A Case of Immotile Cilia Syndrome Diagnosed by Lack of Dynein Inner Arms on Electron Microscopy.
- Author:
Sung Kil KANG
1
;
Hyun CHOI
;
Dal Hyun KIM
;
Dae Hyun LIM
;
Jung Hee KIM
;
Byong Kwan SON
;
Hye Seung HAN
Author Information
1. Department of Pediatrics, College of Medicine, Inha University, Incheon, Korea.
- Publication Type:Case Report
- Keywords:
Immotile cilia syndrome;
Lack of dynein inner arm
- MeSH:
Anti-Bacterial Agents;
Arm*;
Bronchiectasis;
Bronchitis;
Bronchodilator Agents;
Cilia;
Ciliary Motility Disorders*;
Dextrocardia;
Dyneins*;
Infertility;
Microscopy, Electron*;
Mucociliary Clearance;
Otitis Media;
Percussion;
Pneumonia;
Sinusitis;
Situs Inversus;
Thorax
- From:Pediatric Allergy and Respiratory Disease
2002;12(1):60-64
- CountryRepublic of Korea
- Language:Korean
-
Abstract:
Immotile cilia syndrome is an inherited disorder characterized by specific ultrastructural defects of cilia and associated impairment of ciliary motion and mucociliary clearance. Disorders of ciliary structure or function result in chronic sinopulmonary diseases manifested as chronic sinusitis, bronchitis, otitis media, nasal polyposis, and ultimately bronchiectasis. In addition, situs inversus, dextrocardia, and infertility can be associated with dysfunctional ciliary activity. We experienced a case of immotile cilia syndrome presenting with recurrent bronchitis, pneumonia, chronic sinusitis, otitis media, and bronchiectasis. She was diagnosed by lack of dynein inner arm on electron microscopy. Treatment included chest percussion, bronchodilators, antibiotics, and surgical intervention. She has been followed up at regular intervals. We report this case with related literatures.
