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疑似纤毛运动障碍的支气管扩张症患者中慢性鼻-鼻窦炎的患病率。

Prevalence of chronic rhinosinusitis in bronchiectasis patients suspected of ciliary dyskinesia.

机构信息

Department of Otolaryngology-Head and Neck Surgery, University of Alabama at Birmingham, Birmingham, AL.

Department of Pathology, Children's Hospital of Alabama, Birmingham, AL.

出版信息

Int Forum Allergy Rhinol. 2019 Dec;9(12):1430-1435. doi: 10.1002/alr.22414. Epub 2019 Aug 20.

Abstract

BACKGROUND

Mucociliary clearance is a main defense mechanism of the airway and is impaired in ciliary dyskinesia. The objective of this study was to evaluate the prevalence of chronic rhinosinusitis (CRS) and its characteristics in bronchiectasis patients suspected of harboring ciliary dyskinesia.

METHODS

Bronchiectasis patients referred to a rhinology clinic for nasal brush biopsy (NBB) were included in this study. NBB was performed using a curettage technique whereby ciliated epithelial cells were obtained from the surface of the inferior nasal turbinate. Results of transmission electron microscopy findings, primary ciliary dyskinesia (PCD) gene (35 genes) analyses (Invitae), and sinus computed tomography (CT) scans were reviewed.

RESULTS

Twenty-three patients (age, 54 ± 2.9 years) were referred for NBB between 2015 and 2018. Thirteen patients (56.5%) met the criteria for diagnosis of CRS. Nineteen patients had ciliary ultrastructural defects. The most common finding was compound cilia (n = 11, 47.8%). Five patients (21.7%) had central microtubule defects (CMD) with higher forced expiratory volume in 1 second (FEV ) at the time of referral than those without CMD (CMD , 91 ± 3.7%; CMD , 73.5 ± 5.7%; p = 0.023). Of 15 subjects with a PCD gene panel, 67% (9 of 15) carried at least 1 gene associated with PCD. Only 1 patient reached diagnosis of PCD. Approximately 50% of non-PCD carriers had a smoking history (p < 0.05). Lund-Mackay scores did not significantly differ between PCD and non-PCD carriers (p = 0.72).

CONCLUSION

Nearly half of bronchiectasis patients referred for NBB had concurrent CRS. The presence of ciliary abnormalities was not amplified in bronchiectasis patients with CRS compared to those without CRS. Extrinsic factors may be related to ciliary structural abnormalities in non-PCD gene carriers.

摘要

背景

黏液纤毛清除功能是气道的主要防御机制,在纤毛运动障碍中受损。本研究的目的是评估疑似存在纤毛运动障碍的支气管扩张症患者慢性鼻窦炎(CRS)的患病率及其特征。

方法

本研究纳入了因怀疑存在纤毛运动障碍而被转至鼻科诊所进行鼻刷活检(NBB)的支气管扩张症患者。NBB 采用刮除法进行,从下鼻甲表面获得纤毛上皮细胞。我们对透射电子显微镜检查结果、原发性纤毛运动障碍(PCD)基因(35 个基因)分析(Invitae)和鼻窦计算机断层扫描(CT)结果进行了回顾。

结果

2015 年至 2018 年期间,有 23 名(年龄 54 ± 2.9 岁)患者因 NBB 被转至本研究。13 名(56.5%)患者符合 CRS 诊断标准。19 名患者存在纤毛超微结构缺陷。最常见的发现是复合纤毛(n = 11,47.8%)。5 名(21.7%)患者存在中央微管缺陷(CMD),CMD 患者的第一秒用力呼气量(FEV )在就诊时更高(CMD ,91 ± 3.7%;CMD ,73.5 ± 5.7%;p = 0.023)。在 15 名接受 PCD 基因检测的患者中,67%(9 名)至少携带 1 个与 PCD 相关的基因。仅 1 名患者被诊断为 PCD。约 50%的非 PCD 基因携带者有吸烟史(p < 0.05)。PCD 和非 PCD 基因携带者的 Lund-Mackay 评分无显著差异(p = 0.72)。

结论

近一半因 NBB 被转至本研究的支气管扩张症患者同时患有 CRS。与无 CRS 的患者相比,CRS 患者的纤毛异常发生率并未增加。非 PCD 基因携带者的纤毛结构异常可能与外在因素有关。

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