiation. V25% significantly correlated with radiation pneumonitis. Conclusions: In our sample of patients with breast cancer, thoracic radiotherapy seemed to have caused significant losses in respiratory and exercise capacity, probably due to chest wall restriction; SCLN irradiation represented an additional risk factor for the development of radiation pneumonitis.

Keywords: Breast neoplasms; Radiotherapy; Radiation pneumonitis; Respiratory function tests; Exercise test.

To describe and characterize local adverse effects (in the oral cavity, pharynx, and larynx) associated with the use of inhaled corticosteroids (ICSs) in patients with moderate or severe asthma. Methods: This was a cross-sectional study involving a convenience sample of 200 asthma patients followed in the Department of Pharmaceutical Care of the Bahia State Asthma and Allergic Rhinitis Control Program Referral Center, located in the city of Salvador, Brazil. The patients were ≥ 18 years of age and had been using ICSs regularly for at least 6 months. Local adverse effects (irritation, pain, dry throat, throat clearing, hoarseness, reduced vocal intensity, loss of voice, sensation of thirst, cough during ICS use, altered sense of taste, and presence of oral candidiasis) were assessed using a 30-day recall questionnaire. Results: Of the 200 patients studied, 159 (79.5%) were women. The mean age was 50.7 ± 14.4 years. In this sample, 55 patients (27.5%) were using high doses of ICS, with a median treatment duration of 38 months. Regarding the symptoms, 163 patients (81.5%) reported at least one adverse effect, and 131 (65.5%) had a daily perception of at least one symptom. Vocal and pharyngeal symptoms were identified in 57 (28.5%) and 154 (77.0%) of the patients, respectively. The most commonly reported adverse effects were dry throat, throat clearing, sensation of thirst, and hoarseness. Conclusions: Self-reported adverse effects related to ICS use were common among the asthma patients evaluated here.

Keywords: Asthma; Glucocorticoids; Administration, inhalation; Pharmaceutical services.

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.

This is a review of the medical literature regarding inhaled corticosteroids and their effects on growth and adrenal suppression in children and adolescents. A review of the literature, principally that published over the last five years, was conducted using Medline and searching indexes of articles published in national and international scientific journals. There is considerable controversy regarding the side effects of inhaled corticosteroids. In 21 studies evaluating the effect that inhaled corticosteroids have on growth, a statistically significant reduction (growth retarded by 1-1.5 cm) was observed within the first year of treatment with Beclomethasone or Budesonide inhalers. However, in studies of longer duration, no significant difference was found between final adult height and adult height of the parents. However, in ten studies of the use of inhaled corticosteroids and their effect on adrenal suppression, hypoglycemia and arrested development (no height or weight gains), as well as changes in morning serum levels and 24-h urinary levels of cortisol, were reported, especially when high doses of inhaled corticosteroids were used. Inhaled corticosteroids can reduce growth during the first year of use but do not affect adult height. However, further long-term studies are needed in order to determine the full impact of inhaled corticosteroids on final adult height. Height measures are a means of evaluating the safety and efficacy of the use of inhaled corticosteroids in children. Tests that evaluate the hypothalamic-pituitary-adrenal axis and adrenal insufficiency should be correlated with clinical symptoms and side effects.

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 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.

Objective: To evaluate treatment compliance and use of inhaled medications of patients with asthma receiving complementary pharmaceutical care. Methods: A controlled prospective parallel study involving a study group and a control group. We selected 60 patients with persistent asthma and using metered-dose inhalers (MDIs), dry powder inhalers (DPIs) or both. The patients were evaluated three times over 60 days. Instructions were provided to the patients in the study group at all visits but only at the first visit to those in the control group. The patients using < 80% or > 120% of the total number of prescribed doses were classified as noncompliant. The inhalation technique was quantified by a scoring system. A satisfactory technique was defined as a score higher than 7 (maximum, 9) for MDIs and higher than 4 (maximum, 5) for DPIs. Results: The final study sample comprised 28 study group patients and 27 control group patients, of whom 18 (64.3%) and 20 (74.7%), respectively, were considered treatment compliant. From the first to the third visits, there were increases, in the study and control groups, in the median MDI-use score (from 3 [range, 0-5] to 8 [range, 8-9]; p < 0.001; and from 5 [range, 2-6] to 7 [range, 6-8]), as well as in the median DPI-use score (from 3 [range, 2-4] to 5 [range, 4-5] and from 3 [range, 2-4] to 4 [range, 3-5]). Conclusions: The counseling provided by the pharmacist to the patient was important to assist in the implementation of the appropriate inhalation technique, especially for MDI use.

Keywords: Asthma; Pharmaceutical services; Administration, inhalation;Metered dose inhalers; Medication adherence.

