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Folio edition · Set in Instrument Serif & Archivo

Paeds Vivasrespiratory-sleep-and-airway

Paeds Vivas · respiratory-sleep-and-airway

Bronchiectasis in children — structured oral (viva)

Branching structured oral on a child with a chronic wet cough, testing the suppurative lung disease continuum, the vicious cycle, the aetiological work-up, and the management of childhood bronchiectasis.

branching clinical structured oral
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Target exams

RACP DCEMRCPCH ClinicalRCPSC Pediatrics

Target exams

RACP DCEMRCPCH ClinicalRCPSC Pediatrics
Prompt
A 7-year-old girl has a daily wet cough that has lasted five months and only ever partly settles with antibiotics. She had unexplained neonatal respiratory distress at term, has had recurrent glue ear and chronic nasal discharge since infancy, and has early digital clubbing. Her growth is on the 25th centile and a chest radiograph shows non-specific peribronchial changes.

Branch 1 — The wet cough and first move

Examiner: "This girl has coughed for five months. What is the single feature that concerns you most, and what is your first thought?" Candidate: The feature that concerns me most is that this is a daily wet, productive cough lasting far beyond four weeks that only ever partly settles with antibiotics. That pattern is chronic suppurative lung disease until proven otherwise — the reversible front end of bronchiectasis — rather than a run of viral infections. My first thought is that this needs proper assessment, a chest high-resolution CT, and a systematic search for a cause, not another short antibiotic course. [1]

Branch 2 — Pattern and differential

Examiner: "The clinical cluster here is striking. What does it point to?" Candidate: The combination of unexplained neonatal respiratory distress in a term infant, chronic glue ear and nasal discharge from infancy, and a chronic wet cough points strongly to primary ciliary dyskinesia. In ciliary dyskinesia the cilia are immotile, so mucus is never swept upward, and the same clearance failure that causes the sinopulmonary disease drives bronchiectasis. I would also keep cystic fibrosis and an immunodeficiency firmly on the list, because bronchiectasis is a symptom that always demands a cause. [1] [2]

Examiner: "Why does the neonatal history matter?" Candidate: Unexplained respiratory distress in a term neonate is a recognised early feature of primary ciliary dyskinesia, and combined with chronic rhinosinusitis and recurrent otitis media it forms a classic cluster. I would specifically ask about situs inversus, which occurs in about half of these children. [2]

Branch 3 — Investigation

Examiner: "How will you investigate her?" Candidate: I would confirm the airway change with a chest high-resolution CT, since a plain film is insensitive and here shows only non-specific change. In parallel I would do the aetiological work-up: nasal nitric oxide screening followed by specialist ciliary function and structural studies for primary ciliary dyskinesia, a sweat test and CF genetics, an immune screen with immunoglobulins and vaccine-response titres, and a lower-airway culture to define the organisms and flag Pseudomonas aeruginosa. [2] [1]

Branch 4 — Management and why timing matters

Examiner: "Assume the CT confirms bronchiectasis and she has primary ciliary dyskinesia. How do you manage her, and why act now?" Candidate: Management rests on daily airway clearance physiotherapy and prompt, prolonged, culture-guided antibiotics for exacerbations, alongside optimised nutrition, full immunisation, smoke avoidance, and specialist multidisciplinary care for the ciliary dyskinesia. I would eradicate Pseudomonas if isolated and consider long-term macrolide prophylaxis only for a high exacerbation burden. Acting now matters because in young children early bronchiectasis can regress with intensive treatment, whereas late diagnosis and chronic Pseudomonas infection commit her to fixed, progressive airway damage. [3] [1]

References

  1. [1]Chang AB, Bush A, Grimwood K Bronchiectasis in children: diagnosis and treatment. Lancet, 2018.PMID 30215382
  2. [2]Lucas JS, Barbato A, Collins SA, et al European Respiratory Society guidelines for the diagnosis of primary ciliary dyskinesia. Eur Respir J, 2017.PMID 27836958
  3. [3]Chang AB, Fortescue R, Grimwood K, et al European Respiratory Society guidelines for the management of children and adolescents with bronchiectasis. Eur Respir J, 2021.PMID 33542057