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.

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.

Objectives: To characterize the main identified mutations on cystic fibrosis transmembrane conductance regulator (CFTR) in a group of children and adolescents at a cystic fibrosis center and its association with the clinical and laboratorial characteristics. Method: Descriptive cross-sectional study including patients with cystic fibrosis who had two alleles identified with CFTR mutation. Clinical, anthropometrical, laboratorial and pulmonary function (spirometry) data were collected from patients' records in charts and described with the results of the sample genotyping. Results: 42 patients with cystic fibrosis were included in the study. The most frequent mutation was F508del, covering 60 alleles (71.4%). The second most common mutation was G542X (six alleles, 7.1%), followed by N1303K and R1162X mutations (both with four alleles each). Three patients (7.14%) presented type III and IV mutations, and 22 patients (52.38%) presented homozygous mutation for F508del. Thirty three patients (78.6%) suffered of pancreatic insufficiency, 26.2% presented meconium ileus, and 16.7%, nutritional deficit. Of the patients in the study, 59.52% would be potential candidates for the use of CFTR-modulating drugs. Conclusions: The mutations of CFTR identified more frequently were F508del and G542X. These are type II and I mutations, respectively. Along with type III, they present a more severe cystic fibrosis phenotype. More than half of the sample (52.38%) presented homozygous mutation for F508del, that is, patients who could be treated with Lumacaftor/Ivacaftor. Approximately 7% of the patients (7.14%) presented type III and IV mutations, therefore becoming candidates for the treatment with Ivacaftor.

Keywords: Cystic fibrosis; Mutations; Genetics; Phenotype; Child

Objective: To verify association between left ventricular (LV) mass and thickness and the presence of significant nocturnal hypoxemia in patients with COPD with mild diurnal hypoxemia. Methods: A cross-sectional study carried out in clinically stable outpatients with COPD and mild hypoxemia (oxygen saturation ≥90 to ≤94%, identified by noninvasive oximetry) in a clinic specialized in the treatment of respiratory diseases in Goiânia-GO. All patients were submitted to clinical evaluation, spirometry, polysomnography, echocardiography, arterial blood gas analysis, 6-minute walk test and chest X-ray. Results: Patients with significant nocturnal hypoxemia had echocardiographic parameters associated with increase of LV musculature when compared to patients with mild nocturnal hypoxemia. The LV volume/mass ratio was significantly lower in the group with significant nocturnal hypoxemia (ratio 0.64 ± 0.13 versus 0.72 ± 0.12, p = 0.04), the thickness diastolic diameter of the interventricular septum and the diastolic thickness of the LV posterior wall were significantly higher in this group (9.7 ± 0.92 versus 9.1 ± 0.90 p = 0.03), (9.7 ± 1.0 versus 8.9 ± 1.0, p = 0.01. The time in REM sleep with saturation below 85% significantly predicted septum thickness (adjustment for BMI, age and mean blood pressure, r2 = 0.20; p = 0.046). Conclusion: We observed association between severe REM sleep hypoxemia and echocardiographic parameters indicating increased LV mass in individuals with COPD and significant nocturnal hypoxemia. This suggests that this subgroup of individuals may benefit from an echocardiographic evaluation of the left ventricle.

Keywords: Chronic Obstructive Pulmonary Disease; Left ventricular hypertrophy; Echocardiography.

