Dhar D K, Ganguly K C, Alam S, Hossain A, Sarker U K, Das B K, Haque M J
Department of Medicine, Mymensingh Medical College and Hospital, Mymensingh, Bangladesh.
Mymensingh Med J. 2009 Jan;18(1):75-9.
Kartagener's Syndrome or Immotile Cilia Syndrome, a variant of Primary Ciliary Dyskinesia (PCD), is a rare autosomal recessive genetic disorder caused by defect in the tiny hair like structure, the cilia lining the respiratory tract (upper and lower), sinuses, eustachian tubes, middle ear and fallopian tubes. Here electron microscopy shows abnormal arrangement of ciliary tubules and patients with Kartagener's syndrome has an absence of dynein arms at the base of the cilia. The inability of cilia to move results in inadequate clearance of bacteria from the air passages, resulting in an increased risk of infection and causing bronchiectasis. Another result of ciliary immobility is infertility. A 60 years old lady was diagnosed as a case of Kartagener's syndrome. She had history of chronic cough for 20 years, irregular fever for 20 years and occasional shortness of breath for 5 years. Relevant investigations revealed dextrocardia, situs inversus, bilateral maxillary sinusitis with non pneumatised frontal sinus and bronchiectasis. She was treated with low concentration oxygen inhalation, antibiotic, bronchodilator, chest physiotherapy including postural drainage, vitamins and other supportive treatment.
卡塔格内综合征或不动纤毛综合征，是原发性纤毛运动障碍（PCD）的一种变体，是一种罕见的常染色体隐性遗传病，由呼吸道（上、下呼吸道）、鼻窦、咽鼓管、中耳和输卵管内衬的微小毛发状结构——纤毛的缺陷引起。在此，电子显微镜显示纤毛微管排列异常，卡塔格内综合征患者的纤毛基部缺乏动力蛋白臂。纤毛无法移动导致气道内细菌清除不足，从而增加感染风险并引发支气管扩张。纤毛不动性的另一个结果是不孕。一名60岁女性被诊断为卡塔格内综合征病例。她有20年慢性咳嗽病史、20年不规则发热病史以及5年偶尔气短病史。相关检查显示右位心、内脏转位、双侧上颌窦炎伴额窦未气化以及支气管扩张。她接受了低浓度吸氧、抗生素、支气管扩张剂、包括体位引流的胸部物理治疗、维生素及其他支持性治疗。
