ArticlePediatric pulmonology2026
Early Life Disease Burden and Outcomes in Children Diagnosed With Primary Ciliary Dyskinesia in Infancy.
Article in Pediatric pulmonology, 2026. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 3 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.
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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.
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Who cites it
3 citing papers in PubMed.
- Pulmonary exacerbations in primary ciliary dyskinesia.European journal of pediatrics · 2026Review
- Airway Clearance and Pediatric Pulmonary Rehabilitation in Primary Ciliary Dyskinesia: A Clinical Framework for Children and Adolescents.Journal of clinical medicine · 2026Review
- Early Life Disease Burden and Outcomes in Children Diagnosed With Primary Ciliary Dyskinesia in Infancy.Pediatric pulmonology · 2026Article
Corrections and comments
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Authors and funding
8 authors.
Funding
No grant is acknowledged in the PubMed record.
Abstract
backgroundPrimary ciliary dyskinesia (PCD) is a genetically heterogeneous disorder causing chronic oto-sino-pulmonary disease from birth. Since diagnosis is often delayed into childhood or adulthood, early-life disease burdens remain poorly described.
methodsThis retrospective study analyzed disease burdens until 2 years of age and outcomes at 6-7 years of age in children diagnosed with PCD near birth at two Canadian PCD centers.
resultsThirty-five infants (median diagnostic age 34 days) were included. All received daily chest physiotherapy, while 52% received daily inhaled hypertonic saline therapy (IHS) from diagnosis. By 2 years of age, 66% experienced outpatient respiratory exacerbations, 29% had inpatient respiratory exacerbations, 60% required emergency room visits for respiratory illness, and 14% had Pseudomonas aeruginosa in sputum cultures. Abnormal audiology occurred in 91% screened, and 49% required tympanostomy tubes. Despite early diagnosis and therapy, spirometry showed a trend towards obstruction (mean FEV₁/FVC ratio Z-score -1.43) by 7 years of age. Infants on daily IHS from birth (n = 18, mostly at one center) had significantly fewer Pseudomonas aeruginosa sputum isolates (0% vs. 29%, p = 0.02) but significantly increased inpatient respiratory exacerbations (44% vs. 12%, p = 0.04) compared to infants not on regular IHS (n = 17).
conclusionDespite early diagnosis, ongoing respiratory surveillance, and regular airway clearance, infants with PCD develop frequent respiratory infections and have high clinical care burdens. Infants have markedly increased risk of hearing loss, warranting universal screening and close follow-up. Inhaled hypertonic saline may influence early Pseudomonas acquisition and hospitalization risk, warranting prospective study of this commonly prescribed therapy.
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