scholarly journals Spirometric indices in primary ciliary dyskinesia: systematic review and meta-analysis

2018 ◽  
Author(s):  
Florian S. Halbeisen ◽  
Anu Jose ◽  
Carmen de Jong ◽  
Sylvia Nyilas ◽  
Philipp Latzin ◽  
...  
Keyword(s):  
Systematic Review ◽  
Lung Function ◽  
Primary Ciliary Dyskinesia ◽  
Meta Analysis ◽  
Forced Expiratory Volume ◽  
Chronic Respiratory Diseases ◽  
Explanatory Factors ◽  
Performance And Evaluation ◽  
Meta Analyses ◽  
Ciliary Dyskinesia

AbstractPrimary ciliary dyskinesia (PCD) is a genetic, heterogeneous disease caused by dysfunction of cilia. Evidence is sparse and reports of lung function of PCD patients ranges from normal to severe impairment. This systematic review and meta-analysis of studies of lung function of PCD patients examines the spirometric indices of PCD patients and differences by age group and sex.We searched PubMed, Embase, and Scopus for studies that described lung function in ≥10 patients with PCD. We performed meta-analyses and metaregression to explain heterogeneity. We included 24 studies, ranging from 13-158 patients per study. The most commonly reported spirometric indices were forced expiratory volume in 1 sec (FEV1) and forced vital capacity (FVC) presented as mean and standard deviation of percent of predicted values. We found considerable heterogeneity for both parameters (I2 range 94-96%). The heterogeneity remained when we stratified the analysis by age; however, FEV1 in adult patients was lower. Even after taking into account explanatory factors, the largest part of the between-studies variance remained unexplained. Heterogeneity could be explained by genetic differences between study populations, methodological factors related to the variability of study inclusion criteria, or details on the performance and evaluation of lung function measurements that we could not account for. Prospective studies therefore need to use standardised protocols and international reference values. These results underline the possibility of distinct PCD phenotypes as in other chronic respiratory diseases. Detailed characterisation of these phenotypes and related genotypes is needed in order to better understand the natural history of PCD.

scholarly journals Spirometric indices in primary ciliary dyskinesia: systematic review and meta-analysis

2019 ◽  
Vol 5 (2) ◽  
pp. 00231-2018 ◽  
Author(s):  
Florian S. Halbeisen ◽  
Anu Jose ◽  
Carmen de Jong ◽  
Sylvia Nyilas ◽  
Philipp Latzin ◽  
...  
Keyword(s):  
Systematic Review ◽  
Lung Function ◽  
Primary Ciliary Dyskinesia ◽  
Meta Analysis ◽  
Forced Expiratory Volume ◽  
Chronic Respiratory Diseases ◽  
Explanatory Factors ◽  
Performance And Evaluation ◽  
Meta Analyses ◽  
Ciliary Dyskinesia

Primary ciliary dyskinesia (PCD) is a genetic, heterogeneous disease caused by dysfunction of cilia. Evidence is sparse and reports of lung function in PCD patients range from normal to severe impairment. This systematic review and meta-analysis of studies of lung function in PCD patients examines the spirometric indices of PCD patients and differences by age group and sex.We searched PubMed, Embase and Scopus for studies that described lung function in 10 or more patients with PCD. We performed meta-analyses and meta-regression to explain heterogeneity. We included 24 studies, ranging from 13 to 158 patients per study.The most commonly reported spirometric indices were forced expiratory volume in 1 s (FEV1) and forced vital capacity presented as mean and standard deviation of percent predicted values. We found considerable heterogeneity for both parameters (I2=94–96%). The heterogeneity remained when we stratified the analysis by age; however, FEV1 in adult patients was lower. Even after taking into account explanatory factors, the largest part of the between-studies variance remained unexplained.Heterogeneity could be explained by genetic differences between study populations, methodological factors related to the variability of study inclusion criteria or details on the performance and evaluation of lung function measurements that we could not account for. Prospective studies therefore need to use standardised protocols and international reference values. These results underline the possibility of distinct PCD phenotypes as in other chronic respiratory diseases. Detailed characterisation of these phenotypes and related genotypes is needed in order to better understand the natural history of PCD.

scholarly journals Clinical manifestations in primary ciliary dyskinesia: systematic review and meta-analysis

2016 ◽  
Vol 48 (4) ◽  
pp. 1081-1095 ◽  
Author(s):  
Myrofora Goutaki ◽  
Anna Bettina Meier ◽  
Florian S. Halbeisen ◽  
Jane S. Lucas ◽  
Sharon D. Dell ◽  
...  
Keyword(s):  
Systematic Review ◽  
Primary Ciliary Dyskinesia ◽  
Clinical Symptoms ◽  
Meta Analysis ◽  
Age Groups ◽  
Clinical Manifestations ◽  
Explanatory Factors ◽  
Symptom Prevalence ◽  
Meta Analyses ◽  
Ciliary Dyskinesia

