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At least 19 recordsLinked to original sources
PubMed1980

[Functional evaluation of pancreas cystic fibrosis. Correlations and value of the echocardiogram versus other methods (author's transl)].

The results of a correlation of various parameters used in the functional evaluation of 12 cases of pancreas cystic fibrosis (PCF) are reported. Three clinical scores were measured by four separate observers and average score was taken. Electrocardiogram, phono, vectorcardiogram, pH, blood gases, radiology, spirometry, erythropoietin, blood counts and echocardiogram were also recorded. Each of these were correlated. In the echocardiogram (EC), right and left systolic time intervals, wall and cavity of the right ventricle and the left ventricular function were measured. Some correlations were found to have a statistical significance: the data attained from the EC, clinical score and the spirometry. However, blood count, blood gases, erythropoietin and pH were found to have no statistical significance. The EC was found to be the most accurate and sensitive method of recognising the initial stages of pulmonary hypertension in PCF; detecting its presence at as early an age as six years (on average). These results present a strong argument for the use of the EC as a vital part of each exam of PCF.

Child↗
PubMed1988

Impaired chloride secretion, as well as bicarbonate secretion, underlies the fluid secretory defect in the cystic fibrosis pancreas.

Pancreatic fluid and electrolyte secretion was assessed in 56 patients with cystic fibrosis (CF) and 56 non-CF control subjects undergoing pancreatic function testing while stimulated with cholecystokinin and secretin. Both CF patients and control subjects exhibited a wide range of pancreatic function. Fluid and trypsin outputs were positively correlated in both groups. Fluid output in CF subjects was significantly lower, however, than that of control subjects at any given level of trypsin output. Sodium, bicarbonate, and chloride secretions were all significantly decreased in CF subjects. Bicarbonate and chloride were important determinants of fluid secretion, but at any given bicarbonate or chloride output CF subjects secreted significantly less fluid than control subjects. When bicarbonate and chloride were analyzed as simultaneous predictor variables, adjusted fluid secretion was not significantly different in CF and control subjects. Diminished fluid secretion in CF subjects is therefore caused by impaired chloride, as well as bicarbonate, secretion.

Bicarbonates↗
PubMed1971

Opsonic defect in patients with cystic fibrosis of the pancreas.

Cystic fibrosis of the pancreas is one of the most common inborn errors of metabolism. The high incidence of morbidity and mortality in these patients is primarily due to severe and frequent pulmonary infection. To date, no immune deficiency has been found in cystic fibrosis patients. Their sera contain normal quantities of immunoglobulins and hemolytic complement. In an assay of phagocytosis by alveolar macrophage, six out of nine sera from cystic fibrosis patients failed to support normal phagocytosis of Pseudomonas aeruginosa. This deficiency could be corrected by increasing the concentration of serum used in the assay. By contrast, their sera supported normal phagocytosis of Pseudomonas by blood polymorphonuclear leukocytes and continued to support normal phagocytosis when serum dilutions were used. Two patients with severe isolated deficiences of serum immunoglobulin A were found to have a similar defect in the alveolar macrophage assay, but normal phagocytosis by polymorphonuclear leukocytes. It is postulated that cystic fibrosis patients may have a quantitative and (or) functional defect of IgA antibodies, specific for Pseudomonas, and possibly of importance in the pathogenesis of their pulmonary disease.

Adolescent↗
PubMed1995

Chloride channels and cystic fibrosis of the pancreas.

Cystic fibrosis (CF) affects approximately 1 in 2000 people making it one of the commonest fatal, inherited diseases in the Caucasian population. CF is caused by mutations in a cyclic AMP-regulated chloride channel known as CFTR, which is found on the apical plasma membrane of many exocrine epithelial cells. In the CF pancreas, dysfunction of the CFTR reduces the secretory activity of the tubular duct cells, which leads to blockage of the ductal system and eventual fibrosis of the whole gland. One possible approach to treating the disease would be to activate an alternative chloride channel capable of bypassing defective CFTR. A strong candidate for this is a chloride channel regulated by intracellular calcium, which has recently been shown to protect the pancreas in transgenic CF mice. Pharmacological intervention directed at activating this calcium-activated Cl- conductance might provide a possible therapy to treat the problems of pancreatic dysfunction in CF.

