A Case of Kartagener's Syndrome with Various Ultrastructural Defects.

Lee, Sung Ho; Park, Jung Ho; Jang, Ho Sik; Kim, Hyun Su; Kang, Kyeong Woo; Kim, Ho Chul; Kwon, Kun Young

doi:2002.53.4.457

Tuberc Respir Dis > Volume 53(4); 2002 > Article

Case Report

Tuberculosis and Respiratory Diseases 2002;53(4):457-462.
DOI: https://doi.org/10.4046/trd.2002.53.4.457 Published online October 1, 2002.

A Case of Kartagener's Syndrome with Various Ultrastructural Defects.

Sung Ho Lee, Jung Ho Park, Ho Sik Jang, Hyun Su Kim, Kyeong Woo Kang, Ho Chul Kim, Kun Young Kwon

¹Division of pulmonology, Department of Medicine, Sungkyunkwan University School of Medicine, Masan Samsung Medical Center, Masan, Korea. kangkw@samsung.co.kr
²Department of Pathology, Keimyung University, Taegu, Korea.

Abstract

Kartagener's syndrome is an inherited condition characterized by triad of chronic paranasal sinusitis, situs inversus, and bronchiectasis. Since 1976, Afzelius found a lack of dynein arm in immotile spermatozoa by electron microscopy, numerous recent studies have focused on the ultrastructural defect in the cilia and reported that the variety type of ultrastructural defect in immotile cilia syndrome. We report a female patient who had the Kartagener's triad with rare multiple ultrastructural defect of cilia in one patient. The electron microscopic examination showed partial dynein arm defect, loss of radial spoke, microtubular transposition, and giant cilia.

Key Words: Kartagener's syndrome, Immotile cilia, Dyskinetic cilia