Few original studies have described the prevalence and severity of clinical symptoms of primary ciliary dyskinesia (PCD). This systematic review and meta-analysis aimed to identify all published studies on clinical manifestations of PCD patients, and to describe their prevalence and severity stratified by age and sex.We searched PubMed, Embase and Scopus for studies describing clinical symptoms of ≥10 patients with PCD. We performed meta-analyses and meta-regression to explain heterogeneity.We included 52 studies describing a total of 1970 patients (range 10–168 per study). We found a prevalence of 5% for congenital heart disease. For the rest of reported characteristics, we found considerable heterogeneity (I2 range 68–93.8%) when calculating the weighted mean prevalence. Even after taking into account the explanatory factors, the largest part of the between-studies variance in symptom prevalence remained unexplained for all symptoms. Sensitivity analysis including only studies with test-proven diagnosis showed similar results in prevalence and heterogeneity.Large differences in study design, selection of study populations and definition of symptoms could explain the heterogeneity in symptom prevalence. To better characterise the disease, we need larger, multicentre, multidisciplinary, prospective studies that include all age groups, use uniform diagnostics and report on all symptoms.

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 Primary ciliary dyskinesia in Japan: systematic review and meta-analysis

2019 ◽  
Vol 19 (1) ◽  
Author(s):  
Atsushi Inaba ◽  
Masanori Furuhata ◽  
Kozo Morimoto ◽  
Mahbubur Rahman ◽  
Osamu Takahashi ◽  
...  
Keyword(s):  
Systematic Review ◽  
Primary Ciliary Dyskinesia ◽  
Meta Analysis ◽  
Ciliary Dyskinesia

scholarly journals The Effects of Fruit-Derived Polyphenols on Cognition and Lung Function in Healthy Adults: A Systematic Review and Meta-Analysis

Nutrients ◽  
2021 ◽  
Vol 13 (12) ◽  
pp. 4273
Author(s):  
Lillian Morton ◽  
Andrea J. Braakhuis
Keyword(s):  
Systematic Review ◽  
Lung Function ◽  
Healthy Subjects ◽  
Intervention Studies ◽  
Meta Analysis ◽  
Healthy Adults ◽  
Dietary Intakes ◽  
Lung Capacity ◽  
Intervention Trials ◽  
Meta Analyses

Polyphenols are plant derived nutrients that influence oxidative stress and inflammation and therefore may have positive benefits on cognition and lung function. This systematic review and meta-analysis aimed to evaluate the effects of fruit derived polyphenol intakes on cognition and lung capacity in healthy adults. In August 2020 and October 2021, Medline and Google Scholar were used to search for relevant studies examining the effects of fruit derived polyphenol intakes on cognition and/or lung function in healthy adults (<70 years old). Fourteen studies related to cognition (409 healthy subjects) and seven lung/respiratory studies (20 788 subjects) were used for the systematic review using the preferred reporting items for systematic reviews and meta-analyses (PRISMA) guidelines. The meta-analysis (using six cognition and three lung function studies) indicated a protective effect on lung function from dietary intakes of fruit-derived polyphenols. Neither a benefit nor decrement from fruit-derived polyphenol intakes were detected for cognition. Human intervention trials examining the effects of polyphenol supplementation on lung function in healthy adults are scarce and intervention studies are warranted. More conclusive results are needed to provide recommendations for polyphenol supplementation to support aspects of cognition.

Pesticide exposure and lung function: a systematic review and meta-analysis

2019 ◽  
Vol 70 (1) ◽  
pp. 14-23 ◽  
Author(s):  
J Ratanachina ◽  
S De Matteis ◽  
P Cullinan ◽  
P Burney
Keyword(s):  
Systematic Review ◽  
Lung Function ◽  
Exposure Assessment ◽  
Pesticide Exposure ◽  
Respiratory Health ◽  
Meta Analysis ◽  
Epidemiological Studies ◽  
Forced Expiratory Volume ◽  
Free Text ◽  
Mesh Terms

Abstract Background Epidemiological studies have reported associations between pesticide exposure and respiratory health effects, but the quantitative impact on lung function is unclear. To fill this gap, we undertook a systematic review of the available literature on the association between pesticide exposure and pulmonary function. Aims To examine all available literature regarding the relationship between occupational and environmental exposure to pesticides and lung function. Methods We searched MEDLINE, EMBASE and Web of Science databases to 1 October 2017 without any date or language restrictions using a combination of MeSH terms and free text for ‘pesticide exposure’ and ‘lung function’. We included studies that met the criteria of our research protocol registered in PROSPERO, and we assessed their quality using a modified Newcastle-Ottawa scale. Results Of 2356 articles retrieved, 56 articles were included in the systematic review and pooled in meta-analyses for forced expiratory volume in 1 s (FEV1)/forced vital capacity (FVC), FVC and FEV1. There was tentative evidence that exposure to cholinesterase (ChE) inhibiting pesticides reduced FEV1/FVC and no evidence that paraquat exposure affected lung function in farmers. Conclusions Respiratory surveillance should be enhanced in those exposed to ChE-inhibiting pesticides which reduced FEV1/FVC according to the meta-analysis. Our study is limited by heterogeneity between studies due to different types of exposure assessment to pesticides and potential confounders. Further studies with a more accurate exposure assessment are suggested.

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.

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