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At least 775 records · Page 43Linked to original sources
PubMed1993

Pancreas and gut hormone responses to oral glucose and intravenous glucagon in cystic fibrosis patients with normal, impaired, and diabetic glucose tolerance.

Pancreatic and gut hormone responses to oral glucose, and insulin sensitivity were studied in cystic fibrosis patients with normal (N = 14), impaired (N = 4), and diabetic (N = 12) glucose tolerance, and in 10 control subjects, and beta cell responses to oral glucose and intravenous glucagon were compared. Compared to control subjects, initial insulin and C-peptide responses to oral glucose were lower in all patient groups, and decreased with decreasing glucose tolerance. Insulin sensitivity in patients with impaired and diabetic glucose tolerance was lower than in control subjects. The 6 min post-glucagon C-peptide concentration was positively correlated with the initial insulin response to oral glucose. Fasting levels of pancreatic polypeptide, pancreatic glucagon, total glucagon, glucagon-like peptide-1 7-36 amide, and gastric inhibitory polypeptide were normal in all patient groups. Following oral glucose, pancreatic polypeptide responses were absent in all patients, suppressibility of pancreatic glucagon secretion was increasingly impaired with decreasing glucose tolerance, and gut hormone levels were normal. In conclusion, at cystic fibrosis (a) insulin secretion is impaired even when glucose tolerance and insulin sensitivity are within the normal range, (b) the glucagon test gives valid estimates of residual beta cell function, (c) pancreatic polypeptide response to oral glucose is absent, (d) glucagon suppressibility decreases with decreasing glucose tolerance, and (e) the enteroinsular axis is intact.

Administration, Oral↗
PubMedDate not supplied

Portal vein measurement by ultrasonography in patients with long-standing cystic fibrosis: preliminary observations.

Adult cystic fibrosis (CF) patients are increasingly diagnosed with hepatic cirrhosis, cholecystitis, and cholelithiasis. A continuing diagnostic problem is the early detection of cirrhosis prior to diffuse liver involvement. Sonography has been used in evaluating the pancreas, gallbladder, liver, and spleen in cystic fibrosis patients. We used a real-time mechanical sector scanner to study the portal veins in adult CF patients randomly selected from our CF population and correlated the portal vein diameters with liver function studies. A measurement greater than 12 mm was interpreted as a probable sign of portal hypertension. Of 21 patients studied, 14 had portal veins that measured greater than 12 mm, and 12 patients had (although not necessarily at the time of the exam) elevated serum alkaline phosphatase levels. Three patients had associated splenomegaly and thrombocytopenia, and 10 patients also exhibited cholelithiasis and abnormal gallbladders on sonography. Initial results indicate that portal vein measurement may be a sensitive indicator of early portal hypertension. Sonography may, thus, isolate patients at high risk for possible future complications. Serial sonograms can be performed easily in evaluating the progress of liver disease.

Adolescent↗
PubMed1983

Kallikrein and amylase contents in tissues from a mutant mouse model for human cystic fibrosis.

Kallikrein and amylase activities are decreased in the pancreas and salivary glands from cri/cri homozygote mutant mice. Kallikrein is decreased in the cri/cri kidney too. With reference to nucleic acid concentrations there is no difference between control and mutant mice. The previously described electrolyte abnormalities of the cribriform degeneration (cri) mutant mouse, could be due to the abnormal activity of the kallikrein-kinin system on the transport mechanism of tubular cells in the organs mentioned. These findings represent a new step on our efforts to develop a useful animal model for human cystic fibrosis research.

Amylases↗
PubMed1983

Pancreatic polypeptide secretion. A marker for disturbed pancreatic function in cystic fibrosis.

