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Practice changer · 05 of 05

Nasal nitric oxide with clinical features identified primary ciliary dyskinesia in 7% of bronchiectasis patients

Consider nasal nitric oxide screening for primary ciliary dyskinesia in bronchiectasis, especially with early onset, rhinosinusitis, Pseudomonas or situs inversus.

Design
Prospective cross-sectional diagnostic study within a bronchiectasis registry
Population
373 adolescents and adults with bronchiectasis in Shanghai
Primary outcome
PCD diagnosis by ERS/ATS 2025 criteria and nasal nitric oxide accuracy
Effect
PCD in 7.4%; nNO sensitivity 84.8%, specificity 97.1%

Patients with bronchiectasis in a Shanghai registry had nasal nitric oxide measured. Those with low levels or strong clinical suspicion underwent electron microscopy and exome sequencing.

About one in 14 had confirmed or highly likely primary ciliary dyskinesia. Nasal nitric oxide was an accurate first step, especially combined with clinical clues. Patients with PCD had lower lung function and more Pseudomonas.

Nasal nitric oxide testing is available in few Indian centres, but clinical features alone can prompt referral. A PCD diagnosis matters for airway clearance, fertility counselling and family screening.

  • Nasal nitric oxide at or below 77 nL/min had 85% sensitivity and 97% specificity.
  • Patients with PCD were younger and had earlier-onset bronchiectasis.
  • Rhinosinusitis was strongly associated (OR 25.3).
  • Pseudomonas infection was more common (OR 8.5).
  • About a quarter had situs inversus; DNAH11 was the commonest gene.

Why it matters

PCD is under-diagnosed in adult bronchiectasis, and those affected had worse lung function and more Pseudomonas.

Don't overread it

This was one Chinese centre, and only patients flagged by low nitric oxide or strong suspicion had full testing, so some cases may have been missed.

The statistics, in plain English

Sensitivity of 85% means about one in seven PCD cases would have a normal nitric oxide reading. Because full testing was not done on everyone, the true prevalence could be slightly higher.

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