scholarly journals Cross-sectional validity of the EQ-5D-Y as a generic health outcome instrument in children and adolescents with cystic fibrosis in Germany

2009 ◽  
Vol 9 (1) ◽  
Author(s):  
Daniela Eidt-Koch ◽  
Thomas Mittendorf ◽  
Wolfgang Greiner
Keyword(s):  
Health Outcome ◽  
Cystic Fibrosis ◽  
Children And Adolescents ◽  
Cross Sectional ◽  
Outcome Instrument

scholarly journals FACTORS ASSOCIATED TO QUALITY OF LIFE IN CHILDREN AND ADOLESCENTS WITH CYSTIC FIBROSIS

2020 ◽  
Vol 38 ◽  
Author(s):  
Nelbe Nesi Santana ◽  
Célia Regina Moutinho de Miranda Chaves ◽  
Christine Pereira Gonçalves ◽  
Saint Clair dos Santos Gomes Junior
Keyword(s):  
Quality Of Life ◽  
Cystic Fibrosis ◽  
Nutritional Status ◽  
Children And Adolescents ◽  
Functional Capacity ◽  
Clinical Status ◽  
Forced Expiratory Volume ◽  
Cross Sectional ◽  
Function Test

ABSTRACT Objective: To verify the association between quality of life, functional capacity and clinical and nutritional status in children and adolescents with cystic fibrosis (CF). Methods: Cross-sectional study, including patients from eight to 18 years old with CF. Quality of life, functional capacity, nutritional status and clinical status were evaluated with the Cystic Fibrosis Questionnaire; the 6-minute walk test (6MWT) and manual gripping force (MGF); the height percentiles for age and body mass index for age and respiratory function test, respectively. Pearson and Spearman correlation tests and logistic regression were used to analyze the data. Results: A total of 45 patients, 13.4±0.5 years old, 60% female, 60% colonized by Pseudomonas aeruginosa and 57.8% with at least one F508del mutation participated in the study. When assessing the perception of quality of life, the weight domain reached the lowest values, and the digestive domain, the highest. In the pulmonary function test, the forced expiratory volume of the first second was 77.3±3.3% and the 6MWT and MGF presented values within the normal range. There was an association between quality of life and functional capacity, nutritional status and clinical status of CF patients. Conclusions: The study participants had good clinical conditions and satisfactory values of functional capacity and quality of life. The findings reinforce that the assessment of quality of life may be important for clinical practice in the management of treatment.

scholarly journals TREATMENT ADHERENCE AMONG CHILDREN AND ADOLESCENTS IN A CYSTIC FIBROSIS REFERENCE CENTER

2020 ◽  
Vol 38 ◽  
Author(s):  
Bianca Sampaio Bonfim ◽  
Valmir Machado de Melo Filho ◽  
Fernanda Matos Fontenelle ◽  
Edna Lúcia Souza
Keyword(s):  
Cystic Fibrosis ◽  
Children And Adolescents ◽  
Treatment Adherence ◽  
Multidisciplinary Team ◽  
Laboratory Data ◽  
The Self ◽  
Cross Sectional ◽  
Reference Center ◽  
The Mean ◽  
The One

ABSTRACT Objective: To evaluate the level of self-referenced treatment adherence (TA) and its association with clinical and sociodemographic variables in patients with cystic fibrosis assisted at a reference center, as well as compare the level of self-referenced TA with that presumed by the multidisciplinary team. Methods: This is a cross-sectional study that included children and adolescents aged between 0-20 years with cystic fibrosis. Adolescents older than 14 years or their guardians, when younger than 14 years old, were interviewed using a standardized questionnaire. Professionals from the multidisciplinary clinic filled out another form with their impressions of the patients’ TA. Clinical and laboratory data were obtained in the medical records. The TA was considered satisfactory if the total adherence index (TAI) was equal or higher than 80%. Results: 53 patients were included with a median age of 112 months. The mean TAI was 83.2%. The mean TAIs for dornase alfa, pancreatic enzymes, continued use of inhaled tobramycin, vitamins supplements, nutritional supplements and dietary orientation was respectively: 86.1; 96.6; 78.6; 88.1; 51.8 and 78%. Children younger than 14 years presented better TA (p=0.021). The correlation between the self-referenced TA and the one presumed by the multidisciplinary team ranged from 0,117 to 0.402, being higher for Psychology and Nutrition professionals. Conclusions: The TAI was high particularly among children younger than 14 years. There was a positive correlation between the self-referenced TA and the one presumed by the Psychology (p=0.032) and the nutrition (p=0.012) professionals.

scholarly journals The Level of Physical Activity, Lung Function and Exercise Capacity of Children and Adolescents With Cystic Fibrosis Compared to Healthy Controls

