scholarly journals Alternative inert gas washout outcomes in patients with primary ciliary dyskinesia

2017 ◽  
Vol 49 (1) ◽  
pp. 1600466 ◽  
Author(s):  
Sylvia Nyilas ◽  
Anne Schlegtendal ◽  
Florian Singer ◽  
Myrofora Goutaki ◽  
Claudia E. Kuehni ◽  
...  
Keyword(s):  
Primary Ciliary Dyskinesia ◽  
Small Airways ◽  
Ventilation Inhomogeneity ◽  
Single Breath ◽  
Promising Tool ◽  
Moment Ratio ◽  
Measurement Duration ◽  
Multiple Breath Washout ◽  
Z Scores ◽  
Ciliary Dyskinesia

The lung clearance index (LCI) derived from a nitrogen multiple breath washout test (N2-MBW) is a promising tool to assess small airways disease in primary ciliary dyskinesia, but it is difficult to apply in routine clinical settings because of its long measuring time. In this study, we aimed to assess alternative indices derived from shorter washout protocols.49 patients with primary ciliary dyskinesia (mean age 14.7±6.6 years) and 37 controls (mean age 14.3±1.4 years) performed N2-MBW and double-tracer gas (DTG) single-breath washout tests. Global (LCI and moment ratio (M2/M0)), conductive (Scond) and acinar ventilation inhomogeneity (DTG Slope III (SIII-DTG)) were determined for each individual. The main outcomes were 1) the ability to detect abnormal lung function from washout indices (>1.64 z-scores) and 2) measurement duration.The prevalence of abnormal values for LCI2.5% was 37 out of 47 (79%), for LCI5% was 34 out of 47 (72%), for M2/M0 was 34 out of 47 (72%), for Scond was 36 out of 46 (78%) and for SIII-DTG was 12 out of 35 (34%). Mean±sd duration of measurement was 19.8±11.2 min for LCI2.5%, 10.8±4.6 min for LCI5% and 8.6±2.3 min for Scond.Compared to standard LCI2.5%, ventilation inhomogeneity was detected by LCI5%, moment ratio and Scond with comparable sensitivity while measurement duration was significantly shorter. Longitudinal studies will show which outcome is most suitable and practical in terms of sensitivity, duration and variability within the course of primary ciliary dyskinesia lung disease.

Single breath washout as alternative to multiple breath washout in patients with primary ciliary dyskinesia (PCD)?

2015 ◽  
Author(s):  
Sylvia Nyilas ◽  
Anne Schlegtendal ◽  
Sophie Yammine ◽  
Carmen Casaulta ◽  
Philipp Latzin ◽  
...  
Keyword(s):  
Primary Ciliary Dyskinesia ◽  
Single Breath ◽  
Multiple Breath Washout ◽  
Ciliary Dyskinesia

scholarly journals Growth and nutritional status, and their association with lung function: a study from the international Primary Ciliary Dyskinesia Cohort

2017 ◽  
Vol 50 (6) ◽  
pp. 1701659 ◽  
Author(s):  
Myrofora Goutaki ◽  
Florian S. Halbeisen ◽  
Ben D. Spycher ◽  
Elisabeth Maurer ◽  
Fabiën Belle ◽  
...  
Keyword(s):  
Lung Function ◽  
Primary Ciliary Dyskinesia ◽  
Repeated Measurements ◽  
World Health ◽  
Z Score ◽  
Multilevel Regression ◽  
Growth And Nutrition ◽  
Diagnostic Certainty ◽  
Z Scores ◽  
Ciliary Dyskinesia

Chronic respiratory disease can affect growth and nutrition, which can influence lung function. We investigated height, body mass index (BMI), and lung function in patients with primary ciliary dyskinesia (PCD).In this study, based on the international PCD (iPCD) Cohort, we calculated z-scores for height and BMI using World Health Organization (WHO) and national growth references, and assessed associations with age, sex, country, diagnostic certainty, age at diagnosis, organ laterality and lung function in multilevel regression models that accounted for repeated measurements.We analysed 6402 measurements from 1609 iPCD Cohort patients. Height was reduced compared to WHO (z-score −0.12, 95% CI −0.17 to −0.06) and national references (z-score −0.27, 95% CI −0.33 to −0.21) in male and female patients in all age groups, with variation between countries. Height and BMI were higher in patients diagnosed earlier in life (p=0.026 and p<0.001, respectively) and closely associated with forced expiratory volume in 1 s and forced vital capacity z-scores (p<0.001).Our study indicates that both growth and nutrition are affected adversely in PCD patients from early life and are both strongly associated with lung function. If supported by longitudinal studies, these findings suggest that early diagnosis with multidisciplinary management and nutritional advice could improve growth and delay disease progression and lung function impairment in PCD.

