A fifteen-year-old female was transferred to the intensive paediatric care unit due to pulmonary haemorrhage, requiring mechanical ventilation, and renal insufficiency.
She has no relevant family history and has neonatal antecedents of extreme prematurity and bronchopulmonary dysplasia.
At eleven years of age, she started having episodes of respiratory distress, wheezing, coughing, and stridor that lead to multiple hospitalizations despite therapy with beta2 long-acting agonists, antileukotrienes, and inhaled corticosteroids. In the exacerbations, she presented weak response to salbutamol and improvement with adrenaline and methylprednisolone. In this context, an etiological investigation was carried out at the hospital in her residential area: negative allergy screening; normal immunoglobulin and complement levels; normal alpha-1 antitrypsin level; negative sweat test; negative Ziehl-Neelsen staining and Mycobacterium tuberculosis culture in gastric juice; and normal upper gastrointestinal endoscopy, esophageal-gastroduodenal transit, and thoracic computed tomography.