A Case of Primary Ciliary Dyskinesia

Kang, Sung Hoon; Kim, Jae Hyung; Kim, Hyunjung; Kim, Tae Hoon

doi:2017.24.2.118

J Rhinol > Volume 24(2); 2017

Case Report

Journal of Rhinology 2017;24(2):118-122.
Published online November 30, 2017.
DOI: https://doi.org/10.18787/jr.2017.24.2.118

A Case of Primary Ciliary Dyskinesia

Sung Hoon Kang, Jae Hyung Kim, Hyunjung Kim, Tae Hoon Kim

Department of Otorhinolaryngology-Head and Neck Surgery, Korea University College of Medicine, Seoul, Korea. doctorth@korea.ac.kr

원발성 섬모운동 이상증 1례

강성훈, 김재형, 김현정, 김태훈

고려대학교 의과대학 이비인후-두경부외과학교실

Abstract

Primary ciliary dyskinesia is a disease characterized by unexplained neonatal respiratory distress, otitis media, chronic sinusitis, and chronic bronchiectasis. In approximately half of cases, situs inversus totalis or other laterality defects are found. The incidence ranges from 1 in 4,000 to 1 in 40,000 live births. Early diagnosis is important and the disease is finally confirmed by electron microscopic biopsy. The treatment of primary ciliary dyskinesia is based on appropriate procedures. We present a rare case of a 20-year-old male with bronchiectasis, chronic otitis media and chronic sinusitis.

Key Words: Primary ciliary dyskinesia;Electron microscope;Ultrastructure