Interstitial lung disease in children — structured oral (viva)

Branching structured oral on an infant with persistent tachypnoea and diffuse lung disease, testing the chILD syndrome, the age-based classification, the surfactant-dysfunction and NEHI paradigms, and the diagnostic and management pathway for children's interstitial lung disease.

branching clinical structured oral3 min readVerification in progress

Prompt

A 4-month-old term infant has had fast breathing since early infancy that has never settled despite treatment for presumed bronchiolitis. He is thriving poorly, has a resting respiratory rate of 68 with recession, diffuse fine crackles, and oxygen saturations of 90 per cent that fall during feeds. A chest radiograph shows diffuse haziness only.

Branch 1 — Framing the problem

Examiner: "This baby has been breathing fast for months. What is the single concept that organises your thinking?" Candidate: The concept is the chILD syndrome. This is chronic, diffuse lung disease rather than a run of acute infections, and he meets the syndrome because he has respiratory symptoms, respiratory signs with tachypnoea and diffuse crackles, hypoxaemia at rest and with feeds, and diffuse change on imaging. Once I have excluded the common causes, that combination means children's interstitial lung disease until proven otherwise, and it demands a structured diffuse-lung-disease work-up rather than another course of antibiotics. [1]

Paediatrics Fellowship Pro

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References4Show ledger

[1]Kurland G, Deterding RR, Hagood JS, et al An official American Thoracic Society clinical practice guideline: classification, evaluation, and management of childhood interstitial lung disease in infancy. Am J Respir Crit Care Med, 2013.PMID 23905526
[2]Young LR, Brody AS, Inge TH, et al Neuroendocrine cell distribution and frequency distinguish neuroendocrine cell hyperplasia of infancy from other pulmonary disorders. Chest, 2011.PMID 20884725
[3]Nogee LM, Dunbar AE 3rd, Wert SE, et al A mutation in the surfactant protein C gene associated with familial interstitial lung disease. N Engl J Med, 2001.PMID 11207353
[4]Bush A, Cunningham S, de Blic J, et al European protocols for the diagnosis and initial treatment of interstitial lung disease in children. Thorax, 2015.PMID 26135832