Small airways disease in primary ciliary dyskinesia

2013 ◽  
Vol 14 ◽  
pp. S50-S51
Author(s):  
M. Boon ◽  
M. Proesmans ◽  
F. Vermeulen ◽  
K. De Boeck
Keyword(s):  
Primary Ciliary Dyskinesia ◽  
Small Airways ◽  
Ciliary Dyskinesia

scholarly journals Prevalence and course of disease after lung resection in primary ciliary dyskinesia: a cohort & nested case-control study

2019 ◽  
Vol 20 (1) ◽  
Author(s):  
Panayiotis Kouis ◽  
◽  
Myrofora Goutaki ◽  
Florian S. Halbeisen ◽  
Ifigeneia Gioti ◽  
...  
Keyword(s):  
Lung Function ◽  
Primary Ciliary Dyskinesia ◽  
Statistical Significance ◽  
Lung Resection ◽  
Delayed Diagnosis ◽  
Therapeutic Modality ◽  
Z Score ◽  
Situs Solitus ◽  
Z Scores ◽  
Ciliary Dyskinesia

Abstract Background Lung resection is a controversial and understudied therapeutic modality in Primary Ciliary Dyskinesia (PCD). We assessed the prevalence of lung resection in PCD across countries and compared disease course in lobectomised and non-lobectomised patients. Methods In the international iPCD cohort, we identified lobectomised and non-lobectomised age and sex-matched PCD patients and compared their characteristics, lung function and BMI cross-sectionally and longitudinally. Results Among 2896 patients in the iPCD cohort, 163 from 20 centers (15 countries) underwent lung resection (5.6%). Among adult patients, prevalence of lung resection was 8.9%, demonstrating wide variation among countries. Compared to the rest of the iPCD cohort, lobectomised patients were more often females, older at diagnosis, and more often had situs solitus. In about half of the cases (45.6%) lung resection was performed before presentation to specialized PCD centers for diagnostic work-up. Compared to controls (n = 197), lobectomised patients had lower FVC z-scores (− 2.41 vs − 1.35, p = 0.0001) and FEV1 z-scores (− 2.79 vs − 1.99, p = 0.003) at their first post-lung resection assessment. After surgery, lung function continued to decline at a faster rate in lobectomised patients compared to controls (FVC z-score slope: − 0.037/year Vs − 0.009/year, p = 0.047 and FEV1 z-score slope: − 0.052/year Vs − 0.033/year, p = 0.235), although difference did not reach statistical significance for FEV1. Within cases, females and patients with multiple lobe resections had lower lung function. Conclusions Prevalence of lung resection in PCD varies widely between countries, is often performed before PCD diagnosis and overall is more frequent in patients with delayed diagnosis. After lung resection, compared to controls most lobectomised patients have poorer and continuing decline of lung function despite lung resection. Further studies benefiting from prospective data collection are needed to confirm these findings.

scholarly journals Comparison of Multiple Breath Washout and Spirometry in Children with Primary Ciliary Dyskinesia and Cystic Fibrosis and Healthy Controls

2020 ◽  
Vol 17 (9) ◽  
pp. 1085-1093 ◽  
Author(s):  
BreAnna Kinghorn ◽  
Sharon McNamara ◽  
Alan Genatossio ◽  
Erin Sullivan ◽  
Molly Siegel ◽  
...  
Keyword(s):  
Cystic Fibrosis ◽  
Primary Ciliary Dyskinesia ◽  
Healthy Controls ◽  
Multiple Breath Washout ◽  
Ciliary Dyskinesia

scholarly journals Ventilation inhomogeneity in children with primary ciliary dyskinesia