Animals↗
PubMed1989

Origins of chloride and bicarbonate secretory defects in the cystic fibrosis pancreas, as suggested by pancreatic function studies on control and CF subjects with preserved pancreatic function.

Pancreatic function studies were performed under continuous intravenous stimulation with secretin and cholecystokinin (0.125 mu/kg/min) in 40 patients with CF who had no evidence of steatorrhea and 69 control subjects without CF, who also lacked evidence of steatorrhea. A total of 41 studies in CF patients and 74 in control subjects were analysed for trypsin, electrolyte, and water secretion. 51% of the CF and 51% of the control tests showed trypsin secretion responses between 1000 mu/kg/hr (the lower limit of the normal range) and 2695 mu/kg/hr, while 16.2% of the control tests exceeded this range. Secretion of water and electrolytes, including both chloride and bicarbonate, was significantly lower in the CF subjects when compared to the appropriate controls in the normal and subnormal trypsin output range. Chloride-bicarbonate ratios, although higher in subjects with evidence of pancreatic damage, were identical in CF and control studies. A specific ductal lesion in CF which altered the balance of acinar (predominately chloride) and ductal (predominately bicarbonate) secretion, or encouraged the equilibration of ductal fluid with plasma in the distal ducts, would increase chloride-bicarbonate ratios, as in the subjects with reduced trypsin secretion. Since this did not occur in the patients with normal function, our results suggest that chloride and bicarbonate secretions are impaired to a similar extent in CF, possibly through a dependence on the same phenotypic abnormality.

Bicarbonates↗
PubMed2002

The pancreas in cystic fibrosis.

The pancreas secretes a bicarbonate-rich fluid containing digestive enzymes via the ampulla of Vater into the duodenum. Defective secretion leads to maldigestion of fat and protein with increased faecal losses. Cystic fibrosis (CF) is the major cause of pancreatic exocrine failure in childhood, whereas pancreatic insufficiency in adults is commonly associated with chronic pancreatitis and alcohol ingestion. In cystic fibrosis, pancreatic function correlates with genotype; pancreatic-sufficient (PS) patients have a milder course of respiratory disease, improved survival and lower mean sweat chloride concentrations than those with pancreatic insufficiency. Recent observations suggest that mutant CF alleles are over-represented in patients with chronic pancreatitis. Few show evidence of sino-pulmonary disease or high sweat electrolyte concentrations.

Adult↗
PubMed1991

Localization of the cystic fibrosis transmembrane conductance regulator in pancreas.

Cystic fibrosis (CF) is characterized by an abnormality in cAMP-regulated chloride transport that results from a primary defect in the protein product of the CF gene, the CF transmembrane conductance regulator (CFTR). In this report, antibodies against CFTR peptides were used to localize the CFTR protein in human pancreas. An affinity purified antibody (alpha-1468) raised against a synthetic CFTR peptide identified a 155-170-kD protein on immunoblot. Cytochemical studies with alpha-1468 localized CFTR to small branching, tubular structures. The same structures were recognized by two other antibodies raised against different regions of the CFTR molecule. To identify the cells being stained, double-label immunofluorescence studies were performed using alpha-1468 and a monoclonal antibody which stains pancreatic centroacinar and intralobular duct cells. Both antibodies localized to the same population of cells, with alpha-1468 being confined to the apical domain of these cells. No conclusive staining of acinar cells was evident. These findings suggest that proximal duct epithelial cells play a key role in the early events leading to pancreatic insufficiency in CF, and imply that apical chloride transport by these cells is essential for normal pancreatic secretory function.

Amino Acid Sequence↗