A case of obstructive sleep apnea syndrome (OSAS) with narcoleptic features is described; polysomnographic studies were carried out and revealed a severe nocturnal respiratory impairment due to apneic episodes. A multiple sleep latency test (MSLT) showed early REM sleep onset in two out of five series. Nasal CPAP utilization improved the quality of nocturnal sleep and reverted all narcoleptic features in the next day's MSLT.

Keywords: Obstructive sleep apnea syndrome (OSAS). Narcolepsy. Sleep deprivation. CPAP.

Congenital lobar emphysema is a disease in which symptoms often begin during the first six months of age. In this report, the authors present the case of a twenty-year-old woman whose symptoms started when she had a spontaneous pneumothorax, an exceptional occurrence. The possible etiologies for that congenital lung malformation and the reasons for the performance of a conservative treatment are discussed.

Keywords: Pulmonary emphysema. Pneumothorax. Abnormalities.

Objective: To evaluate clinical predictors of poor sleep quality in COPD patients with and without obstructive sleep apnea (OSA). Methods: Consecutive stable patients with COPD were evaluated for OSA by means of overnight polysomnography; for sleep quality by means of the Pittsburgh Sleep Quality Index (PSQI); and for disease impact by means of the COPD Assessment Test. COPD severity was graded in accordance with the 2020 GOLD guidelines. Predictors of poor sleep quality were evaluated by multivariate logistic regression analysis. Results: We studied 51 patients with COPD alone and 51 patients with COPD and OSA. Both groups had similar age (66.2 ± 9.2 years vs. 69.6 ± 10.7, p = 0.09) and airflow limitation (p = 0.37). Poor sleep quality was present in 74.8% of the study participants, with no significant difference between COPD patients with and without OSA regarding PSQI scores (p = 0.73). Polysomnography showed increased stage 1 non-rapid eye movement sleep and arousal index, as well as reduced sleep efficiency and stage 3 non-rapid eye movement sleep, in the group of patients with COPD and OSA (p < 0.05). Independent predictors of poor sleep quality were GOLD grade C/D COPD (OR = 6.4; 95% CI, 1.79-23.3; p < 0.01), a COPD Assessment Test score = 10 (OR = 12.3; 95% CI, 4.1-36.5; p < 0.01), and lowest SaO2 < 80% (p < 0.0001). Conclusions: Poor sleep quality is quite common in patients with COPD and is associated with severe COPD and poor health status, having a negative impact on overall quality of life. Despite changes in polysomnography, OSA appears to have no impact on subjective sleep quality in COPD patients.

Keywords: Pulmonary disease, chronic obstructive; Sleep Quality; Sleep apnea, obstructive; Health status.

Objective: To report data regarding COPD patients admitted to the ICU of a referral hospital for respiratory diseases, including outcomes and treatment evaluation. Methods: Study of a series of patients with respiratory failure and COPD admitted to the ICU of Nereu Ramos Hospital, located in the city of Florianópolis, Brazil, between October of 2006 and October of 2007. Data related to demographics, causes of hospitalization, pharmacological treatment, ventilatory support, length of hospital stay, in-hospital complications, ICU mortality, and 28-day mortality were obtained from the medical charts of the patients. Acute Physiology and Chronic Health Evaluation II (APACHE II) scores were calculated. Mortality at 18 months was assessed by subsequent telephone calls. Results: During the study period, 192 patients were admitted to the ICU, 24 (12.5%) of whom were diagnosed with respiratory failure and COPD. The mean length of ICU stay was 12.0 ± 11.1 days. Noninvasive ventilation was used in 10 of the 24 patients (41.66%) and failed in 5 of those 10. Invasive mechanical ventilation (IMV) was used in a total of 15 patients (62.5%). Overall ICU mortality and 28-day mortality were 20.83% and 33.33%, respectively. However, 18-month mortality was 62.5%. Conclusions: Respiratory failure associated with COPD was responsible for 12.5% of the ICU admissions. Orotracheal intubation and IMV were necessary in 62.5% of the cases. The ICU mortality rate was in accordance with that predicted by the APACHE II scores. However, late mortality was high.

Keywords: Epidemiology; Respiration, artificial; Respiratory insufficiency; Pulmonary disease, chronic obstructive; Intensive care units; Mortality.

