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[卡塔格内综合征鼻黏膜纤毛超微结构观察]

[Observation of nasal mucosal cilia ultrastructure of Kartagener's syndrome].

作者信息

Zhou B, Guan Y, Liu S, Sun J, Zhang L, Han D

机构信息

Department of Otorhinolarngology, Affiliated Beijing Tongren Hospital, Capital University of Medical Sciences, Beijing 100730, China.

出版信息

Zhonghua Er Bi Yan Hou Ke Za Zhi. 2001 Oct;36(5):323-5.

PMID:12761935
Abstract

OBJECTIVE

To report the ultrastructure of nasal mucosal cilia of Kartagener's syndrome and the operative results of 2 cases.

METHODS

Both two cases were underwent endoscopic sinus surgery. Transmission and scanning electron microscopic examinations of nasal mucosa for case 1 were performed.

RESULTS

All 2 cases presented situs inversus, chronic paranasal sinusitis and bronchiectasis. The electron microscopic examination showed that the shape of nasal cilia and "9 + 2" structure were normal, but the lack of inner dynein arm of cilia and confused arrangement of central pair of microtubules were confirmed. The recurrent of nasal polyps after 1-year follow-up was observed in case 1.

CONCLUSIONS

The disturbance of mucociliary clearance due to congenital ciliary structure defect might be the cause of chronic sinusitis and polyps, which might be one of reasons for poor prognosis after operation and we should pay more close attention.

摘要

目的

报告卡塔格内综合征鼻黏膜纤毛的超微结构及2例手术结果。

方法

2例均行鼻内镜鼻窦手术。对病例1的鼻黏膜进行透射和扫描电子显微镜检查。

结果

2例均表现为内脏转位、慢性鼻窦炎和支气管扩张。电子显微镜检查显示鼻纤毛形态及“9+2”结构正常,但证实存在纤毛内动力蛋白臂缺失及中央微管对排列紊乱。病例1随访1年观察到鼻息肉复发。

结论

先天性纤毛结构缺陷导致的黏液纤毛清除功能障碍可能是慢性鼻窦炎和息肉的病因,这可能是术后预后不良的原因之一,应予以更多关注。

相似文献

1
[Observation of nasal mucosal cilia ultrastructure of Kartagener's syndrome].[卡塔格内综合征鼻黏膜纤毛超微结构观察]
Zhonghua Er Bi Yan Hou Ke Za Zhi. 2001 Oct;36(5):323-5.
2
Cilia motility and structure in primary and secondary ciliary dyskinesia.原发性和继发性纤毛运动障碍中的纤毛运动和结构。
Am J Rhinol Allergy. 2010 May-Jun;24(3):175-80. doi: 10.2500/ajra.2010.24.3448.
3
[How useful is the ultrastructural study of the cilia of the respiratory tract in the diagnosis of an immotile cilia syndrome?].[呼吸道纤毛超微结构研究在诊断纤毛不动综合征中具有多大作用?]
Schweiz Med Wochenschr. 1984 May 5;114(18):610-9.
4
Transposed ciliary microtubules in Kartagener's syndrome. A case report with electron microscopy of bronchial and nasal brushings.
Acta Cytol. 1985 May-Jun;29(3):248-53.
5
Screening for ciliary dyskinesia - a spectrum of defects of motility and structure.筛查纤毛运动障碍——一系列运动和结构缺陷。
Eur J Respir Dis Suppl. 1983;127:71-7.
6
The immotile cilia syndrome: phase contrast light microscopy, scanning and transmission electron microscopy.
Pediatrics. 1980 Apr;65(4):698-702.
7
[Ultrastructure of cilia in Kartagener syndrome].
Laryngol Rhinol Otol (Stuttg). 1984 Jan;63(1):33-40.
8
[Observation of maxillary mucosa restoration after the endoscopic sinus surgery operation of chronic sinusitis and nasal polyps].[慢性鼻窦炎和鼻息肉鼻内镜鼻窦手术后上颌黏膜修复的观察]
Zhonghua Er Bi Yan Hou Ke Za Zhi. 2004 Jul;39(7):402-6.
9
Specific types of abnormal ciliary motility in Kartagener's syndrome and analogous respiratory disorders. A quantified microphoto-oscillographic investigation of 27 patients.卡塔格内综合征及类似呼吸系统疾病中特定类型的异常纤毛运动。对27例患者的定量显微光振荡描记研究。
Eur J Respir Dis Suppl. 1983;127:78-90.
10
[Are ciliary abnormalities always present in Kartagener's syndrome? A study of 16 patients].[卡塔格内综合征中纤毛异常是否总是存在?对16例患者的研究]
Ann Otolaryngol Chir Cervicofac. 1989;106(6):302-5.

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Clinical and genetic spectrum of primary ciliary dyskinesia in Chinese patients: a systematic review.原发性纤毛运动障碍的临床和遗传学特征:系统综述。
Orphanet J Rare Dis. 2022 Jul 19;17(1):283. doi: 10.1186/s13023-022-02427-1.