Kendigelen Pınar, Tütüncü Ayşe Çiğdem, Erbabacan Şafak Emre, Kaya Güner, Altındaş Fatiş
Department of Anaesthesiology and Reanimation, İstanbul University Cerrahpaşa Faculty of Medicine, İstanbul, Turkey.
Turk J Anaesthesiol Reanim. 2015 Jun;43(3):205-8. doi: 10.5152/TJAR.2015.94546. Epub 2015 Feb 16.
Kartagener syndrome is an autosomal recessive disorder characterized by primary ciliary dyskinesia accompanied by sinusitis, bronchiectasis, and situs inversus. Synchronous extrahepatic biliary atresia and Kartagener syndrome are very rare. During the preoperative preparation of patients with Kartagener syndrome, special attention is required for the respiratory and cardiovascular system. It is important to provide suitable anaesthetic management to avoid problems because of ciliary dysfunction in the perioperative period. Further, maintaining an effective pain control with regional anaesthetic methods reduces the risk of pulmonary complications. Infants with biliary atresia operated earlier have a higher chance of survival. Hepatic dysfunction and decrease in plasma proteins are important for the kinetics of drugs. In this presentation, the anaesthetic management of patients with synchronous Kartagener syndrome and biliary atresia, both of which are rare diseases, is evaluated.
卡塔格内综合征是一种常染色体隐性疾病,其特征为原发性纤毛运动障碍,并伴有鼻窦炎、支气管扩张和内脏转位。同步性肝外胆道闭锁和卡塔格内综合征非常罕见。在卡塔格内综合征患者的术前准备过程中,需要特别关注呼吸和心血管系统。提供合适的麻醉管理以避免围手术期因纤毛功能障碍而出现问题很重要。此外,采用区域麻醉方法维持有效的疼痛控制可降低肺部并发症的风险。早期接受手术的胆道闭锁婴儿存活几率更高。肝功能障碍和血浆蛋白降低对药物动力学有重要影响。在本报告中,对同步患有卡塔格内综合征和胆道闭锁这两种罕见疾病的患者的麻醉管理进行了评估。