Kartagener's syndrome with immunoglobulin G subclass deficiency

摘要: Kartagener syndrome is characterized by the triad of situs inversus, bronchiectasis, and chronic paranasal sinusitis. Recurrent sinopulmonary infection, major determinant for diagnosing immunodeficiency, most common clinical manifestation disease. A 17-year-old female patient presented with dyspnea, cough, sputum, nasal congestion, rhinorrhea more than 5 years. Nasal symptoms dyspnea had not been controlled intermittent treatment mucolytics antibiotics from primary clinics since 3 months before visiting our clinic. Chest X-ray computed tomography showed dextrocardia bronchiectasis. Paranasal sinus series revealed mucosal thickening haziness on both maxillary sinus. Serum immunoglobulin (Ig) G4 was decreased, but total IgG within normal range. Under diagnosis IgG4 deficiency, monthly intravenous (IVIG) performed 6 months. Her were well frequency use markedly decreased. We report a having deficiency that successfully 6-month-treatment IVIG. (Allergy Asthma Respir Dis 2013;1:288-291)