The clinical respiratory journal
-
Case Reports
Partial pleural covering for intractable pneumothorax in patients with Birt-Hogg-Dubé Syndrome.
Birt-Hogg-Dubé syndrome (BHD) is an inherited disorder associated with a germline mutation of the folliculin (FLCN) gene. Most patients with BHD have multiple pulmonary cysts, and are at high risk of repeated pneumothorax. Although an increasing number of patients are diagnosed with BHD by genetic testing, therapeutic approaches for intractable pneumothorax have not yet been described. ⋯ Partial pleural covering combined with resection of protruding cysts should be a safe and effective therapeutic approach for BHD patients with intractable pneumothorax. Further investigation is needed to establish a detailed protocol for treatment of pneumothorax that results in minimal functional impairment.
-
Lipoid pneumonia is an uncommon disease caused by the presence of lipid in the alveoli. Here we described a case of a 50-year-old woman with haemoptysis, cough and tachypnea, who was diagnosed with cholesterol pneumonia accompanying with pulmonary artery hypertension. The extremely high pulmonary artery pressure achieved, in this case, is alarming and should alert the physicians that the cholesterol pneumonia may be one of the underlying causes of pulmonary artery hypertension. After a treatment of methylprednisolone, her clinical symptoms were significantly improved, which suggested that steroid might be a promising therapeutic for patients with cholesterol pneumonia.
-
The application of radial probe endobronchial ultrasound (R-EBUS) and virtual bronchoscopic navigation has improved the diagnostic outcome of bronchoscopy for peripheral pulmonary lesions (PPLs). Nonetheless, while existing navigation systems are very useful for selecting the bronchus containing the target lesion, the associated introductory costs are high. Therefore, we focused on virtual bronchoscopy (VB) using the workstation, ziostation that was already available in many countries as an adjunct modality. ⋯ VB offered by the workstation was a valuable tool that facilitated more accurate and rapid bronchoscopy procedure for diagnosis of PPLs.