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Pancreas sonography was performed on 171 patients with cystic fibrosis. The pancreas was visualized in 138 patients (81%) and could not be visualized in 33. 125 patients (90%) had abnormal echogenicity of the pancreas. Cysts were found in 5 patients. Two patients showed a dilatation of the pancreatic duct. The pancreas was abnormal in 98% of all patients aged more than 6 years.
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OBJECTIVE: Sonography of the liver, biliary system, and pancreas in adult patients with cystic fibrosis is by far less systematically documented than in pediatric patients with cystic fibrosis. In this prospective study, duplex sonographic findings of the liver, biliary system, and pancreas in adult patients with cystic fibrosis were compared with those of healthy control subjects. METHODS: Seventy-two consecutive patients with cystic fibrosis and 60 healthy control subjects were examined by high-resolution sonography. The incidence of perihepatic lymphadenopathy, the hepatic echo pattern, the detection rate of liver tumors, the flow patterns in the hepatic and portal veins, and pathologic gallbladder and pancreas findings were recorded. Additionally, cholestasis-indicating enzyme levels (gamma-glutamyl transpeptidase and alkaline phosphatase), liver function test results (alanine aminotransferase and aspartate aminotransferase levels), and amylase and lipase levels were recorded as well. RESULTS: Patients with cystic fibrosis, when compared with healthy subjects on sonographic examination, had a higher incidence of microgallbladder (25% versus 0%) and cystic lesions of the pancreas (18% versus 0%). The number of abnormal echo patterns of the liver was increased (46% versus 15%), with a higher incidence of a nontriphasic flow pattern in the right hepatic vein. The differences proved to be statistically significant (P < .05). CONCLUSIONS: Typical sonographic findings in adult patients with cystic fibrosis are a microgallbladder and small cystic lesions of the pancreas. Pathologic findings of the liver can be shown by B-mode and duplex sonography, but the resulting patterns are less characteristic.
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The computer tomographic appearances of atrophic and lipomatous degeneration of the pancreas in cystic pancreatic fibrosis are described. CT exploration of the pancreas is recommended, particularly in differential diagnostic aspects of cystic fibrosis.
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Abdominal ultrasound imaging was performed in 50 children. Thirty nine were proved to have cystic fibrosis while 11 had respiratory infections and thus acted as controls. The pancreas was abnormal in 75% of cystic fibrosis patients aged under 5 years and in 95% over 5 years. In cystic fibrosis patients over 5 years old who had malabsorption, the pancreas was abnormal in 100%. Abnormalities of the liver parenchyma were found in 23%, of the gallbladder in 24%, and splenomegaly in 8% of the cystic fibrosis patients.
Abdominal ultrasonography was performed on 35 adult patients with proven cystic fibrosis. Thirty-three patients showed pancreatic abnormalities. The most marked ultrasonographic features were increased parenchymal echogenicity, atrophy, non-visualisation of the duct and cyst formation. There was no correlation between these features and the severity of the pulmonary disease, the age of the patient, weight or glucose intolerance. Abnormalities of the biliary tract were demonstrated in nine (26%) patients and were associated with poor nutritional status. Multivariate analysis revealed a significant association between the following: hepatomegaly, increased liver echogenicity, splenomegaly, biliary disease; secondly, between lung function and serum albumin. Ultrasonography is useful in showing organ morphology but not in assessing disease severity.
We have investigated the effect of cystic fibrosis on alimentary hormones in 10 children by measuring the pancreatic and gut hormone rsponse to a milk drink. Plasma insulin and gastric inhibitory peptide were both significantly reduced (P < 0.05 and P < 0.005, respectively, at 15 min) in the patients with cystic fibrosis, compared with controls, even though the early glucose rise was greater in the former group (P < 0.05 at 15 min). Fasting levels of pancreatic polypeptide were significantly lower in the fibrocystic children (P < 0.01), and the normal response to milk was completely abolished in these patients (P < 0.001). Fasting plasma enteroglucagon concentrations were grossly abolished in the cystic fibrosis patients (P < 0.001) and these remained elevated throughout the test. No significant differences were seen in basal or postmilk responses of plasma glucagon, gastrin, secretin, vasoactive intestinal peptide, or motilin in cystic fibrosis. It would thus appear that the pancreatic polypeptide cell is more susceptible to the effects of the disease process than the beta or alpha cell in cystic fibrosis. Some aspects of the abnormalities in the gastrointestinal endocrine system were similar to those seen in celiac disease and tropical sprue and may, therefore, effect a similar hormonal response in these patients with cystic fibrosis to those with mucosal damage.
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The Magnetic Resonance Cholangio Pancreatography (MRCP) provides within a few seconds complete information on cystic degeneration of the pancreas in patients with cystic fibrosis. The authors present a case report of cystic fibrosis with a pathognomonic magnetic resonance cholangio pancreaticography image of the pancreas.
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