Objective: To identify factors associated with the minimal clinically important difference (MCID) for health-related quality of life (HRQoL) after physical conditioning in patients with COPD. Methods: Thirty-five patients were submitted to a 12-week program of physical conditioning (strength training plus low-intensity aerobic exercise). Body composition, incremental treadmill test results, endurance treadmill test results, six-minute walk test results, peripheral muscle strength, MIP, baseline dyspnea index (BDI) and Saint George's Respiratory Questionnaire (SGRQ) scores were assessed at baseline and after the program, thus allowing the variations (Δ) to be calculated. The MCID for HRQoL was defined as a reduction of ≥ 4% in the SGRQ total score. Subjects who responded to the program, achieving the MCID for HRQoL, were allocated to the responders (R) group (n = 24), and the remainder were allocated to the non-responders (NR) group (n = 11). Results: The values obtained for the following variables were significantly higher in group R than in group NR (p < 0.05): FEV1 (1.48 ± 0.54 L vs. 1.04 ± 0.34 L); VEF1/FVC (47.9 ± 11.7% vs. 35.5 ± 10.7%); PaO2 (74.1 ± 9.7 mmHg vs. 65.0 ± 8.9 mmHg); and ΔBDI, expressed as median and interquartile range (2.0 [0.0-3.5] vs. 0.0 [0.0-1.0]). The ΔBDI correlated significantly with the ΔSGRQ symptoms domain score, activity domain score and total score (r = 0.44, 0.60 and 0.62, respectively, p < 0.01 for all). After logistic regression, only ΔBDI remained as a predictor of MCID for HRQoL. Conclusions: Achieving the MCID for HRQoL after physical conditioning is associated with dyspnea reduction in COPD patients. Therefore, there is a need to develop treatment strategies designed to interrupt the dyspnea-inactivity-dyspnea cycle in such patients.

Keywords: Pulmonary disease, chronic obstructive; Quality of life; Dyspnea; Exercise; Rehabilitation.

Objective: To identify, characterize, and quantify associations of various factors with quality of life (QoL) in patients with asthma, according to the pharmacotherapy employed. Methods: This was a cross-sectional study involving 49 patients (≥ 18 years of age) with severe uncontrolled or refractory asthma treated at a specialized outpatient clinic of the Brazilian Unified Health Care System, regularly using high doses of inhaled corticosteroids (ICs) or other medications, and presenting comorbidities. At a single time point, QoL was assessed with the Asthma Quality of Life Questionnaire (AQLQ). The overall AQLQ score and those of its domains were correlated with demographic variables (gender and age); Asthma Control Questionnaire score; pharmacotherapy (initial IC dose, inhaler devices, and polytherapy); and comorbidities. Results: Better AQLQ scores were associated with asthma control-overall (OR = 0.38; 95% CI: 0.004-0.341; p < 0.001), "symptoms" domain (OR = 0.086; 95% CI: 0.016-0.476; p = 0.001), and "emotional function" domain (OR = 0.086; 95% CI: 0.016-0.476; p = 0.001)-and with IC dose ≤ 800 µg-"activity limitation" domain (OR = 0.249; 95% CI: 0.070-0.885; p = 0.029). Worse AQLQ scores were associated with polytherapy-"activity limitation" domain (OR = 3.651; 95% CI: 1.061-12.561; p = 0.036)-and number of comorbidities ≤ 5-"environmental stimuli" domain (OR = 5.042; 95% CI: 1.316-19.317; p = 0.015). Conclusions: Our results, the importance of this issue, and the lack of studies taking pharmacotherapy into consideration warrant longitudinal studies to establish a causal relationship between the identified factors and QoL in asthma patients.

Keywords: Asthma; Asthma/drug therapy; Quality of life; Medication therapy management.