Pancreatic polypeptide, a 36-amino peptide, is released from the pancreas by a variety of stimuli, including intravenous Boots secretin. Studies in a generalized destructive and inflammatory process such as chronic pancreatitis have revealed a markedly diminished response to stimulation. To assess whether pancreatic polypeptide release in response to Boots secretin provides a useful measure of pancreatic destruction in cystic fibrosis, 41 patients with proven cystic fibrosis, aged 14 months-23 years, and seven control subjects, aged 18-24 years were studied. Serum pancreatic polypeptide, measured by radioimmunoassay, rose from a basal of 18.5 +/- 2.7 pmol/liter to a peak of 35.6 +/- 4.3 pmol/liter at 5 min in cystic fibrosis, and from a basal of 10.8 +/- 2.8 pmol/liter to a 5-min peak of 109 +/- 27.7 pmol/liter in control subjects. The basal levels of both groups were similar but the cystic fibrosis patients had a significantly lower peak response than controls (P less than 0.05). The peak over basal pancreatic polypeptide ratio was calculated and was less than five in 93% of cystic fibrosis patients, whereas all control subjects had a ratio greater than five. Pancreatic polypeptide measurements in response to secretin may be a convenient and useful means of following the course of pancreatic disease in a chronic illness such as cystic fibrosis.

Adolescent↗
PubMed1989

The pathophysiology of the pancreatic defect in cystic fibrosis.

Studies of the postnatal development of the pancrease in CF infants show a failure of acinar development and an increase in lumen volume with accumulation of secretory material within the pancreatic ducts. Our evaluation of functional changes within the exocrine pancreas are consistent with the pathologic findings. Impaired pancreatic fluid secretion appears to be a primary phenomenon of CF. We have shown that pancreatic secretions from CF patients also contain significantly higher concentrations of protein in comparison with pancreatic function-matched controls. Since total protein output is not increased, hyperconcentration of protein appears to be a direct result of a primary defect of fluid secretion. Protein hyperconcentration appears to predispose CF patients to protein precipitation and obstruction within small pancreatic ducts which, in turn, produces pancreatic acinar atrophy and fibrosis Our studies suggest that both bicarbonate and chloride transport within pancreatic ducts account for deficient fluid secretion in the pancreas of CF subjects.

Cystic Fibrosis↗
PubMed2003

CFTR pharmacology and its role in intestinal fluid secretion.

The cystic fibrosis transmembrane conductance regulator (CFTR) is a cAMP-activated Cl(-) channel expressed in epithelial cells in the airways, pancreas, intestine and other fluid-transporting tissues. Cystic fibrosis is caused by mutations in the CFTR, resulting in impaired Cl(-) transport and plasma membrane targeting. CFTR is expressed in the lumenal membrane of enterocytes, where it functions as the principal pathway for secretion of Cl(-) and fluid in enterotoxin-induced secretory diarrheas such as cholera. Small-molecule CFTR inhibitors reduce enterotoxin-induced intestinal fluid secretion in animal models. CFTR inhibition might also reduce intestinal fluid losses in cholera and possibly in other infectious and non-infectious diarrheas.

Animals↗
PubMed1996

Exocrine pancreatic alterations in long-lived surviving cystic fibrosis mice.

We investigated the development of the exocrine pancreas in Cftr-/- mice in comparison with age-matched littermates (Cftr+/+, Cftr+/-) up to 100 d postnatally. Controls were weaned either to mouse chow or a liquid diet; Cftr-/- mice were weaned solely to a liquid diet. Solid-fed control mice gained weight and showed a progressive increase in pancreatic protein, DNA, amylase, lipase, trypsin, and chymotrypsin activities. Liquid-fed control mice showed similar postnatal somatic and pancreatic growth, except that amylase and lipase activities were lower than in the solid-fed controls. Cftr-/- mice exhibited significantly lower body and pancreatic weights than did controls. Pancreatic protein content and enzyme activities (notably amylase and lipase) were consistently lower than in the age-matched litter-mates fed either diet. The reduction in lipase activity in Cftr-/- mice was noted before weaning. We concluded that the liquid diet influenced postnatal exocrine pancreatic development in mice. However, a further reduction in postnatal pancreatic growth and enzymatic activities in the Cftr-/- mice was noted. These alterations could be due to the primary cystic fibrosis defect, although secondary factors, such as malnutrition induced by decreased dietary intake or abnormal absorptive capacity, may be responsible.

Amylases↗