Primary ciliary dyskinesia is an autosomal recessive disorder characterized by a history of recurrent upper and lower respiratory tract infections with chronic otitis media, bronchitis and rhinosinusitis, associated with situs inversus in 50% of cases. The diagnosis is established by ciliary ultrastructural analysis of respiratory specimens, after ruling out some disorders as cystic fibrosis, a-1 anti-trypsin deficiency, immune deficiencies (IgG, neutrophils and complement) and Young's syndrome. The purpose of this paper is to review the clinical features, diagnosis and management of primary ciliary dyskinesia, including a diagnostic algorithm.

Keywords: Ciliary dyskinesia. Situs inversus. Diagnosis.

Bronchocentric granulomatosis is histopathologically defined as being characterized by necrotizing granulomatous inflammation centered in the bronchi or bronchioles, which can be idiopathic or associated with infectious, immunologic or neoplastic diseases. This paper reports the case of a 26 year old nonasthmatic young woman with a 1-month history of chest pain, fever and productive cough. Chest radiography and computerized tomography were performed, and an open-lung biopsy was submitted to histological analysis. Radiographs and tomographs revealed consolidation and cavitation in the lingula. Transbronchial needle aspiration showed nonspecific inflammatory lesions, and no etiologic agent was identified. Histological examination of the specimen obtained by open-lung biopsy was consistent with idiopathic bronchocentric granulomatosis. The patient responded positively to prednisone therapy, and no relapse was documented.

Keywords: Bronchocentric granulomatosis. Bronchi. Allergic bronchopulmonary aspergillosis. Prednisone/therapeutic use.