Objective: To assess the 24-month effects of a pulmonary rehabilitation program (PRP) on anxiety, depression, quality of life and physical performance of COPD patients. Methods: Thirty patients with COPD (mean age, 60.8 ± 10 years; 70% males) participated in a 12-week PRP, which included 24 physical exercise sessions, 24 respiratory rehabilitation sessions, 12 psychotherapy sessions and 3 educational sessions. All patients were evaluated at baseline (pre-PRP), at the end of the treatment (post-PRP) and two years later (current) by means of four instruments: the Beck Anxiety Inventory; the Beck Depression Inventory; Saint George's Respiratory Questionnaire; and the six-minute walk test (6MWT). Results: The comparison between the pre-PRP and post-PRP values revealed a significant decrease in the levels of anxiety (pre-PRP: 10.7 ± 6.3; post-PRP: 5.5 ± 4.4; p = 0.0005) and depression (pre-PRP: 11.7 ± 6.8; post-PRP: 6.0 ± 5.8; p = 0.001), as well as significant improvements in the distance covered on the 6MWT (pre-PRP: 428.6 ± 75.0 m; post-PRP: 474.9 ± 86.3 m; p = 0.03) and the quality of life index (pre-PRP: 51.0 ± 15.9; post-PRP: 34.7 ± 15.1; p = 0.0001). There were no statistically significant differences between the post-PRP and current evaluation values. Conclusions: The benefits provided by the PRP in terms of the indices of anxiety, depression and quality of life, as well as the improved 6MWT performance, persisted throughout the 24-month study period.

Keywords: Pulmonary disease, chronic obstructive; Rehabilitation; Exercise; Quality of life; Anxiety; Depression.

Objective: To evaluate the ability of COPD patients to perform activities of daily living (ADL); to identify barriers that prevent these individuals from performing ADL; and to correlate those barriers with dyspnea severity, six-minute walk test (6MWT), and an ADL limitation score. Methods: In COPD patients and healthy, age-matched controls, the number of steps, the distance walked, and walking time were recorded with a triaxial accelerometer, for seven consecutive days. A questionnaire regarding perceived barriers and the London Chest Activity of Daily Living (LCADL) scale were used in order to identify the factors that prevent the performance of ADL. The severity of dyspnea was assessed with two scales, whereas submaximal exercise capacity was determined on the basis of the 6MWT. Results: We evaluated 40 COPD patients and 40 controls. In comparison with the control values, the mean walk time was significantly shorter for COPD patients (68.5  25.8 min/day vs. 105.2  49.4 min/day; p < 0.001), as was the distance walked (3.9  1.9 km/day vs. 6.4  3.2 km/day; p < 0.001). The COPD patients also walked fewer steps/day. The most common self-reported barriers to performing ADL were lack of infrastructure, social influences, and lack of willpower. The 6MWT distance correlated with the results obtained with the accelerometer but not with the LCADL scale results. Conclusions: Patients with COPD are less active than are healthy adults of a comparable age. Physical inactivity and the barriers to performing ADL have immediate implications for clinical practice, calling for early intervention measures.

Keywords: Pulmonary disease, chronic obstructive; Activities of daily living; Exercise tolerance.

Objective: To determine whether the Body mass index, airway Obstruction, Dyspnea, and Exercise capacity (BODE) index correlates with health-related quality of life in patients with COPD. Methods: We evaluated 42 patients with COPD, quantifying the following: lung function parameters; anthropometric variables; exercise capacity, with the six-minute walk test; dyspnea, with the modified Medical Research Council (MRC) scale; the BODE index; and quality of life, with the modified Saint George's Respiratory Questionnaire (mSGRQ). Patients were divided into two groups by disease severity: FEV1 ≥ 50% and FEV1 < 50%. Results: The mean BODE index was 2.58 ± 1.17 and 4.15 ± 1.81, respectively, for the FEV1 ≥ 50% and FEV1 < 50% groups. There was a significant difference between the groups in terms of FEV1 and the FEV1/FVC ratio. There were moderate to significant correlations between the BODE index scores and all of the mSGRQ domains in the group of patients with FEV1 <50%. Conclusions: The BODE index score correlated with the scores of all of the mSGRQ domains in COPD patients with FEV1 < 50%. Therefore, COPD patients with FEV1 < 50% die sooner and have a poorer quality of life.

Keywords: Pulmonary disease, chronic obstructive; Quality of life; Severity of illness index; Prognosis.

