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Results for “CYSTIC FIBROSIS OF PANCREAS”
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[Cystic fibrosis in adults; lesions of the digestive tract].
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[Generalized form of mucoviscidosis with edematous-anemic and icteric syndromes].
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[Exocrine pancreatic secretion in children. Study methods and results].
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[Physiopathology of intestinal malabsorption (report)].
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Use of serum amylase isoenzymes in evaluation of pancreatic function.
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[Mucoviscidosis (Cystic fibrosis)].
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[Study of exocrine pancreatic function in mucoviscidosis].
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Fibrocystic disease of pancreas in India.
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Cystic fibrosis: overview and pulmonary aspects in young adults.
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Exocrine pancreatic function in cystic fibrosis.
Pancreatic dysfunction in cystic fibrosis (CF) begins in utero and, at birth, in most cases, cystic fibrosis is characterized by an elevated level of serum immunoreactive trypsin (IRT). If most patients with CF typically present insufficient pancreatic exocrine function, 10-15% of CF patients have pancreatic sufficiency and this status is genetically determined by one or two 'mild' mutations in CF transmembrane conductance regulator (CFTR). However, with age, these patients can develop pancreatic insufficiency.
Effect of cerulein hyperstimulation on the paracellular barrier of rat exocrine pancreas.
BACKGROUND/AIMS: Cerulein-induced pancreatitis causes a rapid increase in pancreatic enzyme levels in serum and decreases in pancreatic duct secretion and interstitial edema. One mechanism to explain these early events is disruption of the actin tight junction paracellular seal of acinar and intralobular pancreatic duct cells. METHODS: To examine the paracellular barrier of the proximal exocrine pancreas, rats were hyperstimulated with 5.0 micrograms.kg-1.h-1 of cerulein. Actin was visualized with rhodamine phalloidin and by electron microscopy and tight junctions were visualized with antibodies to the tight-junction protein ZO-1. Paracellular permeability was measured by movement of horseradish peroxidase from interstitium into duct or acinar lumens. RESULTS: In controls, linear actin and ZO-1 staining occurred along the apical membrane of intralobular duct cells and extended to the apical pole of acinar cells. Hyperstimulation caused progressive disruption of the linear staining of f-actin and ZO-1. Actin disruption in duct cells was confirmed by electron microscopy. Horseradish peroxidase entered intralobular ducts and acinar lumens of hyperstimulated animals more frequently than those of controls. CONCLUSIONS: The structure and function of the paracellular barrier of acinar and intralobular pancreatic duct cells are disrupted early during cerulein pancreatitis and may contribute to early clinical features.
Antisense oligodeoxynucleotide to the cystic fibrosis transmembrane conductance regulator inhibits cyclic AMP-activated but not calcium-activated cell volume reduction in a human pancreatic duct cell line.
Cystic fibrosis (CF) is characterized by a defect in cAMP-regulated chloride channels in epithelial cells. The CF gene product CF transmembrane conductance regulator (CFTR) is expressed in the apical membrane of pancreatic duct cells, and mutant CFTR accounts for the pathology in the CF pancreas. PANC 1, a pancreatic duct cell line, has not been considered a good model for studying CFTR and pancreatic chloride transport because CFTR mRNA and protein are undetectable using standard methods. Using electronic cell sizing and cell volume reduction under isotonic conditions, PANC 1 cells were found to possess both cAMP and calcium-activated chloride conductances. Using CFTR antisense oligodeoxynucleotides, the cAMP-activated conductance could be specifically inhibited in a concentration- and time-dependent manner. These findings demonstrate that PANC 1 cells express CFTR and a CFTR-independent calcium-activated chloride channel. With electronic cell sizing and CFTR antisense oligodeoxynucleotides, PANC 1 cells can provide an ideal system for the study of pancreatic duct cell physiology and pathophysiology with respect to the role of CFTR in the pancreas. These findings also suggest that antisense oligodeoxynucleotides may provide a more sensitive yet highly specific means of detecting low levels of expression of CFTR than currently available.
New insights into the pathogenesis of cystic fibrosis.
Cystic fibrosis (CF) is the most frequent inheritable disease with a lethal course during childhood. The characteristic high viscosity of the mucoid secretion products in the lungs, pancreas, and gut cause plugging and secondary damage of these organs. In the past 20 years effective treatment of intestinal obstruction in the neonatal period and the infections of the lungs has improved the prognosis significantly. Many patients will reach adulthood in the near future. In the past 10 years new insights into the cause of the disease changed diagnostic procedures and, it is to be hoped, soon also treatment. The first development was the estimation of brush-border enzymes in amniotic fluid. With this method prenatal diagnosis is possible in the 17th-18th week of pregnancy. The recent discovery of the gene on chromosome 7 and its structure is the most important breakthrough. At the same time the process of Cl- transport across the mucosal membrane of many types of epithelium was subject to investigation by several laboratories. We have studied the transport of ions in the small and large intestines of CF patients. The effect of all three types of intracellular signal transfer is abnormal, although the second messengers themselves (cAMP, cGMP, and Ca2+) are present. Evidence is found for K+ instead of Cl- secretion after addition of secretagogues.
Progesterone and estradiol inhibit CFTR-mediated ion transport by pancreatic epithelial cells.
The cystic fibrosis (CF), gene product, CF transmembrane conductance regulator (CFTR), is responsible for adenosine 3',5'-cyclic monophosphate (cAMP)-activated Cl- transport in epithelial cells, and mutant CFTR accounts for the pathology in the CF pancreas. We have previously shown that both isolated rabbit pancreatic acini and the human pancreatic duct cell line PANC-1 possess a cAMP-activated Cl- conductance identified as CFTR. We report here that preincubation in either of the female hormones progesterone or beta-estradiol inhibits activation by cAMP, but not by Ca2+ ionophore, of PANC-1 cell volume reduction under isotonic conditions. cAMP-activated cell volume reduction is sensitive to antisense, but not sense, CFTR oligodeoxynucleotide. Furthermore, progesterone inhibits cAMP-activated Cl- efflux from rabbit acinar cells. Moreover preincubation with progesterone, but not beta-estradiol, reduces CFTR mRNA and protein levels as measured using polymerase chain reaction amplification of reverse-transcribed acinar RNA and Western blots of protein from acinar membranes. We conclude that female hormones inhibit CFTR functional activity in pancreatic epithelial cells by different mechanisms.
[