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).

A síndrome POEMS é uma rara doença de plasmócitos. A ocorrência de hipertensão pulmonar como complicação respiratória da síndrome é pouco frequente e pode estar ligada ao aumento de várias citocinas, quimiocinas e fatores de crescimento como parte dos fenômenos inflamatórios que cercam a fisiopatologia da síndrome POEMS. Descrevemos o caso de uma mulher de 54 anos com síndrome POEMS e hipertensão pulmonar, que foi tratada com corticoide como terapia de primeira linha. Tratava-se de uma paciente com clássicos sintomas dessa síndrome: polineuropatia (confirmada por eletroneuromiografia), organomegalia, hipotireoidismo subclínico, gamopatia monoclonal em dosagem urinária e alterações cutâneas. A cateterização cardíaca direita revelou pressão arterial pulmonar média de 48 mmHg, débito cardíaco de 4,1 L/min e resistência vascular pulmonar de 8,05 Woods. O nível sérico de brain natriuretic peptide (BNP) foi de 150 pg/mL. Nenhuma outra doença foi encontrada durante investigação. Prednisona (1 mg/kg por três meses) foi iniciada, com dramática melhora clínica e funcional, além de normalização dos níveis dos hormônios tireoidianos e de proteína em urina por eletroforese. A pressão arterial pulmonar média caiu para 26 mmHg, o débito cardíaco para 3,8 L/min e a resistência vascular pulmonar para 2,89 Woods. O nível sérico de BNP caiu para 8pg/mL. Nossos achados indicam o potencial papel da corticoterapia como primeira linha de tratamento na hipertensão pulmonar associada à síndrome POEMS. Diante da raridade dessa apresentação, um registro multicêntrico deveria ser desenvolvido para permitir a aquisição de mais dados que suportem essa conduta.

Keywords: Síndrome POEMS; Hipertensão pulmonar; Glucocorticoides.

Objective: To evaluate whether respiratory therapy followed by the use of inhaled albuterol modifies the pulmonary deposition of inhaled tobramycin in patients with cystic fibrosis (CF) and whether pulmonary deposition correlates with disease severity or genotype. Methods: A prospective study was carried out including patients with CF older than 6 years of age and colonized with Pseudomonas aeruginosa. Exclusion criteria were pulmonary exacerbation, changes in therapy between the study phases and FEV1 < 25%. All patients were submitted to pulmonary scintigraphy by means of a scintillation camera equipped with a low-energy all-purpose collimator in order to evaluate drug penetration following the administration of inhaled 99mTc-tobramycin, as well as to pulmonary perfusion with 99mTc-macroaggregated albumin (phase 1). One month later, the same procedure was performed following respiratory therapy and administration of inhaled albuterol (phase 2). Results: We included 24 patients (12 males) aged 5-27 years (mean ± SD: 12.85 ± 6.64 years). The Shwachman score (SS) was excellent/good in 8 patients, moderate/fair in 16 and poor in 0. Genotyping revealed that 7 patients were ΔF508 homozygotes, 13 were ΔF508 heterozygotes; and 4 presented other mutations. In all patients, lung deposition of tobramycin decreased in phase 2, especially in those with moderate/fair SS (p = 0.017) and in heterozygotes (p = 0.043). Conclusions: The use of a respiratory therapy technique and the administration of inhaled albuterol immediately prior to the use of inhaled tobramycin decreased the pulmonary deposition of the latter in CF patients, and this reduction correlates with disease severity and genotype.

Keywords: Cystic fibrosis; Tobramycin; Respiratory therapy; Albuterol; Radionuclide imaging.

Objective: To analyze the effects of Pilates mat exercises in patients with cystic fibrosis (CF). Methods: This was a clinical trial involving 19 CF patients recruited from either the CF Outpatient Clinic of the State University at Campinas Hospital de Clínicas or the Children's Institute of the University of São Paulo School of Medicine Hospital das Clínicas. All of the patients performed Pilates mat exercises for four months (one 60-min session per week). The variables studied (before and after the intervention) were respiratory muscle strength, MIP, MEP, FVC, and FEV1. Results: After the intervention, MIP was significantly higher in the male patients (p = 0.017), as were MIP and MEP in the female patients (p = 0.005 and p = 0.007, respectively). There were no significant differences between the pre- and post-intervention values of FVC or FEV1, neither in the sample as a whole nor among the patients of either gender. Conclusions: Our results show that Pilates mat exercises have beneficial effects on respiratory muscle strength in CF patients. (Brazilian Registry of Clinical Trials - ReBEC; identification number RBR-86vp8x [http://www.ensaiosclinicos.gov.br])

Keywords: Cystic fibrosis; Muscle strength; Exercise movement techniques; Respiratory function tests.