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Paeds Casesrespiratory-sleep-and-airway

Paeds Cases · respiratory-sleep-and-airway

Interstitial lung disease in children — clinical case

Clinical case of an infant with persistent tachypnoea and diffuse lung disease reaching a diagnosis of neuroendocrine cell hyperplasia of infancy, illustrating the chILD syndrome, the exclusion-HRCT-genetics pathway, and supportive management within a specialist chILD centre.

respiratory long case2 min readVerification in progress

Target exams

RACP DCEMRCPCH ClinicalABP General Pediatrics
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Target exams

RACP DCEMRCPCH ClinicalABP General Pediatrics
Prompt
A 7-month-old term infant is seen in a regional clinic with persistent fast breathing that began at around six weeks of age and has never resolved despite two courses of treatment for presumed bronchiolitis. She is on the 3rd centile for weight, has a resting respiratory rate of 66 with subcostal recession, diffuse fine crackles across both lung fields, and oxygen saturations of 90 per cent at rest that fall to 85 per cent during feeds. A chest radiograph shows non-specific diffuse haziness.

Case summary

This infant presents with the defining picture of children's interstitial lung disease in infancy: months of persistent tachypnoea that never settled despite treatment for presumed bronchiolitis, diffuse fine crackles, hypoxaemia unmasked by feeding, and failure to thrive. The essential insight is that chronic, diffuse respiratory trouble of this kind is not a run of infections but an abnormal interstitium, and she meets the chILD syndrome — respiratory symptoms, respiratory signs, hypoxaemia, and diffuse imaging change — once the common causes have been excluded. [1] [2]

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References4ShowHide
  1. [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. [2]Deterding RR, Pye C, Fan LL, et al Persistent tachypnea of infancy is associated with neuroendocrine cell hyperplasia. Pediatr Pulmonol, 2005.PMID 15965897
  3. [3]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
  4. [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
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