Introduction: Smoking is the major preventable risk of morbidity and mortality. However, its prevalence is high in developed countries and increasing in developing countries, even though its effects are now better known. The purpose of this study was to compare the exhaled carbon monoxide concentration (exCO) between smokers and nonsmokers, evaluate the factors that influence this parameter among smokers and the potential influence of passive smoking by measuring exCO in workers and patients of Instituto do Coração HC-FMUSP. Materials and methods: This cross study included 256 volunteers who responded to a questionnaire and were submitted to exCO measuring with the MicroCo meter device. Results: There were 106 males and 150 females. Mean age was 43.4 years (Vmin-max: 15-83). There were 107 smokers, 118 nonsmokers and 31 passive smokers. Mean exCO was 14.01 ppm (Vmin-max: 1-44) among smokers, 2.03 ppm (Vmin-max: 0-5) among passive smokers and 2.50 ppm (Vmin-max: 0-9) among nonsmokers. Significant statistical difference was observed between smokers and the other groups (p < 0.001), but not between nonsmokers and passive smokers. A positive correlation was found between the number of cigarettes smoked per day and exCO values while there was a negative correlation between the exCO values and the timing of the last cigarette. For a reference limit value of 6 ppm, sensitivity was 70% and specificity was 96%. Conclusion: exCO metering is easy to perform, low-cost, noninvasive and allows the obtention of immediate results and the reference limit value of 6 ppm has good specificity to evaluate the smoking habit.

Keywords: Smoking. Carbon monoxide. Tobacco smoke pollution. Brazil.

Objective: Patients with high cervical spinal cord injury are usually dependent on mechanical ventilation support, which, albeit life saving, is associated with complications and decreased life expectancy because of respiratory infections. Diaphragm pacing stimulation (DPS), sometimes referred to as electric ventilation, induces inhalation by stimulating the inspiratory muscles. Our objective was to highlight the indications for and some aspects of the surgical technique employed in the laparoscopic insertion of the DPS electrodes, as well as to describe five cases of tetraplegic patients submitted to the technique. Methods: Patient selection involved transcutaneous phrenic nerve studies in order to determine whether the phrenic nerves were preserved. The surgical approach was traditional laparoscopy, with four ports. The initial step was electrical mapping in order to locate the "motor points" (the points at which stimulation would cause maximal contraction of the diaphragm). If the diaphragm mapping was successful, four electrodes were implanted into the abdominal surface of the diaphragm, two on each side, to stimulate the branches of the phrenic nerve. Results: Of the five patients, three could breathe using DPS alone for more than 24 h, one could do so for more than 6 h, and one could not do so at all. Conclusions: Although a longer follow-up period is needed in order to reach definitive conclusions, the initial results have been promising. At this writing, most of our patients have been able to remain ventilator-free for long periods of time.

Keywords: Spinal cord injuries; Quadriplegia; Respiration, artificial; Pacemaker, artificial; Diaphragm.

Objective: To report the experience of a routine follow-up program based on medical visits and chest CT. Methods: This was a retrospective study involving patients followed after complete surgical resection of non-small cell lung cancer between April of 2007 and December of 2015. The follow-up program consisted of clinical examination and chest CT. Each follow-up visit was classified as a routine or non-routine consultation, and patients were considered symptomatic or asymptomatic. The outcomes of the follow-up program were no evidence of cancer, recurrence, or second primary lung cancer. Results: The sample comprised 148 patients. The median time of follow-up was 40.1 months, and 74.3% of the patients underwent fewer chest CTs than those recommended in our follow-up program. Recurrence and second primary lung cancer were found in 17.6% and 11.5% of the patients, respectively. Recurrence was diagnosed in a routine medical consultation in 69.2% of the cases, 57.7% of the patients being asymptomatic. Second primary lung cancer was diagnosed in a routine medical appointment in 94.1% of the cases, 88.2% of the patients being asymptomatic. Of the 53 patients who presented with abnormalities on chest CT, 41 (77.3%) were diagnosed with cancer. Conclusion: Most of the cases of recurrence, especially those of second primary lung cancer, were confirmed by chest CT in asymptomatic patients, indicating the importance of a strict follow-up program that includes chest CTs after surgical resection of lung cancer.

Keywords: Lung neoplasms/surgery; Neoplasm recurrence, local; Neoplasms, second primary.

