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 ◽  
...  

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.

2019 ◽  
Vol 5 (2) ◽  
pp. 00231-2018 ◽  
Author(s):  
Florian S. Halbeisen ◽  
Anu Jose ◽  
Carmen de Jong ◽  
Sylvia Nyilas ◽  
Philipp Latzin ◽  
...  

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.


2018 ◽  
Author(s):  
Florian S. Halbeisen ◽  
Anu Jose ◽  
Carmen de Jong ◽  
Sylvia Nyilas ◽  
Philipp Latzin ◽  
...  

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.


BMC Surgery ◽  
2021 ◽  
Vol 21 (1) ◽  
Author(s):  
Stefano Granieri ◽  
Francesco Sessa ◽  
Alessandro Bonomi ◽  
Sissi Paleino ◽  
Federica Bruno ◽  
...  

Abstract Background Entero-colovesical fistula is a rare complication of various benign and malignant diseases. The diagnosis is prominently based on clinical symptoms; imaging studies are necessary not only to confirm the presence of the fistula, but more importantly to demonstrate the extent and the nature of the fistula. There is still a lack of consensus regarding the if, when and how to repair the fistula. The aim of the study is to review the different surgical treatment options, focus on surgical indications, and explore cumulative recurrence, morbidity, and mortality rates of entero-vesical and colo-vesical fistula patients. Methods A systematic review of the literature was conducted according to PRISMA guidelines. Random effects meta-analyses of proportions were developed to assess primary and secondary endpoints. I2 statistic and Cochran’s Q test were computed to assess inter-studies’ heterogeneity. Results Twenty-two studies were included in the analysis with a total of 861 patients. Meta-analyses of proportions pointed out 5, 22.2, and 4.9% rates for recurrence, complications, and mortality respectively. A single-stage procedure was performed in 75.5% of the cases, whereas a multi-stage operation in 15.5% of patients. Palliative surgery was performed in 6.2% of the cases. In 2.3% of the cases, the surgical procedure was not specified. Simple and advanced repair of the bladder was performed in 84.3% and 15.6% of the cases respectively. Conclusions Although burdened by a non-negligible rate of complications, surgical repair of entero-colovesical fistula leads to excellent results in terms of primary healing. Our review offers opportunities for significant further research in this field. Level of Evidence Level III according to ELIS (SR/MA with up to two negative criteria).


2019 ◽  
Vol 47 (08) ◽  
pp. 1659-1674 ◽  
Author(s):  
Mei-Hua Wang ◽  
Chiehfeng Chen ◽  
Mei-Ling Yeh ◽  
Jaung-Geng Lin

Studies have demonstrated the effect of acupoint-based interventions in relieving the clinical symptoms of asthma. However, the effect of meridian-based interventions in asthma symptom relief is unknown. This systematic review and meta-analysis determined the effect of multiplex meridian interventions in asthma symptom relief. Eight electronic databases were searched for relevant randomized controlled trials (RCTs) that involved patients with asthma, were published before March 2018, used acupoint stimulation interventions targeting acupoints that correspond to meridians, and considered asthma symptom relief as an outcome. In 204 RCTs that were identified and used in a meta-synthesis, meridians were used 521 times, with the bladder meridian being the most frequently used. Furthermore, 23 RCTs were included in the meta-analysis. Egger’s and inconsistency tests revealed no significant differences among the studies ([Formula: see text]). However, the interventions differed significantly in terms of asthma symptom relief effect, as demonstrated by pairwise (odds ratio [Formula: see text], 95% confidence [Formula: see text]–0.37) and network ([Formula: see text], 95% credibility [Formula: see text]–0.41) meta-analyses. Surface under the cumulative ranking (SUCRA) revealed that the bladder–conception vessel–governor vessel–stomach multiplex meridian intervention was more effective than non-meridian interventions in relieving asthma symptoms. Additionally, either bladder–conception vessel–stomach or bladder–conception vessel–governor vessel–kidney multiplex meridians may be selected in interventions. This study suggests that practitioners target multiplex meridians, especially the meridians of the bladder and conception vessel, to effectively relieve asthma symptoms.


