Clinical features of primary ciliary dyskinesia in Cyprus with emphasis on lobectomized patients

Yiallouros, Panayiotis K.; Kouis, Panayiotis; Middleton, Nicos; Nearchou, Marianna; Adamidi, Tonia; Georgiou, Andreas; Eleftheriou, Adonis; Ioannou, Phivos; Hadjisavvas, Andreas; Kyriacou, Kyriacos

doi:10.1016/j.rmed.2015.01.015

Cited by 35 publications

(31 citation statements)

References 23 publications

(27 reference statements)

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“…We found that females had a poorer lung function than males (FEV1 z-scores females −1.57, males −1.36). Although other studies have reported similar sex differences in PCD [9,27] and CF [28], the processes that affect lung function particularly in females are not well understood. It could reflect treatment variation, differences in therapy adherence or anatomical, hormonal or cultural causes.…”

Section: Discussionmentioning

confidence: 93%

“…Studies have reported contradictory results about associations between age at diagnosis and lung function of PCD patients. Some studies reported that regular treatment can maintain lung function [25,38,39], and early diagnosis may prevent progression of lung disease [27]. However, other studies reported no association between age at diagnosis and decline in lung function [10,11].…”

Section: Discussionmentioning

confidence: 99%

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Lung function in patients with primary ciliary dyskinesia: an iPCD Cohort study

et al. 2018

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Primary ciliary dyskinesia (PCD) has been considered a relatively mild disease, especially compared to cystic fibrosis (CF), but studies on lung function in PCD patients have been few and small.This study compared lung function from spirometry of PCD patients to normal reference values and to published data from CF patients. We calculated z-scores and % predicted values for forced expiratory volume in 1 s (FEV) and forced vital capacity (FVC) using the Global Lung Function Initiative 2012 values for 991 patients from the international PCD Cohort. We then assessed associations with age, sex, country, diagnostic certainty, organ laterality, body mass index and age at diagnosis in linear regression models. Lung function in PCD patients was reduced compared to reference values in both sexes and all age groups. Children aged 6-9 years had the smallest impairment (FEV z-score -0.84 (-1.03 to -0.65), FVC z-score -0.31 (-0.51 to -0.11)). Compared to CF patients, FEV was similarly reduced in children (age 6-9 years PCD 91% (88-93%); CF 90% (88-91%)), but less impaired in young adults (age 18-21 years PCD 79% (76-82%); CF 66% (65-68%)). The results suggest that PCD affects lung function from early in life, which emphasises the importance of early standardised care for all patients.

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Section: Discussionmentioning

confidence: 93%

Section: Discussionmentioning

confidence: 99%

Lung function in patients with primary ciliary dyskinesia: an iPCD Cohort study

et al. 2018

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“…Table 1 lists the 24 studies and their characteristics. The articles [4,51–73] encompassed a total of 1179 patients with a mean number of 49 patients (range 13–158) per study. Most studies were relatively small, with 18 of the 24 including fewer than 50 patients (Table 2).…”

Section: Resultsmentioning

confidence: 99%

“…Preliminary results from 71 patients included in the international PCD registry showed an annual mean decline of 0.59% in percent predicted FEV1 and of 0.39% in percent predicted FVC [40]. Recent, smaller studies have evaluated the effect of managing lung function of PCD patients with, for example, lobectomy [73] and endoscopic sinus surgery [18] without observing significant differences in lung function decline.…”

Section: Resultsmentioning

confidence: 99%

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

Halbeisen

Jose

Jong

et al. 2018

Preprint

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Take home message: Spirometric indices of PCD patients vary between published studies, which not only suggests the possibility of methodological differences between centres, but also real differences in disease expression based on genotype-phenotype associations. 2 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 (I 2 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.

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“…Respiratorische Infekte sollten konsequent antibiotisch behandelt werden [3,21] der Krankheitsverlauf nicht aufgehalten wird und die Lungenfunktion stärker beeinträchtigt wird, sollte dies nur in seltenen Ausnahmefällen erfolgen [24]. Eine Lungentransplantation ist bei terminalem chronischem Lungenversagen eine Therapieoption.…”

Section: » Pcd-patienten Sollten Dem Internationalen Pcdpatientenregiunclassified

Primäre ziliäre Dyskinesie

Werner

Omran

2015

Pneumologe

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Clinical features of primary ciliary dyskinesia in Cyprus with emphasis on lobectomized patients

Abstract: PCD often remains undiagnosed up to adulthood accompanied by appearance of advanced lung disease. Performance of lobectomies seems to be a poor prognostic factor for PCD in adulthood.

Cited by 35 publications

References 23 publications

Lung function in patients with primary ciliary dyskinesia: an iPCD Cohort study

Lung function in patients with primary ciliary dyskinesia: an iPCD Cohort study

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

Primäre ziliäre Dyskinesie

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