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.

Evidence of validity of internal structure of the Functional Assessment of Chronic Illness Therapy-Spiritual Well-Being Scale (FACIT-Sp-12) in Brazilian adolescents with chronic health conditions.

This study explored the evidence of validity of internal structure of the 12-item Functional Assessment of Chronic Illness Therapy-Spiritual Wellbeing Scale (FACIT-Sp-12) in Brazilian adolescents with chronic health conditions. The study involved 301 Brazilian adolescents with cancer, type 1 diabetes mellitus, or cystic fibrosis. Exploratory Factor Analysis (EFA), Confirmatory Factor Analysis (CFA), and Item Response Theory (IRT) were used to test the internal structure. Reliability was determined with Cronbach's Alpha and McDonald's Omega. The EFA suggested a one-dimensional scale structure in contrast to the original 2-factor model or the 3-factor model which were not reproduced in the current CFA. All quality indicators for the EFA one-factor exceeded the required criteria (FDI = 0.97, EAP = 0.97, SR = 3.96 and EPTD = 0.96, latent GH = 0.90. and the observed GH = 0.85). The FACIT-Sp-12 for adolescents yielded strong evidence for a 1-factor model and with good reliability.

Heart rate variability, exercise capacity and levels of daily physical activity in children and adolescents with mild-to-moderate cystic fibrosis.

Background: Autonomic nervous system balance is altered in cystic fibrosis (CF), although its influence on physical fitness has been poorly explored. Objective: This study aimed to evaluate the association of heart rate variability (HRV) with exercise capacity and levels of daily physical activity in children and adolescents with mild-to-moderate CF. Methods: A cross-sectional study including individuals with CF aged 6-18 years, not under CFTR modulator therapy, was performed. Sociodemographic (age, sex) and clinical information (airway colonization, pancreatic insufficiency, and genotyping) were collected. In addition, exercise capacity (modified shuttle test - MST), lung function (spirometry), body composition (bioimpedance), levels of daily physical activity (5-day accelerometer), and HRV (both at rest and during the MST) were evaluated. Results: 30 individuals (20 females) aged 11 . 2 ± 3 . 7 years, mean FEV 1 62 . 8 ± 27 . 6 %, were included. A sympathovagal balance (LF/HF) increase ( p < 0 . 001 ) during the MST was shown, indicating a predominance of sympathetic modulation. The standard deviation of all RR intervals (SDNN) and the high frequency (HF) index during exercise correlated significantly with FEV1 ( r = 0 . 45 , p = 0 . 01 and r = 0 . 46 , p = 0 . 01 ; respectively). MST distance also correlated positively and significantly with SDNN ( r = 0 . 43 , p = 0 . 01 ), square root of the mean of the sums of squares of frequencies between RR intervals greater than 50 ms - RMSSD ( r = 0 . 53 , p < 0 . 01 ), low frequency - LF ( r = 0 . 48 , p < 0 . 01 ), HF ( r = 0 . 64 , p < 0 . 01 ), dispersion of points perpendicular to the short-term identity line - SD1 ( r = 0 . 40 , p = 0 . 02 ) and negatively with LF/HF ( r =- 0 . 57 , p < 0 . 01 ). Regarding daily physical activity, SDNN at rest ( r = 0 . 37 , p = 0 . 04 ) and exercise ( r = 0 . 41 , p = 0 . 02 ) showed positive correlations with time in moderate-to-vigorous activities. When normalizing the SDNN and classifying individuals as normal or altered, those presenting altered SDNN showed poorest FEV1 ( p = 0 . 001 ) and lower exercise capacity ( p = 0 . 027 ). Conclusion: HRV correlates with lung function, exercise capacity and levels of daily physical activity in children and adolescents with CF. The study highlights the influence of CF on autonomic function and suggests HRV measurement as an easy tool to be used in clinical settings as an alternative marker to monitor CF individuals.

Obstructive sleep apnea in children and adolescents with cystic fibrosis and preserved lung function or mild impairment: a systematic review and meta-analysis of prevalence.

