Recessively inherited disorder of epithelial chloride transport caused by mutation of CF transmembrane conductance regulator (CFTR) gene
Pulmonary manifestations are primary cause of morbidity and mortality
CF increasingly seen to affect GI tract due to improved life expectancy
IMAGING
General Features
CT Findings
MR Findings
Ultrasonographic Findings
Other Abdominal Findings
DIFFERENTIAL DIAGNOSIS
PATHOLOGY
General Features
Lab
CLINICAL ISSUES
Presentation
Demographics
Natural History & Prognosis
Treatment
Selected References
Dana J et al: Hepatic manifestations of cystic fibrosis. Curr Opin Gastroenterol. 36(3):192-8, 2020
Lavelle LP et al: Cystic Fibrosis below the Diaphragm: abdominal Findings in Adult Patients. Radiographics. 35(3):680-95, 2015
Keyzer C et al: Cystic fibrosis: unenhanced CT description of the appendix in asymptomatic adults. AJR Am J Roentgenol. 202(4):759-64, 2014
Bilgin M et al: Ultrasonography and magnetic resonance imaging findings of abdominal complications of cystic fibrosis. Eur Rev Med Pharmacol Sci. 16 Suppl 4:48-51, 2012
Tümmler B et al: Transient correction of the basic defect in sweat glands in an individual with cystic fibrosis carrying the complex CFTR allele F508del-R553Q. Thorax. 64(2):179-80, 2009
Haber HP: Cystic fibrosis in children and young adults: findings on routine abdominal sonography. AJR Am J Roentgenol. 189(1):89-99, 2007
Robertson MB et al: Review of the abdominal manifestations of cystic fibrosis in the adult patient. Radiographics. 26(3):679-90, 2006
Taylor CJ et al: The pancreas in cystic fibrosis. Paediatr Respir Rev. 3(1):77-81, 2002
King LJ et al: Hepatobiliary and pancreatic manifestations of cystic fibrosis: MR imaging appearances. Radiographics. 20(3):767-77, 2000
Soyer P et al: Cystic fibrosis in adolescents and adults: fatty replacement of the pancreas--CT evaluation and functional correlation. Radiology. 210(3):611-5, 1999
Related Anatomy
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References
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KEY FACTS
Terminology
Imaging
Top Differential Diagnoses
Clinical Issues
TERMINOLOGY
Abbreviations
Cystic fibrosis (CF)
Synonyms
Mucoviscidosis
Definitions
Recessively inherited disorder of epithelial chloride transport caused by mutation of CF transmembrane conductance regulator (CFTR) gene
Pulmonary manifestations are primary cause of morbidity and mortality
CF increasingly seen to affect GI tract due to improved life expectancy
IMAGING
General Features
CT Findings
MR Findings
Ultrasonographic Findings
Other Abdominal Findings
DIFFERENTIAL DIAGNOSIS
PATHOLOGY
General Features
Lab
CLINICAL ISSUES
Presentation
Demographics
Natural History & Prognosis
Treatment
Selected References
Dana J et al: Hepatic manifestations of cystic fibrosis. Curr Opin Gastroenterol. 36(3):192-8, 2020
Lavelle LP et al: Cystic Fibrosis below the Diaphragm: abdominal Findings in Adult Patients. Radiographics. 35(3):680-95, 2015
Keyzer C et al: Cystic fibrosis: unenhanced CT description of the appendix in asymptomatic adults. AJR Am J Roentgenol. 202(4):759-64, 2014
Bilgin M et al: Ultrasonography and magnetic resonance imaging findings of abdominal complications of cystic fibrosis. Eur Rev Med Pharmacol Sci. 16 Suppl 4:48-51, 2012
Tümmler B et al: Transient correction of the basic defect in sweat glands in an individual with cystic fibrosis carrying the complex CFTR allele F508del-R553Q. Thorax. 64(2):179-80, 2009
Haber HP: Cystic fibrosis in children and young adults: findings on routine abdominal sonography. AJR Am J Roentgenol. 189(1):89-99, 2007
Robertson MB et al: Review of the abdominal manifestations of cystic fibrosis in the adult patient. Radiographics. 26(3):679-90, 2006
Taylor CJ et al: The pancreas in cystic fibrosis. Paediatr Respir Rev. 3(1):77-81, 2002
King LJ et al: Hepatobiliary and pancreatic manifestations of cystic fibrosis: MR imaging appearances. Radiographics. 20(3):767-77, 2000
Soyer P et al: Cystic fibrosis in adolescents and adults: fatty replacement of the pancreas--CT evaluation and functional correlation. Radiology. 210(3):611-5, 1999
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