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

Alanin, Mikkel Christian; Aanæs, Kasper; Høiby, Niels; Pressler, T.; Skov, Marianne; Nielsen, Kim G.; Johansen, Helle Krogh; Buchwald, Christian von

doi:10.1002/alr.21873

Cited by 55 publications

(53 citation statements)

References 39 publications

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“…Among the remaining 163 studies, 37 were either conference abstracts only or it was not possible to find the full text. After reading the full text of the remaining 126 studies, we excluded another 102 articles (Figure 1), including 34 that had overlapping study populations [18–50]. We finally included 24 studies reporting spirometric indices of PCD patients in the meta-analysis.…”

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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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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“…These manuscripts included one prospective single-arm trial [13], one retrospective cohort study [14], one cross-sectional study [7], two case series [15,16], and one case report [17]. The number of people included in each study ranged from one to 44.…”

Section: Resultsmentioning

confidence: 99%

“…A recent study developed a metric to assess health-related quality of life in adults with PCD [27], demonstrating the multi-dimensional effects of the disease, including treatment burden and its effects on social and emotion functioning. Endoscopic sinus surgery (ESS) has been shown to help improve quality of life, lung infections, and lung function in adults with PCD [13], and the performance of ESS demonstrated benefit to children with PCD in one study [28], but outcomes in both children and adults are poorly defined, with high variation from study to study. The rarity of the disease makes long-term prospective studies difficult to perform, highlighting the need for multicenter data sharing.…”

Section: Discussionmentioning

confidence: 99%

A Systematic Review of the Treatment of Chronic Rhinosinusitis in Adults with Primary Ciliary Dyskinesia

2017

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Abstract:Background: Primary ciliary dyskinesia (PCD) may be an underlying factor in some cases of refractory chronic rhinosinusitis (CRS). However, clinical management of this condition is not well defined. This systematic review examines the available evidence for the diagnosis and management of CRS in adults with PCD. Methods: A systematic review was conducted according to the Preferred Reporting Items for Systematic Reviews and Meta-Analysis (PRISMA) guidelines. Pubmed, EMBASE, and Cochrane database were queried for studies pertinent to treatment of PCD in adults. Two investigators performed eligibility assessment for inclusion or exclusion in a standardized manner. Results: Of the 278 articles identified, six studies met the criteria for analysis. These studies had a predominately low level of evidence. Medical therapy included oral antibiotics and nasal saline rinses. Endoscopic sinus surgery (ESS) was described in three of six studies. Outcomes measures were limited and included non-validated questionnaires, subjective reporting of CRS symptoms, and decreased preciptins against pseudomonas following ESS. Recommendation for a standardized therapeutic strategy was not possible with the available literature. Conclusion: A paucity of evidence is available to guide the treatment of PCD in the adult population. Further prospective studies are needed to determine the optimal diagnostic and management strategy for this condition.

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“…Similar to CF, the sinuses might act as a bacterial reservoir in PCD [73] and therefore bacterial eradication through endoscopic sinus surgery might prove beneficial. ALANIN et al [74] reported a significant improvement in health-related QoL SNOT-22 score after endoscopic sinus surgery and adjuvant treatment (i.e. saline nasal rinsing, topical steroids and 2 weeks of antibiotic treatment).…”

Section: Upper Airway Managementmentioning

confidence: 99%

Clinical care for primary ciliary dyskinesia: current challenges and future directions

Rubbo

Lucas

2017

Eur Respir Rev

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Primary ciliary dyskinesia (PCD) is a rare genetic disease that affects the motility of cilia, leading to impaired mucociliary clearance. It is estimated that the vast majority of patients with PCD have not been diagnosed as such, providing a major obstacle to delivering appropriate care. Challenges in diagnosing PCD include lack of disease-specific symptoms and absence of a single, "gold standard", diagnostic test. Management of patients is currently not based on high-level evidence because research findings are mostly derived from small observational studies with limited follow-up period. In this review, we provide a critical overview of the available literature on clinical care for PCD patients, including recent advances. We identify barriers to PCD research and make suggestions for overcoming challenges.

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

Abstract: We demonstrated an improvement in CRS-related symptoms after ESS and adjuvant therapy. In selected PCD patients, the suggested regimen may postpone chronic lung infection with P. aeruginosa and stabilize lung function.

Cited by 55 publications

References 39 publications

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

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

A Systematic Review of the Treatment of Chronic Rhinosinusitis in Adults with Primary Ciliary Dyskinesia

Clinical care for primary ciliary dyskinesia: current challenges and future directions

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