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Answers on p 547.
A 16 year old Irish girl was referred by her general practitioner to the respiratory outpatient clinic with a nine year history of wheeze on exertion, which was getting progressively worse. She had a minimal response to regular inhaled low dose corticosteroids and required short acting β-agonists. There was no history of cough, sputum production, or haemoptysis. Her past medical history was unremarkable, and she described her development as normal during her childhood. She was a regular school attender and never smoked. She was on no other medications, and her siblings and other family members were devoid of any breathing difficulties. Physical examination showed a well built young woman in no respiratory distress, with the following physical characteristics: weight, 45 kg; height, 155 cm; pulse, 80 beats/min; blood pressure, 110/60 mm Hg; and respiratory rate, 18 breaths/min. An examination of the chest revealed forced expiratory rhonchi. The rest of the physical examination was normal. Skin allergy tests showed that she was allergic to the house dust mite and grass mix.
Pulmonary function tests demonstrated a forced expiratory volume in one second (FEV1) of 1.84 l/min (63% of predicted value), forced vital capacity (FVC) of 2.31 l/min (75% of predicted value), and a FEV1 /FVC ratio of 80% of predicted value. Lung volumes, performed by the helium dilution technique, confirmed a restrictive lung defect, with a total lung capacity of 2.83 litres (71% of predicted value), and a residual volume of 0.45 litres (53% of predicted value). Plain radiographs and computed tomography of the chest were performed (figs 1 and 2). The patient was given a trial of high dose inhaled corticosteroids, and was also started on inhaled long acting β-agonists. On review three months later the patient was no better, and bronchoscopy was performed (fig 3).
Describe the findings on radiography and computed tomography.
Describe the findings on bronchoscopy.
What is the most likely diagnosis?
How would you manage this patient?
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