2020 ◽  
Author(s):  
Marjane Cardoso ◽  
Caroline Jacoby Schmidt ◽  
Gabriela Motter ◽  
Gabrielle Costa Borba ◽  
Tatiana Helena Rech ◽  
...  
Keyword(s):  
Physical Activity ◽  
Cystic Fibrosis ◽  
Lung Function ◽  
Children And Adolescents ◽  
Functional Capacity ◽  
Control Group ◽  
Healthy Children ◽  
Healthy Controls ◽  
Cross Sectional ◽  
Significant Difference

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.

scholarly journals Nocturnal hypoxemia in children and adolescents with cystic fibrosis

2013 ◽  
Vol 39 (6) ◽  
pp. 667-674 ◽  
Author(s):  
Regina Terse Trindade Ramos ◽  
Maria Angélica Pinheiro Santana ◽  
Priscila de Carvalho Almeida ◽  
Almério de Souza Machado Júnior ◽  
José Bouzas Araújo-Filho ◽  
...  
Keyword(s):  
Cystic Fibrosis ◽  
Nutritional Status ◽  
Lung Disease ◽  
Children And Adolescents ◽  
Sleep Time ◽  
Cross Sectional Study ◽  
Apnea Hypopnea Index ◽  
Cross Sectional ◽  
Nocturnal Hypoxemia ◽  
Respiratory Events

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.

scholarly journals Physiological predictors of cardiorespiratory fitness in children and adolescents with cystic fibrosis without ventilatory limitation

2022 ◽  
Vol 16 ◽  
pp. 175346662110701
Author(s):  
Marcella Burghard ◽  
Tim Takken ◽  
Merel M. Nap-van der Vlist ◽  
Sanne L. Nijhof ◽  
C. Kors van der Ent ◽  
...  
Keyword(s):  
Cystic Fibrosis ◽  
Children And Adolescents ◽  
Cardiorespiratory Fitness ◽  
Cardiopulmonary Exercise Testing ◽  
Chloride Concentration ◽  
Lung Infection ◽  
Forced Expiratory Volume ◽  
Cross Sectional ◽  
Ventilatory Reserve ◽  
Ventilatory Limitation

Objectives: [1] To investigate the cardiorespiratory fitness (CRF) levels in children and adolescents with cystic fibrosis (CF) with no ventilatory limitation (ventilatory reserve ⩾ 15%) during exercise, and [2] to assess which physiological factors are related to CRF. Methods: A cross-sectional study design was used in 8- to 18-year-old children and adolescents with CF. Cardiopulmonary exercise testing was used to determine peak oxygen uptake normalized to body weight as a measure of CRF. Patients were defined as having ‘low CRF’ when CRF was less than 82%predicted. Physiological predictors used in this study were body mass index z-score, P. Aeruginosa lung infection, impaired glucose tolerance (IGT) including CF-related diabetes, CF-related liver disease, sweat chloride concentration, and self-reported physical activity. Backward likelihood ratio (LR) logistic regression analysis was used. Results: Sixty children and adolescents (51.7% boys) with a median age of 15.3 years (25th–75th percentile: 12.9–17.0 years) and a mean percentage predicted forced expiratory volume in 1 second of 88.5% (±16.9) participated. Mean percentage predicted CRF (ppVO2peak/kg) was 81.4% (±12.4, range: 51%–105%). Thirty-three patients (55.0%) were classified as having ‘low CRF’. The final model that best predicted low CRF included IGT ( p = 0.085; Exp(B) = 6.770) and P. Aeruginosa lung infection (p = 0.095; Exp(B) = 3.945). This model was able to explain between 26.7% and 35.6% of variance. Conclusions: CRF is reduced in over half of children and adolescents with CF with normal ventilatory reserve. Glucose intolerance and P. Aeruginosa lung infection seem to be associated to low CRF in children and adolescents with CF.

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

2022 ◽  
Vol 40 ◽  
Author(s):  
Rodrigo dos Santos Lugao ◽  
Roberta Ribeiro Batista Barbosa ◽  
Pitiguara de Freitas Coelho ◽  
Fernanda Mayrink Gonçalves Liberato ◽  
Pâmela Reis Vidal ◽  
...  
Keyword(s):  
Cystic Fibrosis ◽  
Heart Rate ◽  
Heart Rate Variability ◽  
Pulmonary Function ◽  
Children And Adolescents ◽  
Sleep Disorders ◽  
Sleep Apnea Syndrome ◽  
Forced Expiratory Volume ◽  
Cross Sectional ◽  
Obstructive Sleep

ABSTRACT 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.

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