OBJECTIVE/BACKGROUND: Sleep disorders in cystic fibrosis may be present before daytime clinical manifestations, regardless of lung function impairment, affecting quality of life and disease progression. This study investigated the prevalence of obstructive sleep apnea in children and adolescents with cystic fibrosis and preserved lung function or mild impairment, and evaluated its association with clinical variables. METHODS: A systematic review with meta-analysis of prevalence was conducted, including observational studies with polysomnographies in patients with cystic fibrosis who presented mean lung function values > 60% predicted. The methodological quality of the studies was analyzed, and a meta-analysis was performed to assess the prevalence of obstructive sleep apnea. RESULTS: Of the 2318 studies identified, 7 were included in the systematic review and 6 in the meta-analysis of prevalence. The confounding factors and strategies identified were the items with greatest weakness in the methodological quality assessment. Most studies were cross-sectional, and sample size ranged from 9 to 67 individuals. The most frequent criterion for defining obstructive sleep apnea was apnea-hypopnea index (AHI) > 1 per hour. The prevalence found ranged from 32.3 to 100% and the pooled prevalence was 65% (I2 = 53.4%), considering AHI>1, and 52% (I2 = 89.4%) for AHI>2 per hour. It was not possible to verify the association between obstructive sleep apnea and clinical variables. CONCLUSIONS: A high prevalence of obstructive sleep apnea in children and adolescents with cystic fibrosis was found, regardless of age and lung function impairment, reinforcing the importance of investigating sleep-disordered breathing during clinical visits even when lung function is not yet compromised.

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.

Capacidade de exercício e nível de atividade física diária de crianças e adolescentes com fibrose cística: associação com estado nutricional, função pulmonar, hospitalização e uso de antibióticos / Capacidad de ejercicio y nivel de actividad física diaria de niños y adolescentes con fibrosis quística: asociación con estado nutricional, función pulmonar, hospitalización y uso de antibióticos / Exercise capacity and daily physical activity levels in children and adolescents with cystic fibrosis: association with nutritional status, lung function, hospitalization and antibiotic use

RESUMO O objetivo deste trabalho é avaliar a capacidade de exercício e nível de atividade física diária de crianças e adolescentes com fibrose cística e associar com estado nutricional, função pulmonar, tempo de hospitalização e uso de antibióticos. Trata-se de estudo transversal em indivíduos com fibrose cística entre 6 e 18 anos, registrando-se informações sobre perfil clínico, histórico de hospitalizações e uso de antibióticos. Os participantes foram submetidos à espirometria, bioimpedância, avaliação da capacidade de exercício com teste de Shuttle modificado (MST), o nível da atividade física foi medido por meio do questionário internacional de atividade física (IPAQ) e usando acelerômetro por 5 dias. Participaram 30 indivíduos com idade de 11,2 ± 3,6 anos e volume expirado forçado (VEF1) de 68,0 ± 24,8%. A mediana da distância percorrida no MST foi de 820 metros (66,3%) e demonstrou associação com a função pulmonar (r = 0,78), estado nutricional (r = 0,38), tempo de hospitalização (r = -0,42) e uso de antibióticos (r = -0,46). De acordo com o questionário, 20 pacientes (64,6%) foram classificados como sedentários, o acelerômetro revelou que os indivíduos passam 354,2 minutos em atividades sedentárias e apenas 14,9 minutos em atividades moderadas a vigorosas por dia. Quanto maior a porcentagem de tempo em atividade física moderada a vigorosa, maior o índice de massa corpórea (IMC) e menor o tempo de hospitalização. Assim, a capacidade de exercício e nível de atividade física em crianças e adolescentes com fibrose cística apresentou-se reduzida e associada com menores valores de IMC e aumento do tempo de hospitalização.

RESUMEN El propósito de este trabajo fue evaluar la capacidad de ejercicio y el nivel de actividad física diaria de los niños y adolescentes con fibrosis quística, así como asociarlos con el estado nutricional, la función pulmonar, la duración de la permanencia hospitalaria y el uso de antibióticos. Este es un estudio transversal realizado con individuos con fibrosis quística con edades comprendidas entre los 6 y los 18 años, y que registra información sobre el perfil clínico, los antecedentes de hospitalizaciones y el uso de antibióticos. Los participantes se sometieron a la espirometría, la bioimpedancia, la evaluación de la capacidad de ejercicio con la prueba de Shuttle modificada (MST); para medir el nivel de actividad física se aplicó el Cuestionario Internacional de Actividad Física (IPAQ) y el uso del acelerómetro durante 5 días. Participaron 30 personas de entre 11,2 ± 3,6 años de edad y volumen espirado forzado (VEF1) de 68,0 ± 24,8%. La mediana de la distancia recorrida en el MST fue de 820 metros (66,3%) y se mostró asociación con la función pulmonar (r=0,78), el estado nutricional (r=0,38), la duración de la permanencia hospitalaria (r=-0,42) y el uso de antibióticos (r=-0,46). El cuestionario reveló que 20 pacientes (64,6%) estaban sedentarios, y el acelerómetro evidenció que ellos pasan 354,2 minutos en actividades sedentarias y solo 14,9 minutos en actividades moderadas a intensas al día. Cuanto mayor sea el porcentaje de tiempo dedicado a la actividad física moderada a intensa, mayor será el índice de masa corporal (IMC) y menor la duración de la permanencia hospitalaria. Por lo tanto, la capacidad de ejercicio y el nivel de actividad física de niños y adolescentes con fibrosis quística fueron menores y están asociadas con bajos valores de IMC y con una permanencia hospitalaria más prolongada.