Objective: To evaluate the level of self-reported adherence to physical therapy recommendations in pediatric patients (6-17 years) with cystic fibrosis (CF) and to ascertain whether the different levels of adherence correlate with pulmonary function, clinical aspects, and quality of life. Methods: This was a cross-sectional study. The patients and their legal guardians completed a questionnaire regarding adherence to physical therapy recommendations and a CF quality of life questionnaire. We collected demographic, spirometric, and bacteriological data, as well as recording the frequency of hospitalizations and Shwachman-Kulczycki (S-K) clinical scores. Results: We included 66 patients in the study. Mean age, FEV1 (% of predicted), and BMI were 12.2 ± 3.2 years, 90 ± 24%, and 18.3 ± 2.5 kg/m2, respectively. The patients were divided into two groups: high-adherence (n = 39) and moderate/poor-adherence (n = 27). No statistically significant differences were found between the groups regarding age, gender, family income, and total S-K clinical scores. There were statistically significant differences between the high-adherence group and the moderate/poor-adherence group, the latter showing lower scores for the "radiological findings" domain of the S-K clinical score (p = 0.030), a greater number of hospitalizations (p = 0.004), and more days of hospitalization in the last year (p = 0.012), as well as lower scores for the quality of life questionnaire domains emotion (p = 0.002), physical (p = 0.019), treatment burden (p < 0.001), health perceptions (p = 0.036), social (p = 0.039), and respiratory (p = 0.048). Conclusions: Low self-reported adherence to physical therapy recommendations was associated with worse radiological findings, a greater number of hospitalizations, and decreased quality of life in pediatric CF patients.

Keywords: Cystic fibrosis; Patient compliance; Physical therapy modalities; Quality of life.

Objective: To evaluate spirometric patterns of respiratory disorders and their relationship with functional severity and maximal expiratory flows at low lung volumes in patients with cystic fibrosis (CF). Methods: A retrospective cross-sectional study including adolescents and adults with CF. All of the patients were submitted to spirometry. Patients were classified as having preserved respiratory function, obstructive lung disease (OLD), OLD with reduced FVC, presumptive restrictive lung disease (RLD) or mixed obstructive and restrictive lung disease (MORLD). Maximal expiratory flows at low lung volumes were assessed using FEF25-75%, FEF75% and FEF75%/FVC. We included 65 normal subjects, also submitted to spirometry, as a control group. Results: The study group included 65 patients: 8 (12.3%) with preserved lung function; 18 (27.7%) with OLD; 24 (36.9%) with OLD and reduced FVC; 5 (7.7%) with presumptive RLD; and 10 (15.4%) with MORLD. The FEV1 was significantly lower in the OLD with reduced FVC group and the MORLD group than in the other groups (p < 0.001). In the patients with preserved respiratory function, FEF25-75% and FEF75% were significantly reduced in 1 patient, as was FEF75%/FVC in 2 patients. Conclusions: The respiratory pattern was impaired in 88% of the patients with CF. The most common pattern was OLD with reduced FVC. The degree of functional impairment was greater in the OLD with reduced FVC group and in the MORLD group than in the other groups. Maximal expiratory flows at low lung volumes were impaired in a low percentage of patients with preserved respiratory function.

Keywords: Cystic fibrosis; Respiratory function tests; Spirometry; Maximal expiratory flow-volume curves.

Objective: To analyze the shuttle walk test, and its respective retest, comparing children with cystic fibrosis (CF) to normal children. Methods: The children were divided into two groups: the CF group, composed of children in whom the diagnosis had been confirmed through sweat testing; and the control group, composed of normal children with no history of pulmonary diseases and no alterations in respiratory function. The children were submitted to at least two consecutive tests, 30 min apart. We evaluated distance walked, cardiac overload, peripheral oxygen saturation (SpO2) and subjective perception of exertion (dyspnea at rest scale and Borg dyspnea scale). Results: A total of 28 children were evaluated. Ages ranged from 7 to 15 years (11.57 ± 2.50 and 11.28 ± 1.85 years for the CF and control groups, respectively). The Borg scale scores were significantly higher in the controls (p = 0.007). No differences were found regarding cardiac overload and SpO2. In relation to the intergroup retest, the controls presented significant improvements on the second test, both in the distance walked and in dyspnea at rest (p = 0.014 and p = 0.036, respectively). The CF group presented a significant improvement only in the dyspnea at rest score (p = 0.168 and p = 0.042, respectively). Conclusion: The cardiac overload imposed by the test did not differ between the groups. The greater fatigue at the beginning of the second test suggests that the 30 min rest between the tests was insufficient.

Keywords: Cystic fibrosis; Exercise test; Heart rate.

