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不动纤毛综合征的耳科表现。

Otologic manifestations of the immotile-cilia syndrome.

作者信息

Ernstson S, Afzelius B A, Mossberg B

出版信息

Acta Otolaryngol. 1984 Jan-Feb;97(1-2):83-92. doi: 10.3109/00016488409130967.

DOI:10.3109/00016488409130967
PMID:6537791
Abstract

The immotile-cilia syndrome is caused by a hereditary, inborn, ultrastructural defect of the cilia, rendering them immotile or poorly motile and thereby abolishing mucociliary clearance. Five cases are presented and the otologic manifestations are described in some detail. The syndrome should be suspected in children who have a persistent secretory otitis media with recurring bouts of acute otitis media, and a perpetual cough with repeated episodes of bronchitis. A lobar atelectasis is a frequent finding. Half the cases also have situs inversus. The immotile-cilia syndrome is of special interest to the otologist, as it seems to throw some light on the pathogenesis of secretory otitis media.

摘要

不动纤毛综合征由纤毛的遗传性、先天性超微结构缺陷引起,使纤毛不能运动或运动不良,从而消除了黏液纤毛清除功能。本文报告了5例病例,并对耳部表现进行了较为详细的描述。对于患有持续性分泌性中耳炎并反复发作急性中耳炎,以及持续性咳嗽并反复发生支气管炎的儿童,应怀疑患有该综合征。肺叶不张是常见的表现。半数病例还伴有内脏转位。不动纤毛综合征引起了耳科医生的特别关注,因为它似乎有助于阐明分泌性中耳炎的发病机制。

相似文献

1
Otologic manifestations of the immotile-cilia syndrome.不动纤毛综合征的耳科表现。
Acta Otolaryngol. 1984 Jan-Feb;97(1-2):83-92. doi: 10.3109/00016488409130967.
2
Clinical expressions of immotile cilia syndrome.
Pediatrics. 1981 Jun;67(6):805-10.
3
[A case of immotile cilia syndrome and a review of Japanese cases].[一例不动纤毛综合征及日本病例综述]
Nihon Kyobu Shikkan Gakkai Zasshi. 1989 Apr;27(4):505-12.
4
On the pathogenesis of obstructive lung disease. A study on the immotile-cilia syndrome.关于阻塞性肺病的发病机制。不动纤毛综合征的研究。
Scand J Respir Dis. 1978 Apr;59(2):55-65.
5
A human syndrome caused by immotile cilia.一种由不动纤毛引起的人类综合征。
Science. 1976 Jul 23;193(4250):317-9. doi: 10.1126/science.1084576.
6
Otitis media and the immotile cilia syndrome.
Laryngoscope. 1979 May;89(5 Pt 1):769-78. doi: 10.1288/00005537-197905000-00015.
7
[Serous otitis and ciliary dyskinesia syndrome].[浆液性中耳炎与纤毛运动障碍综合征]
Acta Otorrinolaringol Esp. 1991 Sep-Oct;42(5):393-7.
8
Measurements of tracheobronchial clearance in patients with immotile-cilia syndrome and its value in differential diagnosis.
Eur J Respir Dis Suppl. 1983;127:57-63.
9
Ultrastructural, cellular, and clinical features of the immotile-cilia syndrome.不动纤毛综合征的超微结构、细胞及临床特征
Annu Rev Med. 1984;35:481-92. doi: 10.1146/annurev.me.35.020184.002405.
10
Reflection of structural abnormality in the axoneme of respiratory cilia in the clinical features of immotile cilia syndrome.呼吸纤毛轴丝结构异常在不动纤毛综合征临床特征中的反映
Intern Med. 1996 Aug;35(8):617-23. doi: 10.2169/internalmedicine.35.617.

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OTO Open. 2025 Feb 21;9(1):e70079. doi: 10.1002/oto2.70079. eCollection 2025 Jan-Mar.
2
ENT manifestations in patients with primary ciliary dyskinesia: prevalence and significance of otorhinolaryngologic co-morbidities.原发性纤毛运动障碍患者的 ENT 表现:耳鼻喉科合并症的患病率和意义。
Eur Arch Otorhinolaryngol. 2011 Mar;268(3):383-8. doi: 10.1007/s00405-010-1341-9. Epub 2010 Jul 22.