대한흉부영상의학회 Korean Society of Thoracic Radiology

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Weekly Chest CasesCases by Disease Category

Case No : 435 Date 2006-02-27

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  • Courtesy of Hyun Ju Lee, MD. /
  • Age/Sex 15 / F
  • Chief ComplaintSputum
  • Figure 1
  • Figure 2
  • Figure 3
  • Figure 4
  • Figure 5
  • Figure 6

Diagnosis With Brief Discussion

Diagnosis
Immotile Cilia Syndrome
Radiologic Findings
Chest radiograph and chest CT shows diffuse bronchiectasis in both lungs. Minimal bronchiolitis is also seen.

On Waters view of paranasal sinus, total haziness of maxillary, esthmoid, and frontal sinuses suggesting pansinusitis.
Brief Review
Immotile cilia syndrome is a rare disorder in which there is an ultrastructural defect in the cilia in various cells. It is inherited in autosomal recessive manner with an estimated prevalence of 1 in 16,000. It affects the ciliated epithelium of the upper respiratory tract and therefore results in ineffective clearing of mucus.

Parents of a child with primary ciliary dyskinesia generally have no history of chronic lung disease. There is a 25% chance that another child is affected by the condition.

Cilia from the lung, nose, or elsewhere have a structure that is highly conservative and, moreover, resembles that of the sperm flagellum. The central part of the sperm tail, its axoneme, thus has the same well known 9 + 2 pattern as has a cilium, although it is about 50
References
1. Afzelius BA. Immotile cilia syndrome: past, present, and prospects for the future. Thorax. 1998;53:894-897

2. Pedersen H, Rebbe H. Absence of arms in the axoneme of immobile human spermatozoa. Biol Reprod.

1975;12:541-544

3. Afzelius BA, Eliasson R, Johnsen O, et al. Lack of dynein arms in immotile human spermatozoa. J Cell Biol.

1975;66:225-232.

4. Adams R, Churchill ED. Situs inversus, sinusitis and bronchiectasis. J Thorac Surg 1937;7:206-217.

5. Holmes LB, Blennerhasset JB, Austen KF. A reappraisal of Kartageners syndrome. Am J Med Sci.

1968;255:13-28.

6. Stanley P, McWilliam L, Greenstone M, et al. Efficacy of a saccharin test for the screening to detect abnormal

mucociliary clearance. Br J Dis Chest. 1984;78:62-65.

7. Jorissen M, Bertrand B. Ciliary dyskinesia in the nose and paranasal sinuses. Acta Otolaryngol Belge.

1997;51:353-366.
Keywords
Lung, Multiple organ, Congenital,

No. of Applicants : 59

▶ Correct Answer : 17/59,  28.8%
  • - National Taiwan University Hospital, Taiwan Kao-Lang Liu
  • - CHRA, Annecy France Bing Fabrice
  • - Yongdong Severance Hospital, Seoul, Korea Eun-Suk Cho
  • - Asan Medical Center, Seoul, Korea Hyun-Joo Kim
  • - Dong-A University Hospital, Korea Ki-Nam Lee
  • - Konkuk University Hospital, Seoul, Korea Jeong Geun Yi
  • - IRCCS St. Luca Hospital, Milano, Italy Filippo Casolo
  • - Marien Hospital, Germany Davis Chiramel
  • - Ewha Womans University MokDong Hospital, Korea Yookyung Kim
  • - Gwangmyoung Sungae Hospital, Korea Jiyong Rhee
  • - Clinic 1, Moscow, Russia Lepikhina Dasha
  • - Jinju Korea Hospital, Korea JI Hoon Park
  • - Jikei University of Medicine, Tokyo, Japan Shigeki Misumi
  • - Max Hospital, New Delhi, India Vickrant Malhotra
  • - Annecy Hospital, France Gilles Genin
  • - Ultracare , Coimbatore , India Debabrata Das
  • - KMC Manipal Nabil Sherif
▶ Correct Answer as Differential Diagnosis : 12/59,  20.3%
  • - Inje University Ilsan Paik Hospital, Korea Bae Geun Oh
  • - Pgimer, Chandigarg, India Ram Prakash Galwa
  • - Kashan Shahid Beheshti Hospital, Kashan, Iran Ebrahim Razi
  • - Apollo FirstMed Hospital, Chennai, India RG Gopinath
  • - Hangang Sacred Heart Hospital, Korea Eil Seong Lee
  • - University of Pennsylvania, USA Lisa Jones
  • - Radiologie Guiton, La Rochelle, France Denis Chabassiere
  • - Annemasse, Polyclinique de Savoie, France Gay-Depassier
  • - Homs National Hospital, Homs, Syria Rami Abou Zalaf
  • - Yonsei University Severance Hospital, Korea Yong Eun Chung
  • - Yonsei University Shinchon Severance Hospital, Korea Hye-Jeong Lee
  • - Monaldi Hospital, Naples, Italy Gaetano Rea
▶ Semi-Correct Answer : 19/59,  32.2%
  • - Shinsegye Rad Clinic, Korea Gi Beom Kim
  • - Hopital Calmette, Lille, France Toledano Manuel
  • - EPM and Fleury Medical Center, Sao Paulo, Brazil Gustavo Meirelles
  • - Ruby Hall Clinic, Pune, India John Joseph
  • - Doma Hospital, Damascus, Syria Mostafa Dakak
  • - Regional Imaging, Riverina, Australia Rashid Hashmi
  • - Seoul National University Hospital, Korea Kwang Nam Jin
  • - Korea University Hospital, Korea Seok Kyu Eom
  • - Ondokuz Mayis University, Samsun, Turkey Cetin Celenk
  • - Cabinet de Radiologie Guiton, La Rochelle, France Jean-Luc Bigot
  • - Diskapi Yildirim Beyazit Hospital, Ankara, Turkey Meric Tuzun
  • - Hopitaux HIS, Site Ixelles, Brussels, Belgium Emmanuel Agneessens
  • - Long Island Jewish Medical Center, New York, USA Pinar Karakas
  • - Chikuhou Rousai Hospital, Korea Kouei Uchida
  • - Kangnam St. Mary Hospital, Korea ChaeHun Lim
  • - Incheon Sarang Hospital, Korea Jung Hee Kim
  • - Dongguk University International Hospital,Ilsan, Korea Hee Seok Choi
  • - Samcheunpo Jeil Hospital, Korea Su Jin Kang
  • - China Medical University Hospital,Taiwan Jun-Jun Yeh
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