A Case of Primary Ciliary Dyskinesia due to Radial Spokes Deficiency.
- Author:
Kwang Bum KIM
1
;
Jung Lae ROH
;
Jun Kyu LEE
;
Sung Won PARK
Author Information
1. Department of Otolaryngology-Head and Neck Surgery, Wonju College of Medicine, Yonsei University, Wonju, Korea. rhica@unitel.co.kr
- Publication Type:Original Article
- Keywords:
Primary ciliary dyskinesia;
Radial spokes
- MeSH:
Adolescent;
Arm;
Axoneme;
Cilia;
Dyneins;
Female;
Humans;
Kartagener Syndrome*;
Membranes;
Microtubules
- From:Korean Journal of Otolaryngology - Head and Neck Surgery
1998;41(10):1331-1334
- CountryRepublic of Korea
- Language:Korean
-
Abstract:
Primary ciliary dyskinesia encompasses a heterogenous group of inherited condition characterized by clinical, functional, and ultrastructural features. The transmission electronmicroscopic findings of nasal cilia in a 14-year old girl with primary ciliary dyskinesia were studied. The ultrastructure of axonemes showed normal outer membrane, dynein arms, microtubules, and nexin links but partial lack of radial spokes. The nature of the defective spoke is not clear and further studies will be necessary to determine how the radial spokes and central sheath interact and coordinate ciliary movement.
