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PubMedDate not supplied

[Pancreatic cystic fibrosis in Mexicans over 15 years of age].

A better knowledge of cystic fibrosis of the pancreas has contributed to raise the detection of cystic fibrosis in adults. We describe nine Mexican patients older than 15 years with cystic fibrosis. Respiratory symptoms were predominant and they were secondary to bronchiectasis. All patients were infected by mucoid Pseudomona aeruginosa and in some cases, the finding of this microorganism in sputum suggested the diagnosis. In Mexican population the cystyc fibrosis of the pancreas can be found in adult patients, and it should be considered in the differential diagnosis of chronic respiratory diseases in adults.

Adolescent↗
PubMed1985

The chronically pilocarpine-treated rat in the study of cystic fibrosis: investigations on submandibular gland and pancreas.

The chronically pilocarpine-treated rat has been proposed as an animal model for the disease cystic fibrosis, a generalized exocrinopathy. The effect of chronic pilocarpine treatment on structure, composition, and function of the acinar cells of rat submandibular gland and pancreas was investigated by electron microscopy, X-ray microanalysis, and biochemical analysis. The morphological effects of chronic pilocarpine treatment were most pronounced in the pancreas. The number and size of the zymogen granules was increased, and the granules had a less electron-dense appearance. X-ray microanalysis at the cellular level showed in both the submandibular gland and the pancreas a significant increase in calcium and a decrease in sodium. The increase in cellular calcium concentrations can be explained by an increase in the relative volume of secretory material in the cell (assessed by morphometry) and an increase in the local calcium concentration in the secretory material (assessed by X-ray microanalysis at the subcellular level). Chronic pilocarpine treatment caused a disturbance of glycolysis and energy metabolism in the submandibular gland, but no significant effects in this respect were noted in the pancreas. On the other hand, a nearly twofold increase of the pancreatic amylase activity was noted. The pancreas appeared somewhat hyperreactive towards cholinergic stimulation.

Animals↗
PubMed2003

[Fatty replacement of the pancreas in a patient with asymptomatic pulmonary cystic fibrosis].

The authors report the case of a 26-year-old woman with hepatomegaly, who had recurrent and progressive nausea and abdominal pain. Computed tomography and magnetic resonance imaging demonstrated fatty replacement of the entire pancreas resulting from cystic fibrosis. The pulmonary disease was totally asymptomatic. Fatty replacement of the pancreas is the most frequent pattern in older patients with cystic fibrosis. This pattern correlates with pancreatic exocrine dysfunction.

Adipose Tissue↗
PubMed1987

Establishment of a tissue culture system for epithelial cells derived from human pancreas: a model for the study of cystic fibrosis.

A tissue culture system for epithelial cells derived from human foetal pancreas has been established. The cultured cells show many ultrastructural features of interlobular duct cells. Immunocytochemical and histochemical evidence is presented in support of the view that these cells are ductal in origin. They are likely to be one of the few cell types that express the basic defect of cystic fibrosis in vitro. The cells may be passaged and sufficient material obtained to permit biochemical and molecular biological analysis.

Cells, Cultured↗
PubMedDate not supplied

[Endocrine pancreas function in mucoviscidosis].

Patients with cystic fibrosis of the pancreas show an incidence of diabetes mellitus tenfold higher than is found in the general pediatric population. Considering this fact glucagon and insulin responses to oral glucose and intravenous arginine were studied in 22 CF children and adolescents. Some investigated patients had suffered from the disease for ten years and more. On the one hand the results show that pancreatic alpha cell function is normal. The kinetics of endogenous glucagon release are unaltered. On the other hand the data reveal that there is only a defect in the beta cell function consisting in a delayed insulin release selective to glucose whereas responsiveness to other stimuli for example tolbutamide and arginine is undisturbed. Furthermore there is a diminution in insulin-output to oral glucose as well as to intravenous arginine. Yet in patients with normal oral glucose tolerance endogenous insulin secretion is significantly reduced. Their regular carbohydrate tolerance may be a function of the patient's ability to maintain increased receptor numbers in the face of hypoinsulinemia. Despite greater quantities of secreted hormone a degree of relative peripheral insulin insensitivity has developed in the presence of hyperglycemia. This may be a consequence of impaired affinity of the specific target cell receptors. The insulin secretion pattern is proven to be identical to that of chemical diabetes mellitus in adults. Quantitative diminution in arginine stimulated insulin-output has been found to be independent of the degree of carbohydrate intolerance.(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗
PubMed2005

Simultaneous liver and pancreas transplantation in patients with cystic fibrosis.

BACKGROUND: Improved survival in patients with cystic fibrosis (CF) has led to an increased incidence of extrapulmonary complications of this disease. Of these, cirrhosis and pancreatic insufficiency, including CF-related diabetes (CFRD) and exocrine insufficiency, are significant causes of morbidity and mortality. Liver transplantation is the treatment of choice for cirrhosis in this setting, but the addition of an isolated simultaneous pancreas transplant in patients with CFRD has not been reported. METHODS: Two female patients with CF underwent simultaneous pancreas and liver transplantation. Both had pancreatic insufficiency, CFRD, cirrhosis, and preserved renal function. In each case, the liver and pancreas were procured from a single cadaveric donor. The liver transplant was performed first. A lower midline extension was added for improved exposure of the iliac vessels. The donor pancreas transplant was performed with systemic venous drainage and enteric exocrine drainage. Immunosuppression included rabbit anti-thymocyte globulin, tacrolimus, mycophenolate mofetil, and early steroid withdrawal. RESULTS: Both patients recovered well with normal liver function, resolution of portal hypertension, and normal blood glucoses independent of insulin. As a result of the enteric exocrine drainage of the pancreas, they are now independent of supplemental pancreatic enzymes. CONCLUSIONS: Simultaneous liver and pancreas transplantation in CF patients provides the advantages of normalization of glucose and improved nutrition for patients requiring liver transplantation and should be considered in CF patients with CFRD who require liver transplants.

Adolescent↗
PubMed1993

Quantitative assessment of pancreatic function by magnetic resonance in cystic fibrosis.

To determine the value of magnetic resonance imaging in the analysis of pancreatic function in cystic fibrosis, 11 adult cystic fibrosis patients with exocrine pancreatic insufficiency were studied by magnetic resonance and a set of non-invasive tests of exocrine and endocrine pancreatic function. Magnetic resonance imaging, comprising the calculation of intensity ratios (pancreas/liver) of the pancreas on T1 ([SE] TR/TE = 300/20) and T2 weighted ([SE] TR/TE = 2000/50-100)images and the measurement of several dimensions of the pancreas, was compared with the results of the indirect pancreatic function tests. Although magnetic resonance showed the typical morphology of the insufficient pancreas in cystic fibrosis, including atrophy, lobular fatty enlargement and infiltration, it was not related to the quantitative exocrine and endocrine function assessed by indirect pancreatic function tests.

Adult↗