Nelson Textbook of Pediatrics 22nd Edition | Bronchiolitis obliterans (post-infectious: adenovirus, Mycoplasma; post-transplant), follicular bronchitis (lymphoid hyperplasia, COPA mutations), pulmonary alveolar microlithiasis (SCL34A2 mutations, sandstorm appearance). Clinical: chronic cough, wheezing, dyspnea, hypoxemia. Diagnosis: HRCT (mosaic attenuation, air trapping), lung biopsy (gold standard). Treatment: supportive, corticosteroids, immunomodulators, lung transplantation for end-stage disease.
๐ paeds.online โ Pakistan's Pediatric Platform| Disease | Key Features | Treatment |
|---|---|---|
| Bronchiolitis obliterans (post-infectious) | Post-adenovirus, M. pneumoniae, measles. Irreversible obstruction, mosaic attenuation on HRCT | Supportive, corticosteroids (pulse), azithromycin, lung transplant if severe |
| Bronchiolitis obliterans syndrome (post-transplant) | Chronic lung allograft rejection, declining FEV1 | Optimize immunosuppression, azithromycin, photopheresis, re-transplantation |
| Follicular bronchitis | Lymphoid hyperplasia, associated with immune dysregulation (CVID, COPA mutations), lymphocytic interstitial pneumonia | Corticosteroids, immunomodulators, treat underlying immunodeficiency |
| Pulmonary alveolar microlithiasis | ||
| No specific treatment; lung transplantation for end-stage disease |