대한흉부영상의학회 Korean Society of Thoracic Radiology

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Weekly Chest CasesCases by Disease Category

Case No : 1255 Date 2021-11-09

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  • Courtesy of Seok Young Koh / Seoul National University Hospital
  • Age/Sex 19 / F
  • Chief ComplaintLong standing sputum, since birth
  • Figure 1
  • Figure 2
  • Figure 3
  • Figure 4
  • Figure 5

Diagnosis With Brief Discussion

Diagnosis
Primary ciliary dyskinesia
Radiologic Findings
Primary ciliary dyskinesia is a chronic lung disease caused by defects in ciliary structure. It has heterogeneous group of autosomal recessive trait with a prevalence of approximately 1/12,000-1/60,000. Individuals with primary ciliary dyskinesia have abnormal or absent ciliary motion and cannot move mucus out of the respiratory passages leading to airway damage and increasing the risk for the development of bronchiectasis. In 50% of patients, situs inversus can be associated and Kartagener's syndrome, a subset of primary ciliary dyskinesia, includes triad of situs inversus, bronchiectasis, and sinusitis. Common symptoms of primary ciliary dyskinesia are chronic rhinitis, recurrent or chronic sinusitis, recurrent or chronic bronchitis, bronchiectasis, olfactory impairment, and infertility.
The diagnosis is complex and based on suggestive clinical findings, pathologic study for ultrastructural ciliary defect, nasal NO measurement and/or genetic anaylsis. CT typically shows varicose bronchiectasis, a chronic volume loss, a tree in bud pattern, and mucous plugging. Later, a predominance of bronchiectasis in the middle and lower lung lobes is typical
The treatment includes airway clearance therapy, antibiotics, bronchodilators, steroids and mucus thinners (mucolytics). Lung transplantation is an option for severe, advanced lung disease.
Brief Review
References
1. Nadel HR, Stringer DA, Turner JA, Sturgess JM. The immotile cilia syndrome: radiological manifestations. Radiology. 1985;154:651-5.
2. High-Resolution CT of patients with primary ciliary dyskinesia. AJR. 2007;188:1232-8.
3. Computed tomography in adult patients with primary ciliary dyskinesia: Typical imaging findings. PLoS One. 2018 Feb 6;13(2):e0191457.
Keywords

