Clinical and anthropometric evolution of individuals with cystic fibrosis during COVID-19 pandemic: A 24-month cohort study.

OBJECTIVES: To analyze the evolution of clinical and anthropometric characteristics of children and adolescents with cystic fibrosis (CF) over 24 months, including the period of the COVID-19 pandemic. METHODS: A longitudinal study with data collection from May 2018 to November 2020 in physical and electronic records from a pediatric reference center, including individuals with CF aged up to 18 years. RESULTS: The sample encompassed 72 individuals. Weight (p < 0.01), height (p < 0.01), and body mass index (BMI) (p = 0.043) were higher in 2020 than in 2018. There were no significant changes in BMI-Z (p = 0.977) and in percentiles of weight (p = 0.540), height (p = 0.458), and BMI percentile (p = 0.454) between both periods. Pancreatic insufficiency was observed in 91.7% of patients in 2020, and there were twice as many confirmed cases of diabetes compared to 2018. There was a 9.7% increase in individuals colonized by the oxacillin-sensitive Staphylococcus aureus (OSSA) (p = 0.039) and an 11.1% reduction in non-colonized individuals (p = 0.008). CONCLUSION: Although there was an increase in weight, height, and BMI from 2018 to 2020, there were no significant changes in BMI-Z and in percentiles of weight, height, and BMI percentile, suggesting that the anthropometric aspects of nutritional status did not change in this period of 2 years. Moreover, there was an increase in the prevalence of individuals colonized by OSSA and a reduction in the prevalence of individuals non-colonized with any bacteria.

Association of sleep disorders with heart rate variability in children and adolescents with cystic fibrosis.

OBJECTIVE: To assess the association of sleep disorders with the findings of heart rate variability (HRV) in children and adolescents with cystic fibrosis (CF). METHODS: Cross-sectional study including children and adolescents aged six to 18 years with a clinical diagnosis of CF. Sociodemographic and clinical data were collected. Sleep disorders were evaluated using baseline nocturnal polysomnography. The autonomic nervous system (ANS) was evaluated through resting HRV. RESULTS: A total of 30 individuals (11.2 years) with a mean forced expiratory volume in the first second (FEV1) of 62.7% were included. The respiratory disturbance index presented a median of 2.6 and obstructive sleep apnea syndrome (OSAS) was identified in 30%. In the HRV analysis, a mean standard deviation of all inter-beat (RR) intervals (SDNN) of 60.8±45.9ms was found. There was a significant correlation between the HRV low-frequency/high-frequency (LF/HF) global modulation index and the minimum SpO2 during sleep in patients with FEV1<60% (r=0.71; p=0.02). The prevalence of sleep disorders and HRV abnormalities was higher in individuals with lesser pulmonary function (FEV1<60%). CONCLUSIONS: The results indicate a weak correlation of sleep disorders (minimum SpO2) with HRV parameters (LH/HF) in children and adolescents with CF. When pulmonary function was reduced, a stronger correlation was found, highlighting the influence of disease severity. A high prevalence of ANS disorders, nocturnal hypoxemia, and presence of OSAS was also found.

Sleep disorders are distinctively associated with exercise intolerance and sedentary behavior in children with cystic fibrosis.

OBJECTIVE: To evaluate the presence of sleep disorders and its associations with exercise capacity and daily physical activity levels among children and adolescents with CF. METHODS: Children age 6-18 years with a diagnosis of CF were recruited. Information regarding sociodemographic profile, pulmonary function and nutritional status were collected. Sleep disorders (polysomnography), exercise capacity (modified shuttle test - MST) and daily physical activity levels (questionnaire and five days accelerometer use) were evaluated. RESULTS: Thirty-one patients, median age of 9.6 years and forced expiratory volume in 1 s (FEV1) of 68.1 ± 24.4%, were included. Obstructive sleep apnea syndrome (OSAS) was present in 32.3% and nocturnal hypoxemia in 29%. The MST distance correlated with the mean peripheral oxyhemoglobin saturation (SpO2) during sleep (r = 0.40) and the percent of total sleep time with SpO2<90% (r = -0.49). The final MST SpO2 correlated with the occurrence of OSAS (r = -0.48) and mean nocturnal SpO2 (r = 0.45). Sedentary activities, as measured by accelerometry, correlated with sleep architecture, including the percent of stage II (r = 0.60) and rapid eye movement (REM) stage sleep (r = -0.37). Patients with OSAS and nocturnal hypoxemia presented lower values (p < 0.05) of distance and final SpO2 in the MST. Nocturnal hypoxemia was the main variable to influence exercise capacity (r2 = 0.521). CONCLUSION: Sleep disorders are distinctively related with exercise capacity and daily physical activity levels, as nocturnal hypoxemia is associated with exercise intolerance and sleep architecture disorders are associated with sedentary physical activity levels.