Kim Chang Hun, Son Bong Soo, Son Joohyung, Kim Do Hyung
Department of Thoracic and Cardiovascular Surgery, Pusan National University Yangsan Hospital, Pusan National University School of Medicine, Yangsan, Korea.
J Chest Surg. 2022 Feb 5;55(1):77-80. doi: 10.5090/jcs.21.086.
Kartagener syndrome (KS) is a rare disease with an incidence of 1 in 20,000 to 30,000 births. There is no cure for KS, and conservative medical treatments are used to relieve symptoms and prevent disease progression. Lung transplantation (LT) is the only treatment option for end-stage KS. Since patients with KS have anatomical abnormalities such as situs inversus totalis, which often require surgery to correct, most reports are related to surgical techniques. Reports about morphological adaptations and changes in transplanted lung structure after LT in patients with KS are rare. We performed LT in a patient with KS and observed morphological adaptation of the lungs for 6 months on chest computed tomography using a quantitative evaluation tool (Chest Image Platform; Harvard University Disability Resources, Cambridge, MA, USA).
卡塔格内综合征（KS）是一种罕见疾病，发病率为每20000至30000例出生中有1例。KS无法治愈，采用保守药物治疗来缓解症状并防止疾病进展。肺移植（LT）是终末期KS的唯一治疗选择。由于KS患者存在诸如全内脏转位等解剖学异常，这通常需要手术矫正，因此大多数报告都与手术技术有关。关于KS患者LT后移植肺结构的形态学适应和变化的报告很少。我们对一名KS患者进行了LT，并使用定量评估工具（胸部图像平台；美国马萨诸塞州剑桥市哈佛大学残疾资源中心）通过胸部计算机断层扫描观察了肺部的形态学适应情况，为期6个月。
