SynthesisQuality of life research : an international journal of quality of life aspects of treatment, care and rehabilitation2017
The patient's experience of primary ciliary dyskinesia: a systematic review.
Synthesis in Quality of life research : an international journal of quality of life aspects of treatment, care and rehabilitation, 2017. The graph could read no effect estimate from its abstract, so it casts no vote on the map. It is linked to trial NCT04895150 (Investigation of Upper Extremity Exercise Capacity, Muscle Oxygenation, Balance and Physical Activity Levels in Patients With Primary Ciliary Dyskinesia), which is not on this map. Cited by 38 papers.
What it found
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The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.
The trial behind it
Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.
Investigation of Upper Extremity Exercise Capacity, Muscle Oxygenation, Balance and Physical Activity Levels in Patients With Primary Ciliary Dyskinesia
Who cites it
38 citing papers in PubMed, 52 citations in OpenAlex.
- Active video gaming in primary ciliary dyskinesia: a randomized controlled trial.European journal of pediatrics · 2022Trial
- Pulmonary exacerbations in primary ciliary dyskinesia.European journal of pediatrics · 2026Review
- Mapping challenges in the delivery of care for primary ciliary dyskinesia: an international survey on barriers and priorities.ERJ open research · 2026Article
- Advances in Health-Related Quality of Life in Primary Ciliary Dyskinesia.Pediatric pulmonology · 2026Review
- Pulmonary Exacerbations and Treatment in PCD: A Narrative Review.Pediatric pulmonology · 2026Review
- The Relationship Between Structural and Functional Pulmonary Status and Quality of Life in Children With Primary Ciliary Dyskinesia.Pediatric pulmonology · 2026Article
- Patients' research priorities and participation in primary ciliary dyskinesia research.BMJ open respiratory research · 2025Article
- Evidence of inequities experienced by the rare disease community with respect to receipt of a diagnosis and access to services: a scoping review of UK and international evidence.Orphanet journal of rare diseases · 2025Article
- Acute Exposure to Aerosolized Nanoplastics Modulates Redox-Linked Immune Responses in Human Airway Epithelium.Antioxidants (Basel, Switzerland) · 2025Article
- Definition of sinonasal and otological exacerbation in patients with primary ciliary dyskinesia: an expert consensus.ERJ open research · 2024Article
- Priorities and barriers for research related to primary ciliary dyskinesia.ERJ open research · 2024Article
- 'Don't let it hold you back' - The experience of transition to adulthood in young people with primary ciliary dyskinesia: An interpretative phenomenological analysis.Journal of health psychology · 2024Article
- Clinical Characteristics and Immune Responses in Children with Primary Ciliary Dyskinesia during Pneumonia Episodes: A Case-Control Study.Children (Basel, Switzerland) · 2023Article
- Bronchiectasis Assessment in Primary Ciliary Dyskinesia: A Non-Invasive Approach Using Forced Oscillation Technique.Diagnostics (Basel, Switzerland) · 2023Article
- Outcomes of Endoscopic Sinus Surgery for Chronic Rhinosinusitis in Adults with Primary Ciliary Dyskinesia.International archives of otorhinolaryngology · 2023Article
- Observational
- Translation of the quality of life questionnaire for primary ciliary dyskinesia (QOL-PCD) into Hebrew: The Israeli experience.Pediatric pulmonology · 2022Article
- Primary ciliary dyskinesia in Volendam: Diagnostic and phenotypic features in patients with a CCDC114 mutation.American journal of medical genetics. Part C, Seminars in medical genetics · 2022Article
- Chronic airway disease in primary ciliary dyskinesia-spiced with geno-phenotype associations.American journal of medical genetics. Part C, Seminars in medical genetics · 2022Review
- Impact of Motile Ciliopathies on Human Development and Clinical Consequences in the Newborn.Cells · 2021Review
Corrections and comments
PubMed lists nothing against this paper. Absence here is not a guarantee, only a check that was made.
Authors and funding
4 authors at 2 institutions in 2 countries.
Funding
Abstract
backgroundPrimary ciliary dyskinesia (PCD) is a rare genetic disorder characterised by progressive sinopulmonary disease, with symptoms starting soon after birth. The aim of this study is to critically review, analyse, and synthesise the literature in order to understand the experiences of patients with primary ciliary dyskinesia (PCD) and the impact on health-related quality of life.
methodMEDLINE, EBSCO, Cumulative Index to Nursing and Allied Health Literature (CINAHL), PsycINFO and EMBASE were searched according to the inclusion criteria. A qualitative analysis of 14 studies was conducted.
resultsFourteen studies were included in the review, five with qualitative methodologies. Studies originated from the UK, USA, Italy, Denmark and Belgium, one study included a survey distributed internationally. Significant relationships were found between age and worsening of respiratory symptoms, physical, and mental domains of health-related quality of life, with a greater decline compared with reference populations. Variations between the UK and Italy were found for health-related quality of life and its correlation with time since diagnosis. PCD was found to have a physical impact in all age groups: patients found it difficult to keep up with others, and found energy levels were easily depleted compared to family or peers. In terms of social impact, symptoms lead to embarrassment and a sense of isolation, with patients concealing symptoms and/or their diagnosis. In turn, isolation was also linked with the lack of public and medical knowledge. In relation to emotional impact, anxiety was reported in a number of qualitative studies; patients were anxious about getting sick or when thinking about their future health. The burden of treatment and factors influencing adherence were also discussed in depth.
conclusionHealth-related quality of life decreases with age in patients with PCD. For all age groups, PCD was found to greatly impact physical, emotional, social functioning, and treatment burden. More research is needed on the psychosocial impact of the illness, disease burden and its effect on quality of life.
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Registered trials
Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.