ABSTRACT To evaluate the exercise capacity and daily physical activity level among children and adolescents with cystic fibrosis, and its association with nutritional status, lung function, hospitalization time, and days taking antibiotics. This is a cross-sectional study in individuals with cystic fibrosis aged 6 to 18 years. Information on clinical profile, history of hospitalizations and antibiotic use were collected. Participants were submitted to spirometry, bioimpedance, and an assessment of exercise capacity with modified shuttle test (MST), and the level of physical activity was measured with the International Physical Activity Questionnaire (IPAQ) using an accelerometer for 5 days. In total, 30 individuals participated, aged 11.2±3.6 years, and 68.0±24.8% in forced expired volume in the first second (FEV1). The median distance covered in the MST was 820 meters (66.3%), showing association with lung function (r=0.78), nutritional status (r=0.38), hospitalization time (r=-0.42) and antibiotic use (r=-0.46). According to the questionnaire, 20 patients (64.6%) were classified as sedentary, the accelerometer revealed that the individuals spend 354.2 minutes in sedentary activities and only 14.9 minutes in moderate to vigorous activities per day. The higher the percentage of time in moderate to vigorous physical activity, the higher the body mass index (BMI) and the shorter the hospitalization time. Exercise capacity and level of physical activity in children and adolescents with cystic fibrosis is reduced and associated with lower BMI values and with an increase in hospitalization time.

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.

Adherence and barriers to general and respiratory exercises in cystic fibrosis.

OBJECTIVES: To investigate the adherence and the self-reported barriers to general and respiratory exercises reported by individuals with cystic fibrosis (CF). STUDY DESIGN: An exploratory, experimental study. METHODS: Community-dwelling individuals aged 16 years and over, diagnosed with CF, who were accompanied in referral centers were included. Information regarding adherence to exercises was obtained by a questionnaire and reported as a ratio between prescribed exercises and self-reported adherence. The weekly frequency was used to verify adherence to exercise initiation, and the amount of session duration concluded was used to verify adherence to exercise duration. Values above 0.70 were considered as high adherence. Eight demographic and clinical factors were examined to explore their relationships with adherence, and the barriers to exercises were also collected by questionnaire. RESULTS: Thirty-four participants met the inclusion criteria. Overall, adherence to exercise initiation was 0.40 (standard deviation [SD] = 0.3) for general exercises and 0.63 (SD = 0.4) for respiratory exercises. Adherence to exercise duration was 0.76 (SD = 0.4) for general exercises and 0.73 (SD = 0.4) for respiratory exercises. Forced vital capacity (r = 0.39; P = .02) was associated with adherence to the duration of general exercises, and body mass index (r = -0.33; P = .05) was associated with adherence to the duration of respiratory exercises. The main reported barriers were lack of interest, motivation and time, tiredness, noncommitment, and do not recognize the benefits of exercises. CONCLUSIONS: Individuals with CF minded completing the sessions of prescribed exercises once they have initiated it, but most of the days they did not practice general or respiratory exercises.

Sleep-disordered breathing and markers of morbidity in children and adolescents with cystic fibrosis.