Objective: To investigate the correlation of HRCT findings with pulmonary metabolic activity in the corresponding regions using 18F-FDG PET/CT and inflammatory markers in patients with systemic sclerosis (SSc)-associated interstitial lung disease (ILD). Methods: This was a cross-sectional study involving 23 adult patients with SSc-associated ILD without other connective tissue diseases. The study also involved 18F-FDG PET/CT, HRCT, determination of serum chemokine levels, clinical data, and pulmonary function testing. Results: In this cohort of patients with long-term disease (disease duration, 11.8 ± 8.7 years), a nonspecific interstitial pneumonia pattern was found in 19 (82.6%). Honeycombing areas had higher median standardized uptake values (1.95; p = 0.85). Serum levels of soluble tumor necrosis factor receptor 1, soluble tumor necrosis factor receptor 2, C-C motif chemokine ligand 2 (CCL2), and C-X-C motif chemokine ligand 10 were higher in SSc patients than in controls. Serum levels of CCL2-a marker of fibroblast activity-were correlated with pure ground-glass opacity (GGO) areas on HRCT scans (p = 0.007). 18F-FDG PET/CT showed significant metabolic activity for all HRCT patterns. The correlation between serum CCL2 levels and GGO on HRCT scans suggests a central role of fibroblasts in these areas, adding new information towards the understanding of the mechanisms surrounding cellular and molecular elements and their expression on HRCT scans in patients with SSc-associated ILD. Conclusions: 18F-FDG PET/CT appears to be unable to differentiate the intensity of metabolic activity across HRCT patterns in chronic SSc patients. The association between CCL2 and GGO might be related to fibroblast activity in these areas; however, upregulated CCL2 expression in the lung tissue of SSc patients should be investigated in order to gain a better understanding of this association.

Keywords: Tomography; Lung diseases, interstitial; Scleroderma, systemic; Cytokines.

Objective: Lymphangioleiomyomatosis (LAM) is a rare, destructive disease of the lungs with a limited number of determinants of disease activity, which are a critical need for clinical trials. FGF23 has been implicated in several chronic pulmonary diseases. We aimed to determine the association between serum FGF23 levels and pulmonary function in a cohort of patients with LAM. Methods: This was a descriptive single-center study in which subjects with LAM and controls with unreported lung disease were recruited. Serum FGF23 levels were measured in all subjects. Clinical data, including pulmonary function testing, were retrospectively obtained from electronic medical records of LAM subjects. Associations between FGF23 levels and clinical features of LAM were explored via nonparametric hypothesis testing. Results: The sample comprised 37 subjects with LAM and 16 controls. FGF23 levels were higher in the LAM group than in the control group. In the LAM group, FGF23 levels above the optimal cutoff point distinguished 33% of the subjects who had nondiagnostic VEGF-D levels. Lower FGF23 levels were associated with impaired DLCO (p = 0.04), particularly for those with isolated diffusion impairment with no other spirometric abnormalities (p = 0.04). Conclusions: Our results suggest that FGF23 is associated with pulmonary diffusion abnormalities in LAM patients and elicit novel mechanisms of LAM pathogenesis. FGF23 alone or in combination with other molecules needs to be validated as a biomarker of LAM activity in future clinical research.

Keywords: Lymphangioleiomyomatosis; Fibroblast growth factor-23; Pulmonary diffusing capacity.

To evaluate handgrip strength (HGS) as a diagnostic tool for frailty risk in elderly patients with asthma, as well as to investigate the prevalence of frailty in this population. Methods: This was a cross-sectional study including 96 patients = 60 years of age diagnosed with moderate to severe asthma and treated at a tertiary referral center in Brazil. We measured HGS using a calibrated hydraulic hand dynamometer. We used a frailty scale and the AUC to assess the diagnostic accuracy of the HGS test. Results: The median age of participants was 67 years. Most (78%) were women and non-White (91%) of low socioeconomic status. HGS identified those at risk for frailty, with an AUC of 71.6% (61.5-80.4%; p < 0.002), as well as a sensitivity of 73.58% and a specificity of 67.53%, on the basis of a cutoff of = 19 kgf. Conclusions: HGS appears to be a simple, reliable tool for clinicians to determine frailty risk in older asthma patients in a point-of-care setting.

Keywords: Hand strength; Frailty; Asthma; Aged.