Objective: To determine the nocturnal oximetry pattern in chronic obstructive pulmonary disease patients having no sleep apnea and presenting mild daytime hypoxemia, to identify probable daytime parameters capable of predicting nocturnal desaturation, and to evaluate the influence of nocturnal desaturation on the sleep pattern of these patients. Methods: Twenty-five patients were divided into two groups: those with nocturnal desaturation and those without. Results: Comparing the first group (52%) with the second, we found the following: age, 63 + 5 years versus 63 + 6 years; forced expiratory volume in the first second = 53 + 31% versus 56 + 19% predicted; ratio of forced expiratory volume in the first second to forced vital capacity, 49 + 14% versus 52 + 10%; arterial oxygen tension, 68 + 8 mmHg versus 72 + 68 mmHg; and arterial oxygen saturation, 93 + 2% versus 94 + 1%. Patients in the nocturnal desaturation group presented lower daytime arterial oxygen saturation and nocturnal arterial oxygen saturation by pulse oximetry. There was no difference between the two groups in terms of the sleep patterns observed. The ratio of forced expiratory volume in the first second to forced vital capacity was found to correlate with forced vital capacity, daytime arterial oxygen tension and daytime arterial oxygen saturation. In addition, arterial oxygen saturation by pulse oximetry during exercise was found to correlate with nocturnal arterial oxygen saturation by pulse oximetry. However, only daytime arterial oxygen saturation was predictive of nocturnal desaturation. Conclusion: The only variable capable of predicting nocturnal desaturation was daytime arterial oxygen saturation. Nocturnal desaturation did not influence the sleep patterns of patients with chronic obstructive pulmonary disease accompanied by mild daytime hypoxemia.

Keywords: Sleep, REM; Pulmonary disease, chronic obstructive; Anoxemia; Spirometry; Wakefulness

Objective: Depression and anxiety have been documented in people with cystic fibrosis (CF), jeopardizing treatment adherence. To date, no studies have assessed the prevalence of psychosocial issues in adolescents with CF in Brazil. We sought to assess the prevalence of depression and anxiety in adolescents with CF in Brazil, as well as the impact of depression and anxiety on treatment adherence. Methods: This was a multicenter, prospective, observational, longitudinal study conducted between 2017 and 2019 at 14 CF referral centers in Brazil. We used standardized tools such as the nine-item Patient Health Questionnaire (for depression), the seven-item Generalized Anxiety Disorder scale (for anxiety), and the eight-item Morisky Medication Adherence Scale (for treatment adherence) in order to collect data on 218 CF patients at two different time points. Results: The prevalence of depression was 19.1% at time point 1 and 15.4% at time point 2. The prevalence of anxiety was 19.1% at time point 1 and 18.0% at time point 2. Depression and anxiety were significantly higher in female patients and lower in those who underwent home physiotherapy or had psychological support. Significant correlations were found between depression and anxiety at both time points, the associations being strongest at time point 1 (r = 0.68; p < 0.001). Most (66.7%) of the study participants reported low adherence to treatment, and the remainder reported either average adherence (in 28%) or high adherence (in 5.3%). Depression and anxiety showed inverse correlations with treatment adherence. Conclusions: The prevalence of depression and anxiety in adolescents with CF in Brazil appears to be similar to that reported in other countries, being higher in females and lower in those undergoing home physiotherapy or receiving psychological care. Depression and anxiety appear to correlate with lower treatment adherence. Treating psychosocial issues may effectively improve rates of treatment adherence in adolescents with CF.

Keywords: Cystic fibrosis; Anxiety; Depression; Treatment adherence and compliance; Adolescent.

