scholarly journals Inherited Causes of Exocrine Pancreatic Dysfunction

1997 ◽  
Vol 11 (2) ◽  
pp. 145-152 ◽  
Author(s):  
Peter R Durie
Keyword(s):  
Cystic Fibrosis ◽  
Lipase Activity ◽  
Exocrine Pancreas ◽  
Adult Life ◽  
Pancreatic Function ◽  
Amylase Secretion ◽  
Pancreatic Amylase ◽  
Pancreatic Dysfunction ◽  
Secondary Phenomenon ◽  
Hereditary Conditions

The exocrine pancreas is functionally immature at birth. Protease function is probably adequate, but lipase activity approximates 5% to 10% of adult values in the newborn and remains low in infancy. Pancreatic amylase secretion is essentially absent at birth and remains low through the first years of life. Functional disturbances of the exocrine pancreas are less frequent in childhood than in adult life. Causes of pancreatic dysfunction in childhood can be divided in two general categories: hereditary conditions, which directly affect the pancreas; and acquired disorders, in which loss of pancreatic function is a secondary phenomenon. Most inherited causes of pancreatic dysfunction are due to a generalized disorder. Cystic fibrosis is, by far, the most common inherited cause of disturbed pancreatic function among Caucasian children. All other inherited causes of exocrine pancreatic dysfunction (eg, Johanson-Blizzard syndrome) are uncommon or rare.

Alterations of pancreatic amylase secretion in the reserpinized rat model of cystic fibrosis

1994 ◽  
Vol 16 (1) ◽  
pp. 37-44
Author(s):  
Jean Morisset ◽  
France-Line Béruhé ◽  
Micheline Vanier ◽  
Ouhida Benrezzak
Keyword(s):  
Cystic Fibrosis ◽  
Rat Model ◽  
Amylase Secretion ◽  
Pancreatic Amylase

The effects of kynurenine on pancreatic amylase secretion in the rats

Pancreatology ◽  
2012 ◽  
Vol 12 (6) ◽  
pp. 509
Author(s):  
K. Nawrot-Porąbka ◽  
A. Leja-Szpak ◽  
J. Jaworek
Keyword(s):  
Amylase Secretion ◽  
Pancreatic Amylase

Serum Immunoreactive Pancreatic Lipase and Cationic Trypsinogen for the Assessment of Exocrine Pancreatic Function in Older Patients With Cystic Fibrosis

PEDIATRICS ◽  
1986 ◽  
Vol 77 (3) ◽  
pp. 301-306
Author(s):  
Geoffrey Cleghorn ◽  
Lynne Benjamin ◽  
Mary Corey ◽  
Gordon Forstner ◽  
Francesco Dati ◽  
...  
Keyword(s):  
Cystic Fibrosis ◽  
Pancreatic Lipase ◽  
Elevated Serum ◽  
Serum Levels ◽  
Exocrine Pancreatic Function ◽  
Pancreatic Function ◽  
Exocrine Function ◽  
Serum Lipase ◽  
Significant Difference ◽  
Cationic Trypsinogen

Indirect and qualitative tests of pancreatic function are commonly used to screen patients with cystic fibrosis for pancreatic insufficiency. In an attempt to develop a more quantitative assessment, we compared the usefulness of measuring serum pancreatic lipase using a newly developed enzyme-linked immunosorbent immunoassay with that of cationic trypsinogen using a radioimmunoassay in the assessment of exocrine pancreatic function in patients with cystic fibrosis. Previously, we have shown neither lipase nor trypsinogen to be of use in assessing pancreatic function prior to 5 years of age because the majority of patients with cystic fibrosis in early infancy have elevated serum levels regardless of pancreatic function. Therefore, we studied 77 patients with cystic fibrosis older than 5 years of age, 41 with steatorrhea and 36 without steatorrhea. In addition, 28 of 77 patients consented to undergo a quantitative pancreatic stimulation test. There was a significant difference between the steatorrheic and nonsteatorrheic patients with the steatorrheic group having lower lipase and trypsinogen values than the nonsteatorrheic group (P <.001). Sensitivities and specificities in detecting steatorrhea were 95% and 86%, respectively, for lipase and 93% and 92%, respectively, for trypsinogen. No correlations were found between the serum levels of lipase and trypsinogen and their respective duodenal concentrations because of abnormally high serum levels of both enzymes found in some nonsteatorrheic patients. We conclude from this study that both serum lipase and trypsinogen levels accurately detect steatorrhea in patients with cystic fibrosis who are older than 5 years but are imprecise indicators of specific pancreatic exocrine function above the level needed for normal fat absorption.

