Khalid Aizaz, Khan Ali Raza
Surgery, Hameed Latif Hospital, Lahore, PAK.
Cureus. 2021 Nov 5;13(11):e19281. doi: 10.7759/cureus.19281. eCollection 2021 Nov.
Kartagener's syndrome is a rare autosomal recessive disorder characterized by the situs inversus, bronchiectasis, and chronic sinusitis. It is found in about half of the individuals with primary ciliary dyskinesia, a disorder of dynein arms in the cilia which renders the mucociliary apparatus inefficient. One of the manifestations of this disorder is the inability to clear secretions from the respiratory pathway leading to recurrent infections and their complications. We present a case of a 16-year-old female with the classical triad of Kartagener's syndrome who developed left-sided empyema thoracis and needed video-assisted thoracoscopic decortication for her condition.
卡塔格内综合征是一种罕见的常染色体隐性疾病，其特征为内脏转位、支气管扩张和慢性鼻窦炎。在原发性纤毛运动障碍患者中，约半数会出现该综合征，原发性纤毛运动障碍是一种纤毛中动力蛋白臂异常的疾病，可导致黏液纤毛装置功能低下。这种疾病的表现之一是无法清除呼吸道分泌物，从而导致反复感染及其并发症。我们报告一例16岁女性患者，她具有卡塔格内综合征的典型三联征，并发左侧胸腔积脓，需要通过电视辅助胸腔镜剥脱术来治疗。
