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PubMed1978

Salivary amylase in cystic fibrosis: a marker of disordered autonomic function.

Total serum amylase was determined in 83 patients with cystic fibrosis (CF) (14 of whom had no steatorrhea), 21 control patients, and 6 patients with Shwachman's syndrome. Patients with CF who had steatorrhea and therefore lacked pancreatic function had the same serum levels pre- and postprandially as the control subjects, whereas patients without steatorrhea and, therefore, with evidence of pancreatic function, had significantly higher levels. The patients with Shwachman's syndrome had significantly lower total serum amylase than the three other patient groups. Only the patients with Shwachman's syndrome demonstrated a significant postprandial rise in activity. Only the salivary isoenzyme was detected in patients with Shwachman's syndrome or CF and apparently absent pancreatic function, whereas CF patients without steatorrhea had both salivary and pancreatic isoenzymes. These results demonstrate that patients with CF have an increase in circulating salivary amylase isoenzyme whereas patients with Shwachman's syndrome do not. The increase probably reflects an autonomic hyperstimulation of salivary secretion which is primary to CF, and unrelated to the state of pancreatic activity.

Amylases↗
PubMed1980

Exocrine pancreatic secretion in rats treated with reserpine after stimulation with pilocarpine, dopamine, and caerulein.

Pancreatic juice was collected in vivo from control and reserpine-treated rats after stimulation with pilocarpine (0.2 mg/100 g body weight), dopamine (6--7 mg/100 g body weight), caerulein (90--100 pmole total dose) or with a combination of caerulein and secretin (6 u/100 g body weight) and the volume, amylase, bicarbonate and chloride outputs were compared. The results indicate that the secretory response to the three secretagogues was significantly reduced in the drug treated animals. Thus, the volumes of pancreatic juice were 57.0, 60.5, and 15.7% of those obtained in control rats after stimulation with, respectively, pilocarpine, dopamine, and caerulein. Amylase output was 63.8, 67.1, and 21.0% and bicarbonate output was 29.9, 46.8, and 6.2% of those observed in untreated rats after the same stimulants. Fluid secretion increased in the treated animals to 71.3% of that of controls when both caerulein and secretin were administered together and amylase output became greater than in control rats (151%). However, bicarbonate output was still 55.2% of that of controls with this combined stimulation. It is concluded that chronic reserpine administration impairs exocrine pancreatic secretion and that this effect involves both the acinar and ductal portions of the gland. This impairment involves the physiologic responses of these two segments of the glandular epithelium to both neural and hormonal stimulants. These findings suggest that the exocrine pancreatic disturbance in reserpine-treated rats may be similar to that observed in cystic fibrosis (CF), and because the treated rat has been proposed as a model for the human disease, they suggest the use of this model as a test system for the study of the pancreatic secretory abnormality in CF.

Amylases↗
PubMed2001

[Cystic fibrosis in adulthood].

Cystic fibrosis (CF) is an autosomal recessive disorder caused by mutations of the CFTR gene. The number of adult CF patients increased dramatically, since life expectancy is now around thirty years. CF is usually a pediatric disease. In adult patients the disease associate a diffuse bronchectasia with chronic colonisation of sputum with Pseudomonas aeruginosa, and pancreatic insufficiency. Mortality is usually related to respiratory insufficiency. One third of adult patients develop diabetes mellitus. A diagnosis of CF can be made in adult patients particularly when it exists male infertility with congenital absence of vas deferens, chronic sinusitis or diffuse bronchectasia or chronic pancreatitis, acute and recurrent pancreatitis, allergic bronchopulmonary aspergillosis. The diagnosis is established with positive sweat chloride concentration, or double CFTR mutations and/or other suggestive organ involvement.

Adult↗
PubMed2001

Regulation and functional significance of airway surface liquid pH.

In gastrointestinal tissues, cumulative evidence from both in vivo and in vitro studies suggests a role for the cystic fibrosis transmembrane conductance regulator (CFTR) in apical epithelial bicarbonate conductance. Abnormal lumenal acidification is thus hypothesized to play a role in the genesis of cystic fibrosis (CF) pancreatic disease. However, consensus regarding CFTR's participation in pH regulation of airway surface liquid (ASL) and thus the contribution of ASL pH to the etiology of CF lung disease, is lacking. The absence of data reflects difficulties in both sampling ASL in vivo and modeling ASL biology in vitro. Here we evaluate the evidence in support of a lumenal acidification hypothesis in the CF lung, summarize current knowledge of pH regulation in the normal airway and illustrate how hyper-acidified airway secretions could contribute to the pathogenesis of CF lung disease.

Animals↗
PubMed1976

Amylase polymorphism: studies of sera and duodenal aspirates in normal individuals and in cystic fibrosis.

Prior genetic studies of the human pancreatic amylase (Amy2) locus have been directed principally to the electrophoretic analysis of serum and urine, on the assumption that these fluids receive negligible contributions from the salivary (Amy 1) locus. In support of that assumption was the observation that the isozyme bands were lacking in patients with cystic fibrosis and in a postpancreatectomy patient. We have examined the sera of 97 patients having cystic fibrosis and find normal levels of serum amylase. On electrophoresis, three-quarters of the cystic fibrosis patients have a pattern (F-pattern) not observed in normal sera. The pattern is characterized by the absence of Pa 1. Comparative electrophoresis and mixing experiments indicate that the F-pattern is of salivary origin and is unmasked in cystic fibrosis by the absence of a pancreatic contribution. The normal serum pattern is considered to be an admixture of salivary and pancreatic amylase. On the assumption that duodenal fluids might more closely reflect the pancreatic (Amy 2) locus, electrophoretic studies were performed on 148 normal individuals and 37 individuals with cystic fibrosis. Electrophoretic phenotypes in duodenal aspirates are more complex than previously reported in studies of urine and serum; presumably because of the higher concentrations of amylase in the aspirates. Comparative electrophoresis and mixing experiments indicate that the phenotypes observed in duodenal aspirates also reflect admixture of pancreatic and salivary amylase. This recognition of pancreatic and salivary admixture in sera fortunately does not alter our prior understanding of the genetics of the Amy 2 polymorphism. The extensive studies which led to the delineation of the Amy 2 polymorphism were essentially based on the presence or absence of a variant band which proves now to be outside the zone of admixture.

Amylases↗