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DOI: 10.1148/rg.263055101
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RadioGraphics 2006;26:679-690
© RSNA, 2006


EDUCATION EXHIBIT

Review of the Abdominal Manifestations of Cystic Fibrosis in the Adult Patient1

Michael B. Robertson, MD, Kyuran A. Choe, MD and Patricia M. Joseph, MD

1 From the Departments of Radiology (M.B.R., K.A.C.) and Internal Medicine (P.M.J.), University of Cincinnati, 234 Goodman St, ML 0761, Cincinnati, OH 45267-0761. Presented as an education exhibit at the 2004 RSNA Annual Meeting. Received April 25, 2005; revision requested May 24 and received July 15; accepted July 20. All authors have no financial relationships to disclose. Address correspondence to K.A.C. (e-mail: choeka{at}email.uc.edu).

Cystic fibrosis is a common inherited fatal disease. As the life expectancy of affected individuals continues to increase with advances in disease management, this disease is no longer limited to the pediatric population. Currently, 40% of patients with cystic fibrosis are adults. In addition, patients may not present until adulthood and frequently have extrapulmonary symptoms. Abdominal manifestations are common and affect multiple organ systems. Hepatobiliary manifestations include fatty infiltration of the liver, gallbladder abnormalities, bile duct abnormalities, focal biliary fibrosis, and multinodular cirrhosis. Manifestations in the pancreas include acute pancreatitis, fatty replacement, calcifications, cysts, duct abnormalities, and carcinoma. Gastrointestinal manifestations include gastroesophageal reflux, peptic ulceration of the gastric and duodenal mucosa, distal intestinal obstruction syndrome, intussusception, appendicitis, fibrosing colonopathy, pneumatosis intestinalis, rectal mucosal prolapse, malignancies, and pseudomembranous colitis. Renal manifestations include nephrolithiasis, as well as secondary renal complications such as interstitial nephritis due to antibiotic therapy and amyloidosis. Awareness of these manifestations is important to successfully guide management of cystic fibrosis in adult patients.

© RSNA, 2006




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