scholarly journals Recovery of baseline lung function after pulmonary exacerbation in children with primary ciliary dyskinesia

2016 ◽  
Vol 51 (12) ◽  
pp. 1362-1366 ◽  
Author(s):  
Meera Sunther ◽  
Andrew Bush ◽  
Claire Hogg ◽  
Lauren McCann ◽  
Siobhán B. Carr
Keyword(s):  
Lung Function ◽  
Primary Ciliary Dyskinesia ◽  
Pulmonary Exacerbation ◽  
Ciliary Dyskinesia

Lung clearance index predicts pulmonary exacerbations in individuals with primary ciliary dyskinesia: a multicentre cohort study

Thorax ◽  
2021 ◽  
pp. thoraxjnl-2020-215504
Author(s):  
Florian Singer ◽  
Anne Schlegtendal ◽  
Sylvia Nyilas ◽  
François Vermeulen ◽  
Mieke Boon ◽  
...  
Keyword(s):  
Cohort Study ◽  
Lung Function ◽  
Primary Ciliary Dyskinesia ◽  
Response To Treatment ◽  
Pulmonary Exacerbation ◽  
Lung Clearance ◽  
Lung Clearance Index ◽  
Pulmonary Exacerbations ◽  
The Impact ◽  
Ciliary Dyskinesia

BackgroundLung clearance index (LCI) is a promising lung function outcome in individuals with primary ciliary dyskinesia (PCD). The impact of events clinically important for individuals with PCD, such as pulmonary exacerbations, on LCI is unknown.MethodsWe conducted an international, multicentre, observational cohort study to assess the association of LCI and risk of pulmonary exacerbation, specific changes in LCI during pulmonary exacerbation and global variability of LCI across four visits every 4 months. Ninety individuals with PCD, aged 3–41 years, underwent nitrogen multiple-breath washout (MBW) and spirometry measurements. The association of LCI and pulmonary exacerbations was assessed by Cox proportional hazards and random-effects regression models.ResultsWe obtained 430 MBW and 427 spirometry measurements. In total, 379 person-years at risk contributed to the analysis. Per one unit increase (deterioration) in LCI, the risk of future pulmonary exacerbation increased by 13%: HR (95% CI), 1.13 (1.04 to 1.23). If LCI changed from a range of values considered normal to abnormal, the risk of future pulmonary exacerbations increased by 87%: 1.87 (1.08 to 3.23). During pulmonary exacerbations, LCI increased by 1.22 units (14.5%). After pulmonary exacerbations, LCI tended to decline. Estimates of variability in LCI suggested lower variation within individuals compared with variation between individuals. Findings were comparable for forced expiratory volume in 1 s.ConclusionOn a visit-to-visit basis, LCI measurement may add to the prediction of pulmonary exacerbations, the assessment of lung function decline and the potential lung function response to treatment of pulmonary exacerbations.

scholarly journals Clinical features and management of children with primary ciliary dyskinesia in England

2020 ◽  
Vol 105 (8) ◽  
pp. 724-729 ◽  
Author(s):  
Bruna Rubbo ◽  
Sunayna Best ◽  
Robert Anthony Hirst ◽  
Amelia Shoemark ◽  
Patricia Goggin ◽  
...  
Keyword(s):  
Lung Function ◽  
Hearing Impairment ◽  
Hearing Aids ◽  
Primary Ciliary Dyskinesia ◽  
Service Evaluation ◽  
Forced Expiratory Volume ◽  
Management Service ◽  
National Data ◽  
Randomised Controlled ◽  
Ciliary Dyskinesia

ObjectiveIn England, the National Health Service commissioned a National Management Service for children with primary ciliary dyskinesia (PCD). The aims of this study were to describe the health of children seen in this Service and compare lung function to children with cystic fibrosis (CF).DesignMulti-centre service evaluation of the English National Management PCD Service.SettingFour nationally commissioned PCD centres in England.Patients333 children with PCD reviewed in the Service in 2015; lung function data were also compared with 2970 children with CF.ResultsMedian age at diagnosis for PCD was 2.6 years, significantly lower in children with situs inversus (1.0 vs 6.0 years, p<0.001). Compared with national data from the CF Registry, mean (SD) %predicted forced expiratory volume in one second (FEV1) was 76.8% in PCD (n=240) and 85.0% in CF, and FEV1 was lower in children with PCD up to the age of 15 years. Approximately half of children had some hearing impairment, with 26% requiring hearing aids. Children with a lower body mass index (BMI) had lower FEV1 (p<0.001). One-third of children had positive respiratory cultures at review, 54% of these grew Haemophilus influenzae.ConclusionsWe provide evidence that children with PCD in England have worse lung function than those with CF. Nutritional status should be considered in PCD management, as those with a lower BMI have significantly lower FEV1. Hearing impairment is common but seems to improve with age. Well-designed and powered randomised controlled trials on management of PCD are needed to inform best clinical practice.

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.

Sinus surgery can improve quality of life, lung infections, and lung function in patients with primary ciliary dyskinesia

2016 ◽  
Vol 7 (3) ◽  
pp. 240-247 ◽  
Author(s):  
Mikkel Christian Alanin ◽  
Kasper Aanaes ◽  
Niels Høiby ◽  
Tania Pressler ◽  
Marianne Skov ◽  
...  
Keyword(s):  
Quality Of Life ◽  
Lung Function ◽  
Primary Ciliary Dyskinesia ◽  
Sinus Surgery ◽  
Improve Quality ◽  
Lung Infections ◽  
Ciliary Dyskinesia

scholarly journals Longitudinal lung function and structural changes in children with primary ciliary dyskinesia

2012 ◽  
Vol 47 (8) ◽  
pp. 816-825 ◽  
Author(s):  
Marie Lémery Magnin ◽  
Pierrick Cros ◽  
Nicole Beydon ◽  
Malika Mahloul ◽  
Aline Tamalet ◽  
...  
Keyword(s):  
Lung Function ◽  
Primary Ciliary Dyskinesia ◽  
Structural Changes ◽  
Ciliary Dyskinesia

LATE-BREAKING ABSTRACT: Lung function in patients with primary ciliary dyskinesia (PCD): A multinational study

2015 ◽  
Author(s):  
Florian Halbeisen ◽  
Myrofora Goutaki ◽  
Elisabeth Maurer ◽  
Mieke Boon ◽  
Carmen Casaulta ◽  
...  
Keyword(s):  
Lung Function ◽  
Primary Ciliary Dyskinesia ◽  
Multinational Study ◽  
Ciliary Dyskinesia

Lung function and nutritional status in children with cystic fibrosis and primary ciliary dyskinesia

2016 ◽  
Author(s):  
Woolf Walker ◽  
Amanda Harris ◽  
Bruna Rubbo ◽  
Victoria Keenan ◽  
Amanda Friend ◽  
...  
Keyword(s):  
Cystic Fibrosis ◽  
Lung Function ◽  
Nutritional Status ◽  
Primary Ciliary Dyskinesia ◽  
Ciliary Dyskinesia
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