대한흉부영상의학회 Weekly Chest Cases

Case No : 897 Date 2015-01-05

Courtesy of Hyoun Cho, Tae Sung Kim / Samsung Medical Center
Age/Sex 22 / F
Chief ComplaintCough and sputum for two days. Past history: Pneumonia (21years ago), Pulmonary TB (5years ago), Pulmonary NTM disease (1 year ago) : M.abscessus

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Diagnosis With Brief Discussion

Diagnosis: Cystic fibrosis
Radiologic Findings: On chest radiography, there are bronchiectasis and cavity lesions with fibrotic change in the both upper lung zones, located within 2-3cm of the pleura.
Chest CT demonstrates diffuse cystic bronchiectasis with thickening of the bronchial wall, mucus plugging and centrilobular nodules predominantly in both upper lung zones. The pattern of these lesions has bilateral symmetric distribution and upper lobe predominance.
Gene mutation analysis was done and CFTR gene mutation was detected. So, familiar study was also performed, her mother and brother were heterozygous carrier.
Brief Review: Cystic fibrosis is caused by cystic fibrosis transmembrane conductance regulator (CFTR) gene mutations which causes abnormal mucus formation in the airway, leading to luminal obstruction and recurrent bronchial inflammation and infection. Cystic fibrosis is the most common autosomal recessive disease in Caucasian, but it is very rare in nonwhites.
It presents obstructive pulmonary disease which manifest as recurrent respiratory infection associated with productive cough, wheezing, and dyspnea. The basic abnormality consists of abnormal secretions from various exocrine glands including the salivary and sweat glands, pancreas, large bowel and tracheobronchial tree. The treatment includes antibiotics, physiotherapy, and replacement of pancreatic enzyme for conservative purpose.
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 findings of cystic fibrosis include cylindrical or cystic forms of bronchiectasis involving more severe in the upper lobes. Consolidation or atelectasis is noted in 80% of patient. Cystic or bullous lesions are frequently presents in the subpleural regions of the upper lobes. Branching or nodular centrilobular opacity, so called tree-in-bud pattern, may be an early sign of disease. Air trapping on expiratory CT is also common.
Please refer to
References: 1. Muller NL, Silva CS. Imaging of the Chest. Saunders 2008: Vol2; 1039-1044
2. P. Farrell et al., Guidelines for Diagnosis of Cystic Fibrosis in Newborns through Older Adults: Cystic Fibrosis Foundation Consensus Report. J pediatr 2008;153:S4-S14
Keywords: Airway, Lung, Non-infectious inflammation,

No. of Applicants : 72

▶ Correct Answer : 31/72, 43.1%

- The University of Tokyo Hospital , Japan Akifumi Hagiwara
- university of montreal , Canada Andrei Gorgos I
- Niigata City General Hospital , Japan Takao Kiguchi
- SNUH , Korea (South) Eui Jin Hwang
- The University of Tokyo Hospital , Japan Toshihiro Furuta
- Diskapi Yildirim Beyazit Hospital, Ankara , Turkey Meric Tuzun
- James Paget Hospital , U.K , United Kingdom nabil mahmood
- Otsu Red Cross Hospital , Japan Hirotsugu Nakai
- University of Tsukuba Hospital , Japan Sodai Hoshiai
- The University of Tokyo Hospital , Japan Takeyuki Watadani
- Northern Yokohama Showa university , Japan Kota Watanabe
- Niigata University , Japan Atsushi Uehara
- Shiga University of Medical Science , Japan Akitoshi Inoue
- Beaulieu clinic Geneva , Switzerland gilles GENIN
- Hanyang University Guri Hospital , Korea (South) Seung Hyun Kim
- Ondokuz Mayis University , Turkey Cetin Celenk
- Ishikawa Matto Central Hospital , Japan Manabu Akimoto
- IRSA La Rochelle , France Denis Chabassiere
- National Center hospital of Neurology and Psychiatry , Japan Kaoru Sumida
- Nagasaki University Hospital, Department of Pathology , Japan TOMONORI TANAKA
- CLINIQUE STE CLOTILDE , Reunion patrick MASCAREL
- All India Institute of medical sciences , India Justin Moses
- CLCC Rennes , France nicolas gautier
- NASA SCANS , India RAKESH BHATIA
- ZIGONG TCM HOSPITAL OF CHINA , China Cao Cunyou
- Ibaraki-gazou-shindan , Japan Shoichi Katoh
- Wuhan Union Hospital , China Qiguang Cheng
- IRSA LA ROCHELLE , France, Metropolitan BIGOT
- jaslok hospital & research centre mumbai , India JAINENDRA JAIN
- Chia Nan University of Pharmacy and Science ,Taiwan, R.O.C. , Taiwan Jun Jun Yeh
- DAYA General Hospital,Thrissur,Kerala , India Raveendran TK

▶ Correct Answer as Differential Diagnosis : 18/72, 25.0%

- McGill University Health Center , Canada Alexandre Semionov
- Kmc chennai , India chellaraja c
- CHRU Lille , France Paul Lebert
- University of British Columbia , Canada Amr Ajlan
- �stanbul , Turkey Ayhan Yilmaz
- GHICL , France manuel toledano
- Oita University, Faculty of Medicine , Japan Fumito Okada
- Seirei Hamamatsu General Hospital , Japan Kenichi Mizuki
- Asan medical center , Korea (South) Sang Young Oh
- Asan Medical Center, Ulsan University , Korea (South) Mi Young Kim
- Asan Medical Center , Korea (South) Eunsol Lee
- Gifu Central Hospital , Japan Haruo Watanabe
- Kizawa Memorial Hospital , Japan Yo Kaneko
- Onomichi municipal hospital , Japan Hirofumi Mifune
- Okayama University Hospital , Japan Toshiyuki Komaki
- Onomichi Municipal Hospital , Japan Yoshihisa Masaoka
- Showa University Fujigaoka Hospital , Japan NOBUYUKI TAKEYAMA
- Medicheck health care , Korea (South) Chae Lim

대한흉부영상의학회 Korean Society of Thoracic Radiology

Archive of Old Cases

Case No : 897 Date 2015-01-05

Discussion

No. of Applicants : 72