scholarly journals Lung function determinants and mortality of children and adolescents with cystic fibrosis in South Africa 2007‐2016

2020 ◽  
Vol 55 (6) ◽  
pp. 1381-1387
Author(s):  
Natalie J. Vandenbroucke ◽  
Marco Zampoli ◽  
Brenda Morrow
Trials ◽  
2019 ◽  
Vol 20 (1) ◽  
Author(s):  
Karolinne Souza Monteiro ◽  
Matheus de Paiva Azevedo ◽  
Lucas Menescal Jales ◽  
Fernanda Elizabeth Pereira da Silva ◽  
Ricardo Fernando Arrais ◽  
...  

Abstract Background Individuals with cystic fibrosis (CF) may develop CF-related diabetes (CFDR). This comorbidity is related to a poorer quality of life, microvascular complications, a decline in lung function, and an increase in exacerbations, as well as delayed growth and puberty. Evidence exists that physical exercise contributes to glycemic control in individuals with non-CF-related diabetes. This exercise is usually continuous with moderate intensity and long duration, which can cause muscle dyspnea and fatigue in CF individuals. Aerobic interval training (AIT) emerges as a safe and effective alternative for treating these individuals. The objective of this study is to evaluate the effects of AIT on glucose tolerance in children and adolescents with CF. Methods This study will be a two-arm, prospectively registered, randomized controlled trial with blind assessors and twenty 6- to 18-year-old individuals with cystic fibrosis (CF) from two different Brazilian states. People with CF will be randomly allocated to either the experimental or control group using block randomization, stratified by puberty stage,. Participants from both groups will receive an educational intervention and will be asked to continue their usual daily treatment for the full duration of the study. Those in the experimental group will perform AIT on a cycle ergometer at home three times a week, for 8 consecutive weeks. The sample characterization will include an assessment of puberty stage, socioeconomic status, dyspnea, and anthropometry. The primary outcome will be the change in glucose tolerance, while the secondary outcomes will include lung function, exercise tolerance, respiratory muscle strength, quality of life, and CF exacerbations. All outcomes will be assessed at baseline, week 9, and week 17. Discussion This is the first study to evaluate the effects of AIT on glucose tolerance in children and adolescents with CF. This study will serve as a basis for guiding clinical practice and decision-making in treating glucose intolerance and CF-related diabetes (CFRD) in children and adolescents with CF. Trial registration ClinicalTrials.gov Protocol Registration System: NCT03653949. Registered on August 31, 2018.


Children ◽  
2021 ◽  
Vol 8 (10) ◽  
pp. 857
Author(s):  
Ioanna Loukou ◽  
Maria Moustaki ◽  
Agni Deligianni ◽  
Olympia Sardeli ◽  
Konstantinos Douros

Spirometry is considered the gold standard method for monitoring lung function of patients with cystic fibrosis (CF) but it requires patients’ cooperation and therefore it is not useful for the majority of preschool-aged children. Oscillometry is an alternative modality for lung function monitoring that requires minimal cooperation and can be applied in children as young as 3 years of age. Furthermore, it generates lesser aerosol compared to spirometry, an issue that is of considerable importance in the COVID-19 era. The aim of this review was to present the existing clinical data regarding the application of oscillometry in children and adolescents with CF. The method seems to have acceptable feasibility and repeatability. However, there is conflicting data regarding the correlation of oscillometry values with the clinical symptoms of CF patients either in clinically stable or in exacerbation periods. Furthermore, it is not clear to what extent oscillometry measurements correlate with the spirometry indices. Based on current evidence, spirometry cannot be substituted by oscillometry in the monitoring of the respiratory status of children and adolescents with CF.


2021 ◽  
pp. 1-10
Author(s):  
Pitiguara de Freitas Coelho ◽  
Roberta Ribeiro Batista Barbosa ◽  
Rodrigo dos Santos Lugao ◽  
Fernanda Mayrink Gonçalves Liberato ◽  
Pâmela Reis Vidal ◽  
...  