Thorax ◽  
2011 ◽  
Vol 67 (1) ◽  
pp. 49-53 ◽  
Author(s):  
Kent Green ◽  
Frederik F Buchvald ◽  
June Kehlet Marthin ◽  
Birgitte Hanel ◽  
Per M Gustafsson ◽  
...  
Keyword(s):  
Primary Ciliary Dyskinesia ◽  
Ventilation Inhomogeneity ◽  
Ciliary Dyskinesia

scholarly journals Lung function from school age to adulthood in primary ciliary dyskinesia

2021 ◽  
Author(s):  
Florian S Halbeisen ◽  
Myrofora Goutaki ◽  
Eva Sophie L Pedersen ◽  
Ben D Spycher ◽  
Israel Amirav ◽  
...  
Keyword(s):  
Lung Function ◽  
Primary Ciliary Dyskinesia ◽  
Forced Expiratory Volume ◽  
Linear Regression Models ◽  
Adult Age ◽  
Diagnostic Certainty ◽  
Z Scores ◽  
Low Bmi ◽  
Ciliary Dyskinesia ◽  
Over Time

Abstract Primary ciliary dyskinesia (PCD) presents with symptoms early in life and the disease course may be progressive, but longitudinal data on lung function are scarce. This multinational cohort study describes lung function trajectories in children, adolescents, and young adults with PCD. We analysed data from 486 patients with repeated lung function measurements obtained between the age of 6 and 24 years from the international PCD Cohort (iPCD) and calculated z-scores for forced expiratory volume in the first second (FEV1) and forced vital capacity (FVC) using the Global Lung Function Initiative 2012 references. We described baseline lung function and change of lung function over time and described their associations with possible determinants in mixed-effects linear regression models. Overall, FEV1 and FVC z-scores declined over time (average crude annual FEV1 decline was -0.07 z-scores) but not at the same rate for all patients. FEV1 z-scores improved over time in 21% of patients, remained stable in 40% and declined in 39%. Low BMI was associated with poor baseline lung function and with further decline. Results differed by country and ultrastructural defect, but we found no evidence of differences by sex, calendar year of diagnosis, age at diagnosis, diagnostic certainty, or laterality defect. Our study shows that on average lung function in PCD declines throughout the entire period of lung growth, from childhood to young adult age, even among patients treated in specialized centres. It is essential to develop strategies to reverse this tendency and improve prognosis.

scholarly journals Ventilation distribution during histamine provocation

1997 ◽  
Vol 83 (6) ◽  
pp. 1907-1916 ◽  
Author(s):  
S. Verbanck ◽  
D. Schuermans ◽  
A. Van Muylem ◽  
M. Paiva ◽  
M. Noppen ◽  
...  
Keyword(s):  
Forced Expiratory Volume ◽  
Small Airways ◽  
Ventilation Distribution ◽  
Diffusion Front ◽  
Ventilation Inhomogeneity ◽  
Airway Caliber ◽  
Nonresponder Group ◽  
Multiple Breath Washout ◽  
Significant Change

Verbanck, S., D. Schuermans, A. Van Muylem, M. Paiva, M. Noppen, and W. Vincken. Ventilation distribution during histamine provocation. J. Appl. Physiol. 83(6): 1907–1916, 1997.—We investigated ventilation inhomogeneity during provocation with inhaled histamine in 20 asymptomatic nonsmoking subjects. We used N2multiple-breath washout (MBW) to derive parameters S condand S acin as a measurement of ventilation inhomogeneity in conductive and acinar zones of the lungs, respectively. A 20% decrease of forced expiratory volume in 1 s (FEV1) was used to distinguish responders from nonresponders. In the responder group, average FEV1 decreased by 26%, whereas S condincreased by 390% with no significant change in S acin. In the nonresponder group, FEV1 decreased by 11%, whereas S cond increased by 198% with no significant S acin change. Despite the absence of change in S acin during provocation, baseline S acin was significantly larger in the responder vs. the nonresponder group. The main findings of our study are that during provocation large ventilation inhomogeneities occur, that the small airways affected by the provocation process are situated proximal to the acinar zone where the diffusion front stands, and that, in addition to overall decrease in airway caliber, there is inhomogeneous narrowing of parallel airways.

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