RadioGraphics
In CF, a genetic defect governing epithelial chloride ion transport produces systemic disease. The pathologic and radiologic features are closely linked to the accumulation of viscous gland secretions within hollow organs and solid organ ducts. Intestinal obstruction may herald the diagnosis of CF in infants and young children. As the patient ages, pancreatic and hepatobiliary disease increases in prevalence. Pulmonary disease remains the principal determinant of morbidity and death in CF patients. However, the protean gastrointestinal expressions of CF have assumed increasing clinical importance as patient survival has dramatically improved. Consequently, an appreciation of the spectrum of gastrointestinal findings in CF is necessary as more young adults undergo diagnostic imaging studies.