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KEY FACTS
Terminology
Imaging
Top Differential Diagnoses
Clinical Issues
Diagnostic Checklist
TERMINOLOGY
Abbreviations
- Cystic fibrosis (CF)
Synonyms
- Mucoviscidosis
Definitions
- Autosomal recessive disorder causing mutations of CF transmembrane conductance regulator (CFTR) gene, which regulates chloride transport
- Abnormally viscous secretions from exocrine glands (salivary and sweat glands, pancreas, large bowel, tracheobronchial tree)
- Multiorgan involvement, primarily lungs and pancreas
- Most common fatal hereditary disease in Caucasians
- Accounts for up to 25% of adult cases of bronchiectasis
- Autosomal recessive disorder causing mutations of CF transmembrane conductance regulator (CFTR) gene, which regulates chloride transport
IMAGING
General Features
Radiographic Findings
CT Findings
MR Findings
Angiographic Findings
Imaging Recommendations
DIFFERENTIAL DIAGNOSIS
PATHOLOGY
General Features
Microscopic Features
CLINICAL ISSUES
Presentation
Demographics
Natural History & Prognosis
Treatment
DIAGNOSTIC CHECKLIST
Consider
Image Interpretation Pearls
Selected References
- Flors L et al: Hyperpolarized gas magnetic resonance lung imaging in children and young adults. J Thorac Imaging. 31(5):285-95, 2016
- Milliron B et al: Bronchiectasis: mechanisms and imaging clues of associated common and uncommon diseases. Radiographics. 35(4):1011-30, 2015
- Wielpütz MO et al: Magnetic resonance imaging of cystic fibrosis lung disease. J Thorac Imaging. 28(3):151-9, 2013
- O'Connell OJ et al: Radiologic imaging in cystic fibrosis: cumulative effective dose and changing trends over 2 decades. Chest. 141(6):1575-83, 2012
- Eichinger M et al: Computed tomography and magnetic resonance imaging in cystic fibrosis lung disease. J Magn Reson Imaging. 32(6):1370-8, 2010
- Kang J et al: Manifestations of systemic diseases on thoracic imaging. Curr Probl Diagn Radiol. 39(6):247-61, 2010
- Robinson TE: Imaging of the chest in cystic fibrosis. Clin Chest Med. 28(2):405-21, 2007
- de Jong PA et al: Progression of lung disease on computed tomography and pulmonary function tests in children and adults with cystic fibrosis. Thorax. 61(1):80-5, 2006
- Flume PA et al: Massive hemoptysis in cystic fibrosis. Chest. 128(2):729-38, 2005
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Tables
KEY FACTS
Terminology
Imaging
Top Differential Diagnoses
Clinical Issues
Diagnostic Checklist
TERMINOLOGY
Abbreviations
- Cystic fibrosis (CF)
Synonyms
- Mucoviscidosis
Definitions
- Autosomal recessive disorder causing mutations of CF transmembrane conductance regulator (CFTR) gene, which regulates chloride transport
- Abnormally viscous secretions from exocrine glands (salivary and sweat glands, pancreas, large bowel, tracheobronchial tree)
- Multiorgan involvement, primarily lungs and pancreas
- Most common fatal hereditary disease in Caucasians
- Accounts for up to 25% of adult cases of bronchiectasis
- Autosomal recessive disorder causing mutations of CF transmembrane conductance regulator (CFTR) gene, which regulates chloride transport
IMAGING
General Features
Radiographic Findings
CT Findings
MR Findings
Angiographic Findings
Imaging Recommendations
DIFFERENTIAL DIAGNOSIS
PATHOLOGY
General Features
Microscopic Features
CLINICAL ISSUES
Presentation
Demographics
Natural History & Prognosis
Treatment
DIAGNOSTIC CHECKLIST
Consider
Image Interpretation Pearls
Selected References
- Flors L et al: Hyperpolarized gas magnetic resonance lung imaging in children and young adults. J Thorac Imaging. 31(5):285-95, 2016
- Milliron B et al: Bronchiectasis: mechanisms and imaging clues of associated common and uncommon diseases. Radiographics. 35(4):1011-30, 2015
- Wielpütz MO et al: Magnetic resonance imaging of cystic fibrosis lung disease. J Thorac Imaging. 28(3):151-9, 2013
- O'Connell OJ et al: Radiologic imaging in cystic fibrosis: cumulative effective dose and changing trends over 2 decades. Chest. 141(6):1575-83, 2012
- Eichinger M et al: Computed tomography and magnetic resonance imaging in cystic fibrosis lung disease. J Magn Reson Imaging. 32(6):1370-8, 2010
- Kang J et al: Manifestations of systemic diseases on thoracic imaging. Curr Probl Diagn Radiol. 39(6):247-61, 2010
- Robinson TE: Imaging of the chest in cystic fibrosis. Clin Chest Med. 28(2):405-21, 2007
- de Jong PA et al: Progression of lung disease on computed tomography and pulmonary function tests in children and adults with cystic fibrosis. Thorax. 61(1):80-5, 2006
- Flume PA et al: Massive hemoptysis in cystic fibrosis. Chest. 128(2):729-38, 2005
