대한흉부영상의학회 Korean Society of Thoracic Radiology

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Weekly Chest CasesArchive of Old Cases

Case No : 503 Date 2007-06-18

  • Courtesy of Duk Sik Kang, MD. / Kyungpook National University Hospital, Daegu, Korea
  • Age/Sex 16 / F
  • Chief ComplaintChronic cough & sputum
  • Figure 1
  • Figure 2
  • Figure 3
  • Figure 4

Diagnosis With Brief Discussion

Diagnosis
Cystic fibrosis
Radiologic Findings
Chest radiograph shows patchy and nodular infiltration in the both upper lung zone. HRCT shows bronchiectasis with thickening of the bronchial wall, centrilobular nodules and patchy infiltration more in the upper lobes. Follow-up showed repeated aggravation and relapse of infection.
The sweat test revealed increased chloride level of 102mmol/L, which exceeds diagnostic criteria of cystic fibrosis(60mmol/L). Consequently, the diagnosis of cystic fibrosis was made.
Brief Review
Cystic fibrosis is a rare disease in oriental people. An autosomal recessive genetic defect in the structure of the CF transmembrane conductance regulator (CFTR) leads to abnormal chloride transport across epithelial membranes.
The mechanisms are not clear. But an abnormally low water content of airway mucus is at least partially responsible, resulting in decreased mucus clearance, mucous plugging of airways, and an increased incidence of bacterial airway infection. Upper lobes are more involved due to physiologically weak expectoration mechanism.
Radiologic findings are bronchiectasis with or without mucoid impaction, bronchial wall or peribronchial interstitial thickening and tree-in-bud appreance nodules. These findings are caused by secondary infection.
References
1. Cystic Fibrosis: CT Assessment of Lung Involvement in Children and Adults Radiology 1999; 213: 537.
2. Pulmonary cystic fibrosis in the adult: early and late radiologic findings with pathologic correlations Am. J. Roentgenol., Jun 1981; 136: 1131 - 1144.
3. Thoracic Imaging: Pulmonary and Cardiovascular Radiology W.Richard Webb p534 - 536
Keywords
Airway, Lung, Congenital, Bronchiectasis,

No. of Applicants : 55

▶ Correct Answer : 19/55,  34.5%
  • - Ondokuz Mayis University, Samsun Turkey Cetin Celenk
  • - China Medical University Hospital, Taiwan Jun-Jun Yeh
  • - Sinsegye Rad Clinic, Daegu, Korea Kim Gi Beom
  • - Dept of radiology, Inje University Ilsan Paik Hospital, Korea Bae Geun Oh
  • - Maimonides Medical Center; Brooklyn, N.Y., U.S.A. Naomi Twersky
  • - Aditya Imaging centre, Sayajigunj, Vadodara, Gujarat, India Vivek Patel
  • - Nour El-Islam Radiology Center Egypt .Alexandria Elsayed Mousa
  • - Registrar in Radiology, UHL NHS Trust, UK. A. Donuru
  • - Escorts heart institute new delhi, India Monika Aggarwal
  • - Hospital Sao Paulo, Brazil Israel Missrie
  • - NUH, Nottingham, UK Rohit Kochhar
  • - University of Colorado Health Sciences Center, Denver, CO, USA Seth Kligerman
  • - CHU Grenoble, France Bing Fabrice
  • - IRSA La Rochelle, France Jean-Luc BIGOT
  • - Osaka University, Japan Osamu Honda
  • - IRSA, La Rochelle, France Denis Chabassiere
  • - Annemasse, Polyclinique De Savoie, France Gay-Depassier Philippe
  • - Hospital Sotiria Athens, Greece Delaveri keith
  • - EKH-Berlin, Germany Michael Weber
▶ Correct Answer as Differential Diagnosis : 14/55,  25.5%
  • - CD Diagnostics, Sunder Lal Jain Hospital, New Delhi, India Sanjeev Kumar Vachher
  • - Dept. of Radiodiagnosis and Imaging, PGIMER, Chandigarh, India Ram Prakash Galwa
  • - Regional Imaging Riverina, Australia Rashid Hashmi
  • - Ruby Hall Clinic, Pune, India John Joseph
  • - Ruby Hall Clinic, Pune, India Nikhil Unune
  • - Hospital Sotiria, Athens, Greece Vasilios Tzilas
  • - Vital imaging centre, Mumbai, India Ganesh Agrawal
  • - Zulekha Hospital, Dubai, Australia Julie Arora
  • - Armidale Radiology, Australia Saurabh Khandelwal
  • - Annecy hospital, France Gilles Genin
  • - Ultracare, Coimbatore, India Debabrata Das
  • - Doctors Hospital, Nassau, Bahamas N.B.S.Mani
  • - Yongsan Hospital, College of Medicine Chung-Ang University, Korea Jae Seung Seo
  • - Nassau, Bahamas Trupti Dabholkar
▶ Semi-Correct Answer : 19/55,  34.5%
  • - KAUMS, Kashan, Iran Ebrahim Razi
  • - Yashodha Hospital, Hyderabad, Andhra Pradesh, India Satish Babu, Maddkuri
  • - Omega MRI Centre, Sadashiv Peth, Pune, India Abhimanyu Kelkar
  • - Mumbai, India Rahul B Deshmukh
  • - Konkuk University, College of Medicine, Korea Jeong Geun Yi
  • - Balabhai Nanavati hospital, mumbai, India Susheel Kumar
  • - Nirman Diagnostic Centre, Mumbai, India Padma Kannan
  • - Jiangsu Province Official Hospital, Nanjing, Jiangsu Province, China Dong Sheng Jin
  • - Mackay Memorial Hospital, Taipei, Taiwan Chia-Fu Tsai
  • - Makay Memorial Hospital, Taipei, Taiwan Sheng-Yao Yu
  • - MMH, Taipei Taiwan Xavier Wu
  • - SCTIMST, Trivandrum, India Jitender Saini
  • - Hangang Sacred Heart Hospital, Korea Eil Seong Lee
  • - Gyengsang National University Hospital, Korea Kim Ji Eun
  • - National Taiwan University Hospital, Taiwan Bang-Bin Chen
  • - Asan Medical Center, Korea Eun Jin Chae
  • - E-Da hospital, Taiwan Yu-Feng Wei
  • - Homs National Hospital, Homs - Syria Rami Abou Zalaf
  • - Max Hospital, New Delhi, India Vickrant Malhotra
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