2019 ◽  
Vol 19 (1) ◽  
Author(s):  
Atsushi Inaba ◽  
Masanori Furuhata ◽  
Kozo Morimoto ◽  
Mahbubur Rahman ◽  
Osamu Takahashi ◽  
...  

2017 ◽  
Vol 145 (11) ◽  
pp. 2241-2253 ◽  
Author(s):  
A. AWOFISAYO-OKUYELU ◽  
I. HALL ◽  
G. ADAK ◽  
J.I. HAWKER ◽  
S. ABBOTT ◽  
...  

AbstractAccurate knowledge of pathogen incubation period is essential to inform public health policies and implement interventions that contribute to the reduction of burden of disease. The incubation period distribution of campylobacteriosis is currently unknown with several sources reporting different times. Variation in the distribution could be expected due to host, transmission vehicle, and organism characteristics, however, the extent of this variation and influencing factors are unclear. The authors have undertaken a systematic review of published literature of outbreak studies with well-defined point source exposures and human experimental studies to estimate the distribution of incubation period and also identify and explain the variation in the distribution between studies. We tested for heterogeneity using I2 and Kolmogorov–Smirnov tests, regressed incubation period against possible explanatory factors, and used hierarchical clustering analysis to define subgroups of studies without evidence of heterogeneity. The mean incubation period of subgroups ranged from 2·5 to 4·3 days. We observed variation in the distribution of incubation period between studies that was not due to chance. A significant association between the mean incubation period and age distribution was observed with outbreaks involving only children reporting an incubation of 1·29 days longer when compared with outbreaks involving other age groups.


10.2196/18644 ◽  
2020 ◽  
Vol 8 (3) ◽  
pp. e18644 ◽  
Author(s):  
Julie F Vermeir ◽  
Melanie J White ◽  
Daniel Johnson ◽  
Geert Crombez ◽  
Dimitri M L Van Ryckeghem

Background There has been a growing interest in the application of gamification (ie, the use of game elements) to computerized cognitive training. The introduction of targeted gamification features to such tasks may increase motivation and engagement as well as improve intervention effects. However, it is possible that game elements can also have adverse effects on cognitive training (eg, be a distraction), which can outweigh their potential motivational benefits. So far, little is known about the effectiveness of such applications. Objective This study aims to conduct a systematic review and meta-analysis to investigate the effect of gamification on process outcomes (eg, motivation) and on changes in the training domain (eg, cognition), as well as to explore the role of potential moderators. Methods We searched PsycINFO, Cumulative Index to Nursing and Allied Health Literature, ProQuest Psychology, Web of Science, Scopus, PubMed, Science Direct, Excerpta Medica dataBASE, Institute of Electrical and Electronics Engineers Xplore, Association for Computing Machinery, and a range of gray-area literature databases. The searches included papers published between 2008 and 2018. Meta-analyses were performed using a random-effects model. Results The systematic review identified 49 studies, of which 9 randomized controlled trials were included in the meta-analysis. The results of the review indicated that research in this context is still developing and lacks well-controlled empirical studies. Gamification in cognitive training is applied to a large range of age groups and audiences and is mostly delivered at a research site through computers. Rewards and feedback continue to dominate the gamification landscape, whereas social-oriented features (eg, competition) are underused. The meta-analyses showed that gamified training tasks were more motivating/engaging (Hedges g=0.72) and more demanding/difficult (Hedges g=–0.52) than non- or less-gamified tasks, whereas no effects on the training domain were found. Furthermore, no variables moderated the impact of gamified training tasks. However, meta-analytic findings were limited due to a small number of studies. Conclusions Overall, this review provides an overview of the existing research in the domain and provides evidence for the effectiveness of gamification in improving motivation/engagement in the context of cognitive training. We discuss the shortcomings in the current literature and provide recommendations for future research.


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