Objective: To study the effects of nocturnal hypoxemia in patients with chronic obstructive pulmonary disease without obstructive sleep apnea syndrome. Methods: We studied 21 patients-10 desaturators and 11 nondesaturators-submitted to arterial blood gas analysis, polysomnography, spirometry, cardiopulmonary exercise testing (cycle ergometer), and hand-grip dynamometry, as well as measurements of maximal inspiratory pressure, maximal expiratory pressure, and C-reactive protein (CRP) levels. Patients with arterial oxygen tension > 60 mmHg were included; those with an apnea-hypopnea index > 5 events/hour of sleep were excluded. Maximal oxygen uptake, maximal power, systolic blood pressure, diastolic blood pressure (DBP), and maximal heart rate were measured during exercise in order to detect hemodynamic alterations. Patients presenting CRP levels above 3 mg/L were considered CRP-positive. Results: Minimal peripheral oxygen saturation during sleep was significantly higher among nondesaturators (p = 0.03). More desaturators presented CRP > 3 mg/L (p < 0.05). No differences were observed in terms of any variables, However, mean peripheral oxygen saturation during sleep correlated with DBP and maximal inspiratory pressure (p < 0.001 and p = 0.001, respectively). Conclusions: Although nocturnal hypoxemia does not reduce exercise capacity or hand-grip strength in patients with mild/moderate COPD, its effect on maximal exercise DBP seems to depend on the degree of hypoxemia. In addition, there is a positive relationship between maximal inspiratory pressure and mean peripheral oxygen saturation during sleep, as well as evidence of pronounced inflammatory activation in patients with nocturnal hypoxemia.

Keywords: Pulmonary disease, chronic obstructive; Exercise test; Anoxia; Respiratory function tests.

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 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: 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: Although various strategies have been proposed for eradicating Pseudomonas aeruginosa in patients with cystic fibrosis (CF), only a few employ multistep treatment in children colonized by that pathogen for the first time. The aim of this study was to describe the effectiveness of a three-phase eradication protocol, initiated after the first isolation of P. aeruginosa, in children with CF in Brazil. Methods: This was a retrospective real-life study in which we reviewed the medical records of pediatric CF patients in whom the eradication protocol was applied between June of 2004 and December of 2012. The three-phase protocol was guided by positive cultures for P. aeruginosa in airway secretions, and the treatment consisted of inhaled colistimethate and oral ciprofloxacin. Success rates were assessed after each phase, as well as cumulatively. Results: During the study period, 47 episodes of P. aeruginosa colonization, in 29 patients, were eligible for eradication. Among the 29 patients, the median age was 2.7 years, 17 (59%) were male, and 19 (65%) had at least one F508del allele. All 29 patients completed the first phase of the protocol, whereas only 12 and 6 completed the second and third phases, respectively. Success rates for eradication in the three treatment phases were 58.6% (95% CI: 40.7-74.5), 50.0% (95% CI: 25.4-74.6), and 66.7% (95% CI: 30.0-90.3), respectively. The cumulative success rate was 93.1% (95% CI: 78.0-98.1). Treatment failure in all three phases occurred in only 2 patients. Conclusions: In this sample of patients, the multistep eradication protocol was effective and had a high success rate.

Keywords: Cystic fibrosis/therapy; Cystic fibrosis/prevention & control; Pseudomonas aeruginosa; Treatment outcome.

Objective: To determine, through home visits, the rate of compliance with environmental control measures in the homes of children with asthma. Methods: This study involved 98 asthma patients between the ages of 4 and 15. The parents of those children and adolescents received instruction in how to carry out environmental control measures and were encouraged to perform such measures continuously for a period of 90 days. Home visits, which included direct inspection of the domicile and administration of a questionnaire, were made before and after this 90-day period. In cases of noncompliance, parents were asked to explain why they did not carry out the control measures. Statistical analysis was performed using the McNemar test. Results: Overall compliance with the various items studied was 11.1%, ranging from -4.1% (for curtain control, p = 0.63) to 22.6% (for stuffed toys, p < 0.001). Passive smoking was reduced to 9.7% (p = 0.02). Among the families studied, the mean monthly income was 2.5 times the national minimum wage. When asked why they had not adopted the recommended measures, noncompliant parents gave, among others, the following explanations: "economic hardship" (60.1%); "the measures were too difficult to carry out" (6.1%); "nonparticipation of the father" (4%); and "lack of time on the part of the mother" (4%). Conclusion: Environmental control measures were carried out sporadically, possibly reflecting the influence of socioeconomic and cultural factors.

