🫁 Section 448.5 · Interstitial Lung Disease (chILD)

Nelson Textbook of Pediatrics 22nd Edition | Childhood interstitial lung disease (chILD) encompasses rare disorders of pulmonary parenchyma. Major categories: surfactant dysfunction disorders (SFTPB, SFTPC, ABCA3, NKX2-1), neuroendocrine cell hyperplasia of infancy (NEHI), pulmonary interstitial glycogenosis (PIG), hypersensitivity pneumonitis, connective tissue disease-associated ILD, pulmonary alveolar proteinosis (GM-CSF autoantibodies or receptor mutations). Clinical: tachypnea, crackles, hypoxemia, failure to thrive. Diagnosis: HRCT (ground-glass, septal thickening, cysts), genetic testing, lung biopsy (gold standard). Treatment: supportive (oxygen, nutrition), corticosteroids, hydroxychloroquine, azithromycin, lung transplantation.

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📋 30 Clinical Scenarios — Interstitial Lung Disease (chILD)

📇 High‑Yield Cards: Interstitial Lung Disease (Chap 448.5)

🩺 Interactive Clinical Approach: Suspected chILD

Select a presentation for diagnostic and management approach.

📋 Stepwise Management of Childhood ILD

    Whole lung lavage, inhaled GM-CSF, hematopoietic stem cell transplant (genetic)
    Disease CategoryExamplesTreatment
    Surfactant dysfunctionSFTPB, SFTPC, ABCA3, NKX2-1Supportive care, hydroxychloroquine, lung transplantation (SFTPB lethal, ABCA3 variable)
    NEHINeuroendocrine cell hyperplasia of infancySupportive (oxygen, nutrition); improves with age
    PIGPulmonary interstitial glycogenosisOften improves spontaneously, may respond to steroids
    Pulmonary alveolar proteinosis (PAP)GM-CSF autoantibodies (autoimmune), CSF2RA/B mutations
    Connective tissue disease-associated ILDSLE, JIA, sclerodermaTreat underlying disease, corticosteroids, mycophenolate, rituximab

    ⚡ Reflex Prompts — chILD Recognition & Management

    📖 Summary: Interstitial Lung Disease (chILD) — Nelson 22nd Ed