Halvorson D J, Dupree J R, Porubsky E S
Department of Surgery, University of Alabama at Birmingham, 35233, USA.
Ann Otol Rhinol Laryngol. 1998 Nov;107(11 Pt 1):946-52. doi: 10.1177/000348949810701108.
Cystic fibrosis (CF) is an autosomal recessive disorder affecting exocrine gland function. Although CF was formerly a deadly disease of infants and children, recent improvements in antibiotics, nutritional therapy, and supportive care have extended the median survival to adulthood. Patients with CF often present with sinusitis and nasal polyposis in addition to recurrent pulmonary infections. Although the effectiveness of endoscopic sinus surgery in children with CF has been documented, the treatment guidelines and efficacy in the adult CF patient are unknown. We present a series of 16 adult patients with CF and chronic sinusitis. The majority of patients presented with nasal polyposis and concomitant pulmonary complications. Endoscopic findings are reviewed, with an emphasis on improving pulmonary function following endoscopic sinus surgery. Preliminary findings suggest that endoscopic sinus surgery improves symptoms of sinusitis and exercise tolerance and may delay the progressive respiratory failure that often affects the adult CF patient.
囊性纤维化(CF)是一种影响外分泌腺功能的常染色体隐性疾病。虽然CF以前是婴幼儿的致命疾病,但近期抗生素、营养治疗和支持性护理方面的进展已将中位生存期延长至成年期。CF患者除反复肺部感染外,还常伴有鼻窦炎和鼻息肉。虽然内镜鼻窦手术在CF儿童患者中的有效性已有文献记载,但针对成年CF患者的治疗指南和疗效尚不清楚。我们报告了16例成年CF合并慢性鼻窦炎患者。大多数患者伴有鼻息肉和肺部并发症。对内镜检查结果进行了回顾,重点是内镜鼻窦手术后肺功能的改善。初步研究结果表明,内镜鼻窦手术可改善鼻窦炎症状和运动耐量,并可能延缓常影响成年CF患者的进行性呼吸衰竭。