Case 1: An adolescent with chronic cough and wheezing

A 16-year-old-boy was evaluated for a two-year history of nocturnal cough and wheezing. He had not responsed to treatment with inhaled corticosteroids and long-acting beta2-agonists, a proton pump inhibitor and several courses of azithromycin. His medical history revealed no seasonal or food allergies, recurrent infections, sinusitis or diarrhea. Allergy skin testing was negative. Additional history received with the referral noted the boy experienced difficulty swallowing saliva and weight loss of several kilograms during the year before the underlying diagnosis was eventually established. On physical examination, his heart rate, respiratory rate, oxygen saturation and chest auscultation findings were normal. Lung spirometry showed a mild obstructive pattern with flattening of the expiratory flow-volume loops (Figure 1), which remained unchanged after administering salbutamol. Because of his persistent symptoms, flexible bronchoscopy was performed, which identified a marked flattening of the lumen along the full length of the trachea. The cell count in the bronchoalveolar lavage fluid contained 20% neutrophils and 4% eosinophils, and staining with Oil red O was positive, indicating the presence of fat droplets. Subsequent bronchoalveolar lavage culture was negative. Further testing confirmed the diagnosis. Figure 1) Spirometry before and after surgery