No. of Applicants : 104

▶ Correct Answer : 64/104,  61.5%
  • - Ishikawa Matto Central Hospital , Japan MANABU AKIMOTO
  • - , France ARTHUR BRANCHU
  • - Saitama-Sekishinkai Hosptal , Japan MIHOKO YAMAZAKI
  • - , Japan HIROAKI ARAKAWA
  • - Korea University Guro Hospital , Korea (South) SEOKYOUNG LEE
  • - , Japan JUN KANZAWA
  • - ZIGONG TCM HOSPITAL OF CHINA , China CAO CUN YOU
  • - Kinki University Faculty of Medicine, , Japan MITSURU MATSUKI
  • - Showa University Fujigaoka Hospital , Japan KYOKO NAGAI
  • - Ajou University Hospital , Korea (South) SUNG HYUN AN
  • - Mayo Clinic , United States AKITOSHI INOUE
  • - Other , Korea (South) HYEJIN YANG
  • - Affilitated Hospital of Jining Medical college , China JIANG SHENG HUA
  • - , United Kingdom SAMSON KADE
  • - Other , Korea (South) MINGI SHIN
  • - , Italy PAOLO BALDASSARI
  • - , France CHARLES DAMIEN
  • - Tiger Gate Hospital , Japan SHIN-ICHI CHO
  • - Chungbuk National University Hospital , Korea (South) JIYUN KANG
  • - Vita Hospital , Brazil DIOGO LAGO PINHEIRO
  • - Korea University Ansan Hospital , Korea (South) BYUK GYUNG CHOI
  • - Silchar medical college , India MD IMDADUL ISLAM
  • - Other , Korea (South) SEONGSU KANG
  • - , Turkey MEHMET DURAN
  • - zhongshai hospital, fudan university , China FENG HUA
  • - , China FANG YING GEN
  • - university of montreal , Canada Andrei Bogdan Gorgos I
  • - Osaka University , Japan AKINORI HATA
  • - Ajou University Hospital , Korea (South) HAEIN LEE
  • - Shimada General Medical Center , Japan HAYATO NOZAWA
  • - National Center for Global Health and Medicine , Japan MASATOSHI HOTTA
  • - , Korea (South) HYEWON CHOI
  • - The University of Tokyo Hospital , Japan TOSHIHIRO FURUTA
  • - Ajou University Hospital , Korea (South) MINJI KIM
  • - Gifu University Hospital , Japan Yo Kaneko
  • - McGill , Canada MUTAZ ADNAN KHAIRO
  • - Kyoto University Hospital , Japan YASUHISA KURATA
  • - , Korea (South) YOU NA KIM
  • - , Korea (South) EUNJIN LEE
  • - Mallinckrodt Institute of Radiology , United States NAGANATHAN MANI
  • - Chungbuk National University Hospital , Korea (South) MIHYEON PARK
  • - Other , Korea (South) MINSU KIM
  • - University of Tsukuba, Dept of Radiology , Japan MANABU MINAMI
  • - ALHAYAH SCAN RADIOLOGY CENTER , Egypt ELSAYED ELSAYED MOUSA
  • - Centre Hospitalier Regional de Lanaudiere , Canada MATTHIEU STORME
  • - Avrasya Hospital/ISTANBUL , Turkey MURAT ULUSOY
  • - Dokkyo Medical University , Japan HIROAKI ARAKAWA
  • - Columbia asia hospital Hebbal ,Bengaluru , India PRAVIN KUMAR M
  • - Osmania Medical College, Hyderabad , India PURUSHOTHAMA RAO TUMMALA
  • - Kyoto University Hospital , Japan SATOSHI IKEDA
  • - Senior Resident , India ANIL SHARMA
  • - Samsung Medical Center , Korea (South) SUHO KIM
  • - University of Tsukuba Hospital , Japan SODAI HOSHIAI
  • - Toranomon Hospital , Japan KAORU SUMIDA
  • - Chonbuk National University Hospital , Korea (South) DONG HAN SHIN
  • - Ichinomiya Nishi Hospital , Japan Takao Kiguchi
  • - , Japan KAZUMA TERAUCHI
  • - Chonnam National University Hospital , Korea (South) SEUNG WAN KANG
  • - Rasoul Akram generel hospital of Iran university of medical science , Iran MOHAMAD REZA AYOUBPOUR
  • - Govt. Kilpauk Medical College, Chennai , India VIGNESH K
  • - , Japan YUKI HAYASHI
  • - Diagnose.me (BV) / Royal Perth Hospital , Australia YURANGA WEERAKKODY
  • - the first affiliatited hospital of nanjing medical univercity , China HAI XU
  • - Jiangsu province hospital , China WANGJIAN ZHA
▶ Correct Answer as Differential Diagnosis : 12/104,  11.5%
  • - McGill University Health Center , Canada ALEXANDRE SEMIONOV
  • - Osaka Red Cross Hospital , Japan YUSUKE UTSUNOMIYA
  • - Chonbuk National University Hospital , Korea (South) JUNGHWAN KIM
  • - Yeungnam University Medical Center , Korea (South) JONGSOO PARK
  • - AIMS , India NITHYA HARIDAS MALIYAM
  • - Korea University Guro Hospital , Korea (South) HYEJIN PARK
  • - Seoul Veterans Hospital , Korea (South) SEONG WON JANG
  • - Other , Korea (South) KYU-CHONG LEE
  • - Chonbuk National University Hospital , Korea (South) MINJEE KIM
  • - Chonbuk National University Hospital , Korea (South) SEOYUN CHOI
  • - The University of Tokyo Hospital , Japan HIROSHI TAKUMIDA
  • - , Korea (South) WONJU HONG
▶ Semi-Correct Answer : 11/104,  10.6%
  • - Niigata University , Japan ATSUSHI UEHARA
  • - CHCB , France LE GUEN RAPHAEL
  • - National Center of Neurology and Psychiatry , Japan MOTO NAKAYA
  • - Ichinomiya Nishi Hospital , Japan CHIHIRO KIGUCHI
  • - Ajou University Hospital , Korea (South) KYUNGMIN LEE
  • - , Korea (South) JIN YOUNG LEE
  • - HHS , Canada S LEE
  • - The University of Tokyo Hospital , Japan RYO KUROKAWA
  • - Nishida Hospital , Japan SHOJI OKUDA
  • - College of Medicine, CHA University , Korea (South) PARK JONG WON
  • - algeria , Algeria ZAKARIA CHERAGA
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Current Editor : Sang Young Oh, M.D., Ph.D Email : sangyoung.oh@gmail.com

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