Casserly P, Harrison M, O'Connell O, O'Donovan N, Plant B J, O'Sullivan P
Department of Otolaryngology, South Infirmary Victoria University Hospital, Cork, Republic of Ireland.
Cork Adult Cystic Fibrosis Centre, University College Cork, Cork University Hospital, Cork, Ireland.
Eur Arch Otorhinolaryngol. 2015 Nov;272(11):3353-9. doi: 10.1007/s00405-014-3446-z. Epub 2014 Dec 24.
Cystic fibrosis (CF) is a common inherited disorder in Caucasians in Ireland having the highest reported incidence. CF has well-recognised clinical sequelae in several physiological systems. Its' impact on the sinonasal system is less well established. We evaluated symptoms, endoscopic and computerised tomographic (CT) findings in an Irish adult CF group with the aim of characterising the relationship between these clinical features in an Irish CF group. Adult CF patients attending a specialist clinic underwent prospective evaluation of sinonasal symptoms using a specifically designed questionnaire. They subsequently underwent nasoendoscopy and CT scanning of their paranasal sinuses. Abnormalities identified were quantified using established radiological (Lund-Mackay) and endoscopic (Lund-Kennedy) scoring systems. The relationship between symptoms of chronic rhinosinusitis (CRS), endoscopic findings and CT abnormalities were then compared. Sixty-three CF patients (n = 63) were studied. 29 patients had a CT scan. Thirty-three CF patients (52%) had no symptoms of CRS. Fifty CF patients (80% of CF group) had evidence of CRS on nasoendoscopy including thirteen patients (20%) with nasal polyposis. 98% of patients scanned have positive findings on CT scan. There was no significant difference between symptomatic and asymptomatic CF groups with respect to their Lund-Kennedy endoscopic score or their Lund-Mackay CT score. 86% demonstrated one or more hypoplastic sinus. There was no increased incidence of hypoplastic sinuses amongst Δf508 homozygotes than other mutation groups.
囊性纤维化(CF)是爱尔兰白种人中常见的遗传性疾病,报告发病率最高。CF在多个生理系统中具有公认的临床后遗症。其对鼻窦系统的影响尚不明确。我们评估了一组爱尔兰成年CF患者的症状、内镜检查和计算机断层扫描(CT)结果,旨在明确爱尔兰CF患者这些临床特征之间的关系。在专科门诊就诊的成年CF患者使用专门设计的问卷对鼻窦症状进行前瞻性评估。随后他们接受了鼻内镜检查和鼻窦CT扫描。使用既定的放射学(Lund-Mackay)和内镜(Lund-Kennedy)评分系统对发现的异常进行量化。然后比较慢性鼻窦炎(CRS)症状、内镜检查结果和CT异常之间的关系。研究了63例CF患者(n = 63)。29例患者进行了CT扫描。33例CF患者(52%)没有CRS症状。50例CF患者(占CF组的80%)在内镜检查中有CRS证据,其中13例(20%)有鼻息肉。98%接受扫描的患者CT扫描有阳性结果。有症状和无症状CF组之间的Lund-Kennedy内镜评分或Lund-Mackay CT评分没有显著差异。86%的患者显示一个或多个鼻窦发育不全。与其他突变组相比,Δf508纯合子中鼻窦发育不全的发生率没有增加。