Keywords: Asthma; Allergens; Environmental exposure; Hypersensivity/prevention & control; Compliance

The objective of this study was to review the literature on bronchoalveolar lavage fluid cell profiles in healthy children and adolescents, as well as on the use of BAL as a diagnostic and follow-up tool for lung disease patients in this age bracket. To that end, we used the Medline database, compiling studies published between 1989 and 2009 employing the following MeSH descriptors (with Boolean operators) as search terms: bronchoalveolar lavage AND cytology OR cell AND child. In healthy children, the cell profile includes alveolar macrophages (> 80%), lymphocytes (approximately 10%), neutrophils (approximately 2%) and eosinophils (< 1%). The profile varies depending on the disease under study. The number of neutrophils is greater in wheezing children, especially in non-atopic children, as well as in those with pulmonary infectious and inflammatory profiles, including cystic fibrosis and interstitial lung disease. Eosinophil counts are elevated in children/adolescents with asthma and can reach high levels in those with allergic bronchopulmonary aspergillosis or eosinophilic syndromes. In a heterogenous group of diseases, the number of lymphocytes can increase. Evaluation of the BAL fluid cell profile, when used in conjunction with clinical and imaging findings, has proven to be an essential tool in the investigation of various lung diseases. Less invasive than transbronchial and open lung biopsies, BAL has great clinical value. Further studies adopting standard international protocols should be carried out. Such studies should involve various age groups and settings in order to obtain reference values for BAL fluid cell profiles, which are necessary for a more accurate interpretation of findings in children and adolescents with lung diseases.

Keywords: Bronchoalveolar lavage; Bronchoalveolar lavage fluid/cytology; Child; Adolescent.

Cystic fibrosis-related diabetes (CFRD) is the principal extra-pulmonary complication of cystic fibrosis, occurring in 15-30% of adult cystic fibrosis patients. The number of cystic fibrosis patients who develop diabetes is increasing in parallel with increases in life expectancy. The aim of this study was to review the physiopathology, clinical presentation, diagnosis and treatment of CFRD. A bibliographic search of the Medline and Latin American and Caribbean Health Sciences Literature databases was made. Articles were selected from among those published in the last twenty years. Insulin deficiency, caused by reduced β-cell mass, is the main etiologic mechanism, although insulin resistance also plays a role. Presenting features of type 1 and type 2 diabetes, CFRD typically affects individuals of approximately 20 years of age. It can also be accompanied by fasting, non-fasting or intermittent hyperglycemia. Glucose intolerance is associated with worsening of nutritional status, increased morbidity, decreased survival and reduced pulmonary function. Microvascular complications are always present, although macrovascular complications are rarely seen. An oral glucose tolerance test is recommended annually for patients ≥ 10 years of age and for any patients presenting unexplained weight loss or symptoms of diabetes. Patients hospitalized with severe diseases should also be screened. If fasting hyperglycemia persists for more than 48 h, insulin therapy is recommended. Insulin administration remains the treatment of choice for diabetes and fasting hyperglycemia. Calories should not be restricted, and patients with CFRD should be managed by a multidisciplinary team.

Keywords: Cystic fibrosis; Diabetes mellitus.

Objective: To test the hypothesis that disease severity in patients with cystic fibrosis (CF) is correlated with an increased risk of sleep apnea. Methods: A total of 34 CF patients underwent clinical and functional evaluation, as well as portable polysomnography, spirometry, and determination of IL-1β levels. Results: Mean apnea-hypopnea index (AHI), SpO2 on room air, and Epworth Sleepiness Scale score were 4.8 ± 2.6, 95.9 ± 1.9%, and 7.6 ± 3.8 points, respectively. Of the 34 patients, 19 were well-nourished, 6 were at nutritional risk, and 9 were malnourished. In the multivariate model to predict the AHI, the following variables remained significant: nutritional status (β = −0.386; p = 0.014); SpO2 (β = −0.453; p = 0.005), and the Epworth Sleepiness Scale score (β = 0.429; p = 0.006). The model explained 51% of the variation in the AHI. Conclusions: The major determinants of sleep apnea were nutritional status, SpO2, and daytime sleepiness. This knowledge not only provides an opportunity to define the clinical risk of having sleep apnea but also creates an avenue for the treatment and prevention of the disease.

Keywords: Cystic fibrosis; Oxygenation; Sleep apnea, obstructive.