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 [Formula: see text] years, mean FEV[Formula: see text]%, were included. A sympathovagal balance (LF/HF) increase ([Formula: see text]) 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 ([Formula: see text], [Formula: see text] and [Formula: see text], [Formula: see text]; respectively). MST distance also correlated positively and significantly with SDNN ([Formula: see text], [Formula: see text]), square root of the mean of the sums of squares of frequencies between RR intervals greater than 50[Formula: see text]ms — RMSSD ([Formula: see text], [Formula: see text]), low frequency — LF ([Formula: see text], [Formula: see text]), HF ([Formula: see text], [Formula: see text]), dispersion of points perpendicular to the short-term identity line — SD1 ([Formula: see text], [Formula: see text]) and negatively with LF/HF ([Formula: see text], [Formula: see text]). Regarding daily physical activity, SDNN at rest ([Formula: see text], [Formula: see text]) and exercise ([Formula: see text], [Formula: see text]) 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 ([Formula: see text]) and lower exercise capacity ([Formula: see text]). 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.


2020 ◽  
Author(s):  
Marjane Cardoso ◽  
Caroline Jacoby Schmidt ◽  
Gabriela Motter ◽  
Gabrielle Costa Borba ◽  
Tatiana Helena Rech ◽  
...  

Abstract Background : people with Cystic Fibrosis (CF) have progressive limitation to physical exercise and reduced daily living activities. Regular physical activity (PA) and exercise contribute to the quality of live of people with CF. The objective of this study was to evaluate level of PA , lung function and functional capacity in children and adolescents diagnosed with CF and compare them with those of healthy children and adolescents. Methodology: the study had a cross-sectional design with a control group. Patients with CF were followed at the Children’s Pneumology Outpatient Clinic, and were matched for age and sex with healthy controls from a local public school. The evaluations included daily step count, the shuttle walk test and spirometry. Results: 70 children and adolescents were evaluated, 35 diagnosed with CF and 35 healthy controls. The overall mean age was 11.6±2.9 years. There was no significant difference in level of PA between the patient and control groups. Gender analysis revealed no significant difference in level of PA between the groups or within the CF group. The CF group values were significantly lower than the control group for BMI (p=0.04), percentage of predicted FEV 1 and FEV 1 Z-score (p=0.02 and p=0.010). Conclusion: In this sample, children and adolescents with CF had the same level of PA as their healthy peers. Boys and girls with CF had similar level of PA when stratified by sex, as well as when compared to healthy peers of the same gender. Differences were observed between BMI, FEV 1 and some functional capacity test variables between the groups.


Author(s):  
Nicolas Terliesner ◽  
Mandy Vogel ◽  
Anna Steighardt ◽  
Ruth Gausche ◽  
Constance Henn ◽  
...  

AbstractBackground:Impaired glucose metabolism and cystic fibrosis (CF)-related diabetes (CFRD) are associated with insufficient weight gain and impaired lung function in children and adolescents with CF. We have asked whether imminent CFRD may be a cause of poor growth in children and adolescents.Methods:A retrospective case control study including 32 patients with CF with or without diabetes was conducted. Sixteen pairs, matched according to age, gender and exocrine pancreatic insufficiency, were analysed. Standard deviation scores (SDS) of height, growth, weight, body mass index (BMI), forced vital capacity (FVC), forced expiratory volume in the first second (FEV1) and forced expiratory flow at 75% of expired FVC (FEF75) were recorded during a mean observation period of 13 years per patient.Results:SDS of height and weight were reduced in CF patients with diabetes compared to those without, not only at the point of diagnosis (both p<0.05) but years before the evidence of diabetes. Afterwards there was a significant decline in height (p<0.001) and weight (p<0.01) SDS in CFRD patients and an increasing difference between the height and weight of CF patients with or without diabetes. In contrast, no significant reduction of BMI-SDS was observed in CFRD patients. All analysed lung function parameters showed a marked decline in CFRD patients starting 1 year prior to the diagnosis of diabetes.Conclusions:Deteriorating growth, reduced weight and impaired lung function are related to the development of CFRD and are obvious several years before the actual diagnosis of diabetes.


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