대한흉부영상의학회 Korean Society of Thoracic Radiology

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대한흉부영상의학회 Weekly Case 검색
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Weekly Chest CasesArchive of Old Cases

Case No : 238 Date 2002-05-18

  • Courtesy of Choong-Ki Park, M.D. / Hanyang University Kuir Hospital, Korea
  • Age/Sex 16 / M
  • Chief ComplaintCough and dyspnea
  • Figure 1
  • Figure 2
  • Figure 3

Diagnosis With Brief Discussion

Diagnosis
Cystic Fibrosis with Combined Infection and Pneumothorax
Radiologic Findings
A posteroanterior chest radiograph shows pneumothorax in the left hemithorax and marked bronchial wall thickening with extensive bronchiectasis. HRCT scans show bronchiectasis, bronchial wall thickening, mucus filled bronchiectasis, emphysematous change. OMU CT reveals chronic pansinusitis.

Diagnosis of cystic fibrosis was made by sweat iontophoresis (elevated sweat chloride levels: 97 mEq/L and 99 mEq/L). H. influenza and S. aureus were isolated on sputum culture.
Brief Review
Cystic fibrosis (CF) is a hereditary disease of autosomal recessive transmission. The basic abnormality consists of abnormal secretions from variable exocrine glands including the salivary, sweat glands, pancreas (90%), large bowel and tracheobronchial tree. Infants who died of CF shows normal lung, by contrast, older patients who die of the disease invariably shows pulmonary changes, including airway mucus plugging, pneumonia, bronchiolitis obliterans, bronchiectasis, atelectasis and overinflation (1).
The incidence is as high as 1 per 500 in Scotland, 1 per 2000-3500 in whites, 1 per 90,000 in Asian. Asians who have CF may have a more severe clinical course than whate controls (1). As a result of improved medical care, life expectancy has increased. Whereas the survival rate older than 17 years used to be about 5 % by the 1970s, for infants born today in the UK, the predicted mean life expectancy is 40 years.
Chest radiography reveals extensive obstruction of medium-sized and small airways of the lungs, hyperinflation, cylindrical and cystic bronchiectasis, nodular and fingerlike shadows of mucoid impaction. CT can reveal pathologic changes not visible on conventional chest radiograms, particularly mucoid impaction, detailed scoring of bronchiectasis, peribronchial thickening, mucous plugging, atelectasis, consolidation, cysts, bullae and emphysematous change. Paranasal sinus opacification due to chronic sinusitis and polyposis is almost universal in patients with CF (1).
The most common organisms of combined lung infection are P. aeruginosa, S aureus, H. influenza, B. cepacia. Allergic bronchopulmonary aspergillosis occurs in 5-10 % (serum precipitins against A. Fumigatus detected 51 % of patients) (1).
References
1. Fraser RS, M?ler NL, Colman N, Par?JAP. Fraser and Pare뭩 diagnosis of the chest. 4th ed. Saunders 1999: 2298- 2315.
2. Moon HR, Ko TS, Ko YY, Choi JH, Kim YC. Cystic fibrosis--a case presented with recurrent bronchiolitis in infancy in a Korean male infant. Journal of Korean Medical Science 1988, 3(4) 157-162
Keywords
Airway, Non-infectious inflammation, Hereditary,

No. of Applicants : 25

▶ Correct Answer : 14/25,  56.0%
  • - Bhaktivedanta Hospital, Mumbai, India Sunil S. Jaisingh
  • - CHU Nancy-Brabois, France Denis Regent
  • - Dong-A University Hospital, Korea Chan-Sung Kim
  • - Dong-Ulsan Imaging Clinic, Korea Hoon-Hwa, Kim
  • - Gwangmoung Seongae Hospital, Korea Jiyong Rhee
  • - H "La Paz", Madrid. Spain FJ Gomez de Terreros
  • - Harasanshin hospital, Fukuoka, Japan Shunya Sunami
  • - Katterstraat Tielt, Belgium Liong DJOA
  • - Ohio State University, Columbus, Ohio, USA Sumit Seth
  • - Ospedale di Jesi, Italy Giancarlo Passarini
  • - S.Orsola-Malpighi Hospital, Bologna, Italy M. Zompatori
  • - Stedelijk OLV Ziekenhuis Mechelen, Belgium Ivan Pilate
  • - Sungji Hospital, Korea Jung Hee Kim
  • - Taipei Municipal Jen-Ai Hospital, Taiwan Hui-Ju Tsai
▶ Semi-Correct Answer : 7/25,  28.0%
  • - Choong-ju Hospital Konkuk University, Korea Chang Hee Lee
  • - Daejeon Sun Hospital, Korea Dong Jip Na
  • - Dong-Eui Medical Center, Korea Ha-Jong Lee
  • - Korea Cancer Center Hospital, Korea Donghee Park
  • - Samsung Medical Center, Korea Young Cheol Yoon
  • - Xray Clinic, Pune, India M D Rahalkar
  • - Myun Joon Yang
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