Department of Pulmonology and Immunology, Medical Academy, Lithuanian University of Health Sciences, Kaunas, Lituania.
Arch Bronconeumol. 2013 Jan;49(1):28-30. doi: 10.1016/j.arbres.2012.02.021. Epub 2012 Apr 30.
Kartagener's syndrome is a rare autosomal-recessive genetic disease with progressive damage of the respiratory system and situs inversus. Although the management of patients with Kartagener's syndrome remains uncertain and evidence is limited, it is important to follow up these patients with an adequate and shared care system. This report presents a clinical case of Kartagener's syndrome in a 25-year-old woman. Computed tomography showed dextrocardia and bronchiectasis. Abdominal X-ray and ultrasound confirmed situs inversus totalis. After 7 years, good treatment results were achieved: lung function improved and radiological findings showed no changes. The present case discusses the complex interrelationship between the genetic variation and a proper nonspecific management of Kartagener's syndrome.
卡塔格内氏综合征是一种罕见的常染色体隐性遗传疾病，具有呼吸系统进行性损伤和内脏转位。尽管卡塔格内氏综合征患者的管理仍然不确定，证据也有限，但重要的是要通过充分和共享的护理系统对这些患者进行随访。本报告介绍了一例 25 岁女性卡塔格内氏综合征的临床病例。计算机断层扫描显示右位心和支气管扩张。腹部 X 线和超声证实全内脏转位。7 年后，取得了良好的治疗效果：肺功能改善，影像学未见变化。本病例讨论了遗传变异与卡塔格内氏综合征的适当非特异性治疗之间的复杂相互关系。