Presynaptic inhibition by serotonin of nerve-mediated secretion of pancreatic amylase

1995 ◽  
Vol 268 (2) ◽  
pp. G339-G345 ◽  
Author(s):  
A. L. Kirchgessner ◽  
M. D. Gershon
Keyword(s):  
Presynaptic Inhibition ◽  
Cholinergic Neurons ◽  
Acinar Cells ◽  
Presynaptic Receptors ◽  
Amylase Secretion ◽  
Pancreatic Amylase ◽  
Inhibitory Receptor ◽  
Cholinergic Nerves ◽  
Positive Identification ◽  
Fos Expression

Enteric cholinergic and serotonergic neurons innervate pancreatic ganglia. Enteropancreatic cholinergic neurons are secretomotor, bu the function of the serotonergic cells is unknown and was investigated. Postganglionic cholinergic nerve-mediated amylase secretion was evoked by veratridine in isolated pancreatic lobules. This concentration-dependent response was inhibited by tetrodotoxin (1.0 microM), atropine (5.0 microM), 5-hydroxytryptamine (5-HT; 5.0 microM), 5-hydroxyindalpine (5-OHIP; 10.0 microM; a 5-HT1P agonist), and 8-hydroxy-2-(di-n-propylamino)tetralin (8-OH-DPAT; 0.1 microM), but not by hexamethonium (100.0 microM), 2-methyl-5-HT (10 microM), or 5-carboxyamidotryptamine (10 microM). The effects of 5-HT and 5-OHIP were blocked by the 5-HT1P antagonist N-acetyl-5-hydroxytryptophyl-5-hydroxytryptophan amide (5-HTP-DP; 100.0 microM). Carbachol (5.0 microM)-evoked secretion was not affected by 5-HT or 5-OHIP. Veratridine induced c-fos expression in pancreatic neurons and acinar cells. This expression was inhibited by tetrodotoxin, 5-HT, and 5-OHIP. These observations suggest that the serotonergic enteropancreatic innervation inhibits pancreatic secretion via presynaptic receptors on cholinergic nerves. Although the data are consistent with the hypothesis that the inhibitory receptor is a 5-HT1P site, positive identification awaits further study.

Cystic fibrosis--its biochemical detection.

1983 ◽  
Vol 29 (12) ◽  
pp. 2011-2018 ◽  
Author(s):  
A F Heeley ◽  
D Watson
Keyword(s):  
Cystic Fibrosis ◽  
Filter Paper ◽  
Neonatal Screening ◽  
Duodenal Juice ◽  
Heterozygous State ◽  
Intestinal Malabsorption ◽  
Pancreatic Lesion ◽  
Pancreatic Dysfunction ◽  
Pancreatic Isoamylase ◽  
Biochemical Detection

Abstract We examine critically the biochemical methods capable of detecting and monitoring the end-organ disease processes in patients with cystic fibrosis. Although the diagnosis of cystic fibrosis is never justified on the basis of the sweat salt test alone, the original filter-paper technic (Gibson-Cooke, Pediatrics 23:545-549, 1959) for determining Na+ and Cl- concentrations in sweat remains the most discriminating method. We discuss the contributions for neonatal screening of the so-called cystic fibrosis protein, associated decreased enzymic activities in the homo- and heterozygous state, and immunoreactive trypsin. Because evidence of either intestinal malabsorption or a pancreatic lesion must be sought, we review the use and interpretation of some tests of pancreatic dysfunction (meconium albumin, duodenal juice components, serum pancreatic isoamylase, and trypsinogen), both in establishing and in confirming the diagnosis of cystic fibrosis.

Changes in the exocrine pancreas secondary to altered small intestinal function in the CF mouse

2001 ◽  
Vol 281 (4) ◽  
pp. G899-G906 ◽  
Author(s):  
Robert C. De Lisle ◽  
Kathryn S. Isom ◽  
Donna Ziemer ◽  
Calvin U. Cotton
Keyword(s):  
Exocrine Pancreas ◽  
Exocrine Function ◽  
Amylase Secretion ◽  
Mrna Levels ◽  
Intestinal Function ◽  
Small Intestinal ◽  
First Time ◽  
Camp Levels

The exocrine pancreas of the cystic fibrosis (CF) mouse ( cftrm1UNC ) is only mildly affected compared with the human disease, providing a useful model to study alterations in exocrine function. The CF mouse pancreas has ∼50% of normal amylase levels and ∼200% normal Muclin levels, the major sulfated glycoprotein of the pancreas. Protein biosynthetic rates and mRNA levels for amylase were not altered in CF compared with normal mice, and increases in Muclin biosynthesis and mRNA paralleled the increased protein content. Stimulated pancreatic amylase secretion in vitro and in vivo tended to be increased in CF mice but was not statistically significant compared with normal mice. We show for the first time that the CF mouse duodenum is abnormally acidic (normal intestinal pH = 6.47 ± 0.05; CF intestinal pH = 6.15 ± 0.07) and hypothesize that this may result in increased signaling to the exocrine pancreas. There were significant increases in CF intestinal mRNA levels for secretin (310% of normal, P < 0.001) and vasoactive intestinal peptide (148% of normal, P < 0.05). Furthermore, CF pancreatic cAMP levels were 147% of normal ( P < 0.01). These data suggest that the CF pancreas may be chronically stimulated by cAMP-mediated signals, which in turn may exacerbate protein plugging in the acinar/ductal lumen, believed to be the primary cause of destruction of the pancreas in CF.

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