Objective: Barriers to physical activity can affect the functional capacity and quality of life of patients with pulmonary hypertension (PH). This study aimed to identify the main barriers to physical activity in patients with PH and to examine whether those barriers are related to functional capacity, echocardiographic variables, or quality of life. Methods: This was a cross-sectional observational study involving 70 patients. Participants scored seven potential barriers to their activities, with a score = 5 indicating a significant barrier. Participants completed the Medical Outcomes Study 36-item Short-Form Health Survey (SF-36) and Manchester Respiratory Activity of Daily Living questionnaire, as well as the six-minute walk test. Correlation analysis, univariate analysis and multiple logistic regression were performed. Results: As a perceived barrier to physical activity, 'lack of will' or 'lack of energy' was cited by 67% of the patients. The 'lack of will' barrier was found to correlate with all SF-36 domains except bodily pain. We also identified a correlation between the SF-36 vitality domain and the barriers 'lack of energy', 'lack of will' and 'lack of structure'. The logistic regression analysis indicated that the vitality domain correlated significantly with the barriers 'social influence', 'lack of energy', 'lack of will', and 'lack of structure'. For each unit decrease in the vitality score, there was a 10% increase in the probability of citing the barrier 'lack of will'. No significant correlations were identified between any of the perceived barriers and echocardiographic parameters. Conclusions: The perceived barrier most commonly reported was 'lack of will/energy', which correlated with almost all SF-36 domains, especially vitality. The 'lack of will' barrier also correlated with functional capacity.

Keywords: Pulmonary arterial hypertension; Exercise; Motivation; Quality of life; Surveys and questionnaires; Walk test.

Objective: To determine predictors of health-related quality of life (HRQoL) in patients with interstitial lung disease (ILD). Methods: A cross-sectional study comprising 63 patients, all of whom underwent lung function testing and the six-minute walk test. The following instruments were used: the Medical Outcomes Study 36-item Short-form Survey (SF-36), the Saint George's Respiratory Questionnaire (SGRQ), the Beck Anxiety Inventory, the Beck Depression Inventory, and the Modified Medical Research Council Dyspnea Scale. Principal component analysis was used in order to reduce the dimensionality of the data, thereby identifying the predictor variables, and multiple linear regression analysis was used in order to identify the explanatory variables. Results: Of the 63 patients, 34 were female. The mean age was 60.1 ± 13.3 years, the mean FVC was 64.17 ± 15.54% of predicted, and the mean DLCO was 44.21 ± 14.47% of predicted. All of the patients evaluated had impaired HRQoL, scoring worst for the SF-36 physical functioning and SGRQ activity domains. Of the patients evaluated, 60.3% and 57.1% showed symptoms of anxiety and depression, respectively. The principal component analysis identified one predictor of physical HRQoL and one predictor of mental HRQoL. Depression had a strong influence on the predictor of mental HRQoL, and the degree of dyspnea had a strong influence on both predictors of HRQoL in the patients evaluated. Variables related to lung function, exercise capacity, and anxiety had no impact on these predictors. Conclusions: In our sample of patients with ILD, the degree of dyspnea had a major impact on the physical and mental HRQoL, and depression had an impact on mental HRQoL.

Keywords: Anxiety; Depression; Dyspnea; Lung diseases, interstitial; Quality of life; Respiratory function tests.

Objective: To assess ICU patients with COPD, in terms of in-hospital characteristics, two-year mortality and two-year functional status of survivors. Methods: A prospective cohort study involving patients with acute exacerbation of COPD admitted to the ICUs of two hospitals in the city of Porto Alegre, Brazil, between July of 2005 and July of 2006. At two years after discharge, survivors were interviewed by telephone in order to determine Karnofsky scores and scores on a scale regarding activities of daily living (ADL). Results: The sample comprised 231 patients. In-hospital mortality was 37.7%, and two-year post-discharge mortality was 30.3%. Of the 74 survivors, 66 were interviewed (89%). The mean age at ICU admission was 74 ± 10 years, and the mean Acute Physiology and Chronic Health Evaluation II score was 18 ± 7. Two or more comorbidities were present in 87.8% of the patients. Of the 66 interviewees, 57 (86.3%) lived at home, 58 (87.8%) were self-sufficient, 12 (18.1%) required oxygen therapy, and 4 (6.1%) still required ventilatory support. There was a significant reduction in the quality of life and autonomy of the survivors, as evidenced by the Karnofsky scores (85 ± 9 vs. 79 ± 11, p = 0.03) and ADL scale scores (29 ± 5 vs. 25 ± 7; p = 0.01), respectively. Conclusions: In this patient sample, two-year mortality was quite high. Although there was a noticeable reduction in the functional status of the survivors, they remained self-sufficient.

