共查询到6条相似文献，搜索用时 0 毫秒
1.
Marcus Svedberg Per M. Gustafsson Paul D. Robinson Monica Rosberg Anders Lindblad 《Journal of cystic fibrosis》2018,17(2):236-241
Background
Data on long term variability of Lung Clearance Index (LCI) in Cystic Fibrosis (CF) is urgently needed to guide test result interpretation. Our aim was to evaluate LCI variability in clinically stable CF lung disease in school age children.Methods
Paediatric patients, aged 6 to 17 years, attending the outpatient CF clinic performed Multiple Breath Nitrogen Washout (Exhalyzer® D) and spirometry every third month over a period of one year. Clinical stability was assessed by the Cystic Fibrosis Clinical Score (CFCS) at each visit.Results
Twentyfive children were recruited: baseline median (range) FEV1% pred. 91 (55–122)%, LCI 9.1 (6.4–18.6), CFCS 15 (12 ? 23). A total of 107 visits were included in the study, of which 93% were defined as clinically stable. In clinically stable visits, within-subject variability of LCI and FEV1% pred. were 10% and 16%, respectively. The upper limit of normal (ULN, 95% percentile) of LCI variability during clinical stability was 17%.Conclusions
LCI within-subject variability was low and comparable to FEV1% pred. which strengthen the use of LCI to monitor lung disease progression in CF patients. An increase in LCI > 17% compared to previous LCI-measurement in clinically stable CF patients may therefore indicate early lung disease progression. 相似文献2.
Mirjam Stahl Simon Y. Graeber Cornelia Joachim Sandra Barth Isabell Ricklefs Gesa Diekmann Matthias V. Kopp Lutz Naehrlich Marcus A. Mall 《Journal of cystic fibrosis》2018,17(2):249-255
Background
Lung clearance index (LCI) detects early ventilation inhomogeneity and has been suggested as sensitive endpoint in multicenter intervention trials in infants and preschoolers with cystic fibrosis (CF). However, the feasibility of multicenter LCI in this age group has not been determined. We, therefore, investigated the feasibility of LCI in infants and preschoolers with and without CF in a three-center setting.Methods
Following central training, standardized SF6-MBW measurements were performed in 73 sedated children (10 controls, 49 with CF and 14 with other lung diseases), mean age 2.3 ± 1.2 years across three centers, and data were analyzed centrally.Results
Overall success rate of LCI measurements was 91.8% ranging from 78.9% to 100% across study sites. LCI was increased in patients with CF (P < 0.05) and with other lung diseases (P < 0.05) compared to controls.Conclusion
Our results support feasibility of LCI as multicenter endpoint in clinical trials in infants and preschoolers with CF. 相似文献3.
4.
Kathryn A. Ramsey Caroline McGirr Stephen M. Stick Graham L. Hall Shannon J. Simpson 《Journal of cystic fibrosis》2017,16(6):713-718
Background
We assessed the effect of posture on ventilation distribution and the impact on associations with structural lung disease.Methods
Multiple breath washout (MBW) was performed in seated and supine postures in 25 healthy children and 21 children with CF. Children with CF also underwent a chest CT scan. Functional residual capacity (FRC), lung clearance index (LCI) and moment ratios were calculated from the MBW test. CT scans were evaluated for CF-related structural lung disease.Results
FRC was lower in the supine than in the seated posture, whereas LCI was higher in the supine than in the seated posture. In children with CF, associations between LCI and the extent of structural lung disease were stronger when performed in the supine posture.Conclusions
Body posture influences lung volumes and ventilation distribution in both healthy children and children with CF. MBW testing in the supine posture strengthened associations with structural lung damage. 相似文献5.
Anna M. Georgiopoulos Deborah Friedman Elizabeth A. Porter Amy Krasner Sheetal P. Kakarala Breanna K. Glaeser Siena C. Napoleon Janet Wozniak 《Journal of cystic fibrosis》2018,17(2):276-280
Background
International guidelines recommend depression and anxiety screening in individuals with cystic fibrosis (CF), but Attention-Deficit Hyperactivity Disorder (ADHD) remains understudied.Methods
Adults with CF (n = 53) were screened using the Adult ADHD Self-Report Scale-v1.1 Symptom Checklist (ASRS-v1.1), Cystic Fibrosis Questionnaire-Revised (CFQ-R), and a self-report measure of treatment adherence.Results
Elevated ADHD symptoms on the ASRS-v1.1 screener were reported by 15% of participants. Self-reported adherence, Body Mass Index in kg/m2 (BMI), and Forced Expiratory Volume in 1 Second, Percent Predicted (FEV1%pred) did not differ between participants with vs. without elevated ADHD scores. Three CFQ-R scales, Physical Functioning, Role Functioning, and Respiratory Symptoms, were significantly lower in participants with elevated ADHD screens (unadjusted p < 0.05). This difference remained statistically significant for the Role Functioning and Respiratory Symptoms scales following correction for multiple comparisons.Conclusions
The highly specific screening tool ASRS-v1.1 can ascertain previously undetected ADHD symptoms in adults with CF. ADHD was substantially more prevalent than expected in this population. Elevated ASRS-v1.1 screens correlated with poorer Health-Related Quality of Life (HRQoL) in some domains, but not with BMI, FEV1%pred, or self-reported CF treatment adherence. Additional research will elucidate the impact of ADHD and its treatment on HRQoL, CF self-care and health outcomes. 相似文献6.
William Poncin Florian Singer Anne-Sophie Aubriot Patrick Lebecque 《Journal of cystic fibrosis》2017,16(2):258-266