Weeks A M, Buckland M R
Alfred Group of Hospitals, Prahran, Victoria.
Anaesth Intensive Care. 1995 Jun;23(3):332-8. doi: 10.1177/0310057X9502300310.
Cystic fibrosis is an autosomal recessive disorder which affects one in 2500 live births. It is a multisystem disease and has a variety of presentations. The major clinical features affect the gastrointestinal and respiratory tracts. Severe respiratory disease, diabetes and gastroesophageal reflux are common features of concern to anaesthetists. Improved care of young patients has allowed many to survive into adulthood. Lung transplantation has significantly improved the outlook for many patients. At Alfred Hospital, 74 patients with cystic fibrosis underwent 149 procedures from January 1978 to January 1994, with a mortality of 0.6% (95% CI 0.4%-0.8%). This retrospective cohort study describes the anaesthetic management and perioperative care of these patients. Most of the anaesthetics were for procedures related to cystic fibrosis but 12% were for unrelated conditions. Cystic fibrosis related procedures include diagnostic, venous access, enteral feeding procedures, treatment of complications of cystic fibrosis and lung transplantation. Despite extremely poor respiratory function, these patients can be managed with acceptably low postoperative mortality (1%). Pre- and postoperative care must be directed towards optimal clearance of viscous respiratory secretions. Procedures need to be planned so that optimal care can be given by each member of the team caring for cystic fibrosis patients.
囊性纤维化是一种常染色体隐性疾病，每2500例活产儿中就有1例受其影响。它是一种多系统疾病，有多种临床表现。主要临床特征影响胃肠道和呼吸道。严重的呼吸系统疾病、糖尿病和胃食管反流是麻醉医生关注的常见特征。对年轻患者护理的改善使许多人存活至成年。肺移植显著改善了许多患者的前景。在阿尔弗雷德医院，从1978年1月至1994年1月，74例囊性纤维化患者接受了149例手术，死亡率为0.6%（95%可信区间0.4%-0.8%）。这项回顾性队列研究描述了这些患者的麻醉管理和围手术期护理。大多数麻醉用于与囊性纤维化相关的手术，但12%用于无关病症。与囊性纤维化相关的手术包括诊断、静脉通路、肠内喂养手术、囊性纤维化并发症的治疗和肺移植。尽管呼吸功能极差，但这些患者术后死亡率可控制在可接受的低水平（1%）。术前和术后护理必须针对粘性呼吸道分泌物的最佳清除。手术需要精心规划，以便负责护理囊性纤维化患者的团队成员能够提供最佳护理。
