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Abnormal paranasal sinuses in patients with cystic fibrosis of the pancreas. Radiological findings.

Radiographic examinations of the paranasal sinuses and chests of 187 patients with C.F. from five months to 27 years of age were reviewed. One hundred eight-five patients consistently had opaque maxillary and ethmoidal sinuses. Forty-five patients had normal chest radiographs. These observations show that the paranasal sinuses are almost always affected in children with C.F. Although opaque paranasal sinuses do not indicate the diagnosis of C.F., clear paranasal sinuses exclude this disease with reasonable certainty.

Adolescent↗

Sonography of the gallbladder, biliary tree, and pancreas in adults with cystic fibrosis.

Real-time sonography is an established method of evaluating the gallbladder, biliary tree, and pancreas. Adequate visualization with B-scan equipment has been a problem in adults with cystic fibrosis, a group with an increased incidence of gallbladder disease. We performed real-time ultrasonography on 23 adult patients with cystic fibrosis. The gallbladder was visualized in 22 (96%) and was found normal in 14 (61%). Abnormalities in the remainder included cholelithiasis (17%) and microgallbladder (30%). No dilatation of the biliary tree was demonstrated. The pancreas was well visualized in 18 (78%) and showed increased echogenicity in most. We conclude that real-time sonography of the abdomen reliably permits examination of the gallbladder, biliary tree, and pancreas in adult patients with cystic fibrosis.

Adolescent↗

Colonization by Enterobacteriaceae of the respiratory tract of children with cystic fibrosis of the pancreas and their antibody response.

Of 72 patients with fibrosis, 49 harbored Enterobacteriaceae in the respiratory tract, including Escherichia coli, Klebsiella, and Enterobacter. Colonization by two to four genera was documented in 29 subjects. Staphylococcus aureus was recovered from 44 of these 49 patients. The distribution of serogroups of E. coli was similar to that seen in patients with urinary tract infection. Antibody response against the O antigens of the patients' own Enterobacteriaceae was documented in 29 of these 49 children and encountered more often in patients with severe disease. Colonization by Enterobacteriaceae in the absence of Pseudomonas aeruginosa was seen more frequently in children with the mild form of the illness.

Adolescent↗

Digestive and absorptive phase anomalies associated with the exocrine pancreatic insufficiency of cystic fibrosis.

The pancreas has an enormous reserve capacity, and significant malabsorption usually signals complete absence of exocrine function. However, there is evidence that acid lipases of nonpancreatic origin play an important compensatory role. Complete duodenal hydrolysis of fat requires a series of complex interdependent physicochemical events involving pancreatic lipase, colipase, phospholipase A2, and bile salts in an environment where the pH must be close to neutrality. Lipolytic products must then be shuttled through the unstirred water layer to the surface of the microvillus membrane by ionized bile salts, which must be present in sufficient concentrations to form micelles. In pancreatic insufficiency, there is not only a defective lipolytic phase but also an impaired micellar phase. The output of bile salts is decreased because of increased fecal loss. Furthermore, a significant percentage of bile salts precipitate because the duodenum is acidic and there is a large predominance of glycine conjugates. Although much less work has been done on the absorptive phase of patients with pancreatic insufficiency, there is tentative evidence that defective phospholipid absorption, essential fatty acid deficiency, and protein malnutrition could impair the absorptive phase, particularly chylomicron formation. Although significant advances have been made in our understanding of factors responsible for malabsorption associated with pancreatic insufficiency, much remains to be done for the further delineation of defects. It is hoped that this will lead to further refinements of enzyme preparations and to new strategies of intervention.

Cystic Fibrosis↗

Cystic fibrosis: MR imaging of the pancreas.

The appearance of the pancreas in 17 adult patients with cystic fibrosis was evaluated with magnetic resonance (MR) imaging. The pancreas was abnormal in 15 patients. Three patterns were observed: (a) lobulated enlarged pancreas with complete replacement by fatty tissue (n = 9), (b) small atrophic pancreas with partial replacement by fat tissue (n = 5), and (c) diffuse atrophy of the pancreas without fatty replacement (n = 1). Replacement of the pancreas by fat tissue was seen on T1-weighted images with, characteristically, a very high signal intensity. The sensitivity of MR imaging in depicting pancreatic abnormality in cystic fibrosis is 94%, which is comparable to that of computed tomography.

Adolescent↗

Focal fatty sparing of the pancreatic head in cystic fibrosis: CT findings.

The most common imaging appearance of the pancreas in cystic fibrosis is diffuse, complete fatty replacement. We present a case of complete fatty replacement of the pancreatic body and tail with total sparing of the pancreatic head. To our knowledge, this pattern of fatty sparing and its associated computed tomographic appearance have not been previously reported in cystic fibrosis.

Adult↗