BACKGROUND: Studies have shown that sleep disorders occur in cystic fibrosis (CF) patients and may be present before daytime clinical manifestations. OBJECTIVES: To evaluate the presence of sleep disorders among children and adolescents with CF, attempting to identify associations with pulmonary function, nutritional status, days in hospital, and days taking antibiotics. METHODS: Individuals with a diagnosis of CF aged between 6 and 18 years were included. Information on sociodemographic, clinical profile, history of hospitalizations, and use of antibiotics in the last year were collected. Spirometry, bioimpedance, and polysomnography were performed. The presence of nocturnal hypoxemia and obstructive sleep apnea syndrome (OSAS) were evaluated and participants divided according to their presence. RESULTS: Thirty-one patients were included. The prevalence of OSAS was 32.3% and nocturnal hypoxemia was 29.0%. Average nocturnal peripheral oxyhemoglobin saturation (SpO2 ) correlated (P < .001) with forced vital capacity (r = .55) and forced expiratory volume in the first second (r = .62). The higher the percentage of total sleep time (TST) with SpO2 less than 90%, the lower the pulmonary function. Individuals with OSAS and nocturnal hypoxemia had lower spirometric values compared to patients without these disorders, but the nocturnal hypoxemia group also had lower Shwachman-Kulczycki score, longer hospitalization time and antibiotic use. TST with SpO2 less than 90% was associated with length of hospitalization (r2 = .53). CONCLUSION: Children and adolescents with CF have sleep disorders, including OSAS (32.3%) and nocturnal hypoxemia (29%). Individuals with nocturnal hypoxemia presented lower lung function, worse clinical score, and higher morbidity. TST with SpO2 less than 90% was associated with length of hospitalization.

Associação entre pico de fluxo da tosse, colonização bacteriana e estado nutricional em crianças e adolescentes com fibrose cí­stica / Association of cough peak flow with chronic bacterial colonization and nutritional status in children and adolescents with cystic fibrosis

Introdução: Este estudo teve como objetivo avaliar a associação entre pico de fluxo da tosse (PFT), colonização bacteriana crônica e estado nutricional em crianças e adolescentes com Fibrose Cí­stica. Métodos: Estudo transversal, com amostra por conveniência composta por indiví­duos com FC (7-18 anos), cadastrados em um hospital de referência estadual. Foi avaliado o PFT por um medidor portátil de pico de fluxo expiratório. Dados clí­nicos, antropométricos e sobre colonização bacteriana foram colhidos nos prontuários. O estado nutricional foi classificado pelo percentil do í­ndice de massa corporal para a idade. Para a análise estatí­stica foram usados os testes: Shapiro-Wilk, Exato de Fisher, Teste T Student independente, Mann-Whitney e Correlação de Spearman. Foi considerado significante p<0,05. Resultados: Na caracterização da amostra, houve predomí­nio das seguintes caracterí­sticas: redução do PFT (82,35%), risco nutricional (70,6%), colonização bacteriana crônica (82,4%). Indiví­duos colonizados por Pseudomonas aeruginosa apresentaram maior percentual de redução do PFT (p=0,045). Conclusão: O pico de fluxo da tosse apresentou-se reduzido nas crianças e adolescentes com FC da amostra estudada, sendo essa redução mais acentuada nos indiví­duos colonizados por Pseudomonas aeruginosa do que naqueles colonizados por Staphylococcus aureus. A maioria dos indiví­duos apresentava-se em risco nutricional, mas não foi observada correlação entre estado nutricional e pico de fluxo da tosse. (AU)

Introduction: The aim of this study was to evaluate the association among cough peak flow (CPF), chronic bacterial colonization and nutritional status in children and adolescents diagnosed with cystic fibrosis. Methods: A cross-sectional study with a convenience sample of individuals diagnosed with CF (7-18 years old) enrolled in a referral hospital of a Brazilian State. The CPF was evaluated by a portable expiratory peak flow meter. Clinical, anthropometric and bacterial colonization data were collected in the medical records. Nutritional status was classified according to the percentile of the body mass index for age. Statistical analysis was performed using the following tests: Shapiro Wilk, Fisher's Exact, Student's t, Mann-Whitney and Spearman's Correlations, being considered significant p<0.05. Results: The following characteristics predominated: CPF reduction (82.35%), nutritional risk (70.6%), chronic bacterial colonization (82.4%). Individuals colonized by Pseudomonas aeruginosa had a higher percentage of CPF reduction (p=0.045). Conclusion: The cough peak flow was reduced in the children and adolescents with cystic fibrosis in the studied sample, and this reduction was more pronounced in individuals colonized by Pseudomonas aeruginosa than in those colonized by Staphylococcus aureus. Most individuals were at nutritional risk, but no correlation was observed between nutritional status and cough peak flow. (AU)