Keywords: Pulmonary disease, chronic obstructive/mortality; Quality of life; Intensive care units.

Objective: To compare two quality of life questionnaires-the Saint George's Respiratory Questionnaire (SGRQ) and the Medical Outcomes Study 36-item Short-Form Health Survey (SF-36)-in patients with COPD, focusing on the discriminative properties of the questionnaires and correlating their domains with the following variables: Modified Medical Research Council Dyspnea Scale score; Beck Depression Inventory score; visual analog scale general health perception; Mini-Mental State Examination score; and a COPD clinical score developed specifically for the study. Methods: We interviewed 30 COPD patients between May and September of 2006. For the SF-36 and SGRQ, scores (total and domain) were compared and correlated. Results: With the exception of the pain domain, all of the SF-36 domains correlated significantly with the SGRQ total score (r = −0.5 to −0.69; p < 0.01). Of the SGQR domains, only the symptoms domain correlated significantly with all of the variables studied (p < 0.05). Conclusions: The majority of the expected correlations between the SGRQ and the SF-36 were observed, as were those expected between the two questionnaires and the other variables studied. The SGRQ, notably the symptoms domain, presented better discriminative properties than did the generic SF-36 questionnaire. The SF-36 is not an appropriate instrument for determining the affective state of COPD patients.

Keywords: Pulmonary disease, chronic obstructive; Quality of life; Data collection.

Objective: To evaluate the responsiveness of the six-minute step test (6MST) to an aerobic physical training program (PTP) and to determine the efficacy of the PTP regarding spirometric variables during the 6MST, as well as physical performance, sensation of dyspnea, and SpO2 during the 6MST and the six-minute walk test (6MWT), in patients with COPD. Methods: This was a controlled, prospective randomized study involving patients clinically diagnosed with COPD, with an FEV1/FVC ratio < 70%, and having been clinically stable in the last two months. The patients were randomized to undergo a PTP on a treadmill, three times a week, for six weeks (PTP group) or not (control group). Histories were taken from all of the patients, who received regular respiratory therapy during the study period, undergoing physical examination and spirometry before and after bronchodilator use; incremental symptom-limited cardiopulmonary exercise testing; the 6MST; and the 6MWT. Results: Of the 36 patients that completed the study, 21 and 15 were in the PTP and control groups, respectively. In the PTP group, there was a significant increase in the number of steps climbed during the 6MST and in the six-minute walk distance (in m and % of predicted), as well as a significant decrease in the sensation of dyspnea during the 6MWT. Conclusions: The 6MST showed responsiveness to the PTP. However, the 6MWT appears to be more responsive to the PTP proposed.

Keywords: Exercise test; Exercise; Pulmonary disease, chronic obstructive.

Objective: To evaluate characteristics of physical activities in daily life in COPD patients in Brazil, correlating those characteristics with physiological variables. Methods: Physical activities in daily life were evaluated in 40 COPD patients (18 males; 66 ± 8 years of age; FEV1 = 46 ± 16 % of predicted; body mass index = 27 ± 6 kg/m2) and 30 healthy age- and gender-matched subjects, using a multiaxial accelerometer-based sensor for 12 h/day on two consecutive days. We also assessed maximal and functional exercise capacity, using the incremental exercise test and the six-minute walk test (6MWT), respectively; MIP and MEP; peripheral muscle force, using the one-repetition maximum test and the handgrip test; quality of life, using the Saint George's Respiratory Questionnaire (SGRQ); functional status, using the London Chest Activity of Daily Living questionnaire; and dyspnea sensation, using the Medical Research Council (MRC) scale. Results: Mean walking time/day was shorter for COPD patients than for the controls (55 ± 33 vs. 80 ± 28 min/day; p = 0.001), as movement intensity was lower (1.9 ± 0.4 vs. 2.3 ± 0.6 m/s2; p = 0.004). The COPD patients also tended to spend more time seated (294 ± 114 vs. 246 ± 122 min/day, p = 0.08). Walking time/day correlated with the 6MWT (r = 0.42; p = 0.007) and maximal workload (r = 0.41; p = 0.009), as well as with age, MRC scale score and SGRQ activity domain score (−0.31 ≤ r ≤ −0.43; p ≤ 0.05 for all). Conclusions: This sample of Brazilian patients with COPD, although more active than those evaluated in studies conducted in Europe, were less active than were the controls. Walking time/day correlated only moderately with maximal and functional exercise capacity.

Keywords: Pulmonary disease, chronic obstructive; Motor activity; Exercise tolerance.

Background: Hyperhidrosis or excessive sudoresis is a chronic disease associated with important subjective distress. Objective: To propose a specific questionnaire to evaluate the quality of life of patients with hyperhidrosis. Methods: From October 1995 to March 2002, 378 patients (234 females), with a mean age of 26.8 years, were evaluated before and after video-assisted thoracic sympathectomy. Results: Therapeutic success was obtained in 90% of the procedures. The recurrence rate was 10% for palmar and 11% for axillary hyperhidrosis; 27% of the patients who had recurrence were re-operated successfully. No serious complications were reported. Of the total number of patients, 91% answered to the quality of life questionnaire, and 86% of them reported improvement after the procedure. Conclusions: Thoracic sympathectomy is a therapeutic method capable of changing the quality of life of patients with hyperhidrosis. The questionnaire applied has shown these changes.

Keywords: Thoracic sympathectomy. Quality of life.

Objective: To assess the quality of life (QoL) of patients with cystic fibrosis (CF) followed at a university referral center for CF. Methods: A cross-sectional study involving application of the Cystic Fibrosis Questionnaire (CFQ) and Shwachman score in CF patients between April of 2008 and June of 2009. Results: The sample consisted of 75 patients. The mean age was 12.5 ± 5.1 years (range, 6.1-26.4 years). The patients were divided into three groups by age in years: group I (< 12), II (12-14), and III (≥ 14). The highest and lowest CFQ scores were for the nutrition domain in group III (89.3 ± 16.2) and the social domain in group II (59.5 ± 22.3), respectively. Groups I and III differed significantly regarding the treatment domain (p = 0.001). Regarding Shwachman scores, there were significant differences between patients scoring ≤ 70 and those scoring > 70 in the social (group I; p = 0.045), respiratory (group II; p = 0.053), and digestive (p = 0.042) domains. In group III, severity did not correlate with QoL. In groups I and II, patients with an FEV1 < 80% of predicted did not differ from other patients for any CFQ domain. However, in group III, values for the following domains were significantly lower in patients with an FEV1 < 80%: physical (p = 0.012); body image (p = 0.031); respiratory (p = 0.023), emotional (p = 0.041); and social role (p = 0.024). Conclusions: It is important to assess QoL in CF patients, because it can improve treatment compliance.

Keywords: Cystic fibrosis; Quality of life; Questionnaires.

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.

Objective: To evaluate diaphragmatic mobility in relation to lung function, respiratory muscle strength, dyspnea, and physical activity in daily life (PADL) in patients with COPD. Methods: We included 25 patients with COPD, classified according to the Global Initiative for Chronic Obstructive Lung Disease criteria, and 25 healthy individuals. For all of the participants, the following were evaluated: anthropometric variables, spirometric parameters, respiratory muscle strength, diaphragmatic mobility (by X-ray), PADL, and the perception of dyspnea. Results: In the COPD group, diaphragmatic mobility was found to correlate with lung function variables, inspiratory muscle strength, and the perception of dyspnea, whereas it did not correlate with expiratory muscle strength or PADL. Conclusions: In patients with COPD, diaphragmatic mobility seems to be associated with airway obstruction and lung hyperinflation, as well as with ventilatory capacity and the perception of dyspnea, although not with PADL.

Keywords: Pulmonary disease, chronic obstructive; Diaphragm; Spirometry; Dyspnea; Maximal respiratory pressures.

Background: Multidrug resistant tuberculosis is a threat to tuberculosis control worldwide. In many studies, it has been suggested that inadequate treatment is a risk factor for the development of multidrug resistent tuberculosis. Objective: To identify the factors associated with inadequate treatment in a group of patients with multidrug resistent tuberculosis. Method: The authors identified all drug sensitivity test for tuberculosis performed at the Central Laboratory of the state of Ceará from 1990 to 1999. Retrospective and prospective analysis was performed, comparing the characteristics of adequately treated patients with those of inadequately treated patients. Multidrug resistance was defined as resistance to 2 drugs (rifampin and isoniazid) or more, using the Cox proportional hazards model. Results: Of the 1,500 sensitivity test performed at the Central Laboratory of Public Health of the state of Ceará, 266 revealed multidrug-resistant strains. Of those 266 patients, the authors were able to identify only 153, of whom 19 were excluded. Thus, our sampling consisted of 134 patients. Univariate analysis demonstrated that the significant factors associated with inadequate treatment were: noncompliance with the treatment, extreme poverty, drug intolerance, mistakes in the management of the patients, lack of medication being supplied by the health service, two or more previous treatments, pronounced lung cavities and bilateral lesions on chest X-rays. They found a further association (p < 0.0010) between alcoholism or smoking and noncompliance with the treatment. In the multivariate analysis, the factors associated with an outcome of inadequate treatment were: two or more previous treatments (p < 0.0001, OR = 5.9; CI 95%: 2.5-13.7), pronounced cavitation (p < 0.0217, OR = 2.7; CI 95%: 1.2-6.1) and bilateral chest X-ray lesions (p < 0.0226, OR = 3.2; CI 95%: 1.4-7.4) Conclusion: In the study, the authors observed that factors related to inadequate treatment are wide-ranging. An attempt at better control of the disease is warranted, especially in those patients with pronounced cavitation or bilateral lesions in chest X-rays. And those with 2 or more previous treatments.

Keywords: Tuberculosis, multidrug-resistant. Risk factors.

Objective: To correlate health-related quality of life (HRQL) of women with COPD with clinical parameters and with the six-minute walk distance (6MWD; six-minute walk test). Methods: Cross-sectional study involving 30 female patients diagnosed with mild or moderate COPD treated at the Respiratory Outpatient Clinic of the Presidente Dutra University Hospital. Patients completed the Saint George's Respiratory Questionnaire (SGRQ) and were evaluated in terms of respiratory pressures, spirometry parameters and 6MWD. Descriptive statistical analysis was carried out, as were Student's t-tests for dependent variables, together with Pearson's and Spearman's correlation coefficients for numerical and ordinal variables, respectively. Results: According to the SGRQ total scores, HRQL was impaired, to some extent, in most of the participants. Most SGRQ total scores were between the second and the third quartiles, reflecting poor HRQL. The participants also presented poor functional capacity. Mean 6MWD (317.7 m), inspiratory muscle strength (−53.48 cmH2O) and expiratory muscle strength (69.5 cmH2O) were all below reference values. We found that HRQL was not correlated with body mass index or pulmonary function. However, HRQL presented a negative linear correlation with age, MIP and 6MWD, as well as a positive correlation with the sensation of dyspnea and fatigue. Conclusions: In this study, HRQL, determined using the SGRQ, was severely impaired in COPD patients, who presented severe limitations in functional capacity, breath control and personal life.

Keywords: Quality of life; Pulmonary disease, chronic obstructive; Women.

Primary ciliary dyskinesia (PCD) is a genetic disorder that is typically inherited in an autosomal recessive manner. It is clinically characterized by recurrent respiratory infections. However, its repercussions for patient quality of life should not be overlooked. Studies have shown that PCD has a significant impact on the lives of patients, although there are as yet no PCD-specific markers of quality of life. To address that problem, researchers in the United Kingdom developed a quality-of-life questionnaire for patients with PCD. The present communication focuses on the process of translating that questionnaire into Brazilian Portuguese, through a partnership between researchers in Brazil and those in the United Kingdom, as well as its subsequent application in patients in Brazil.

Keywords: Quality of life; Kartagener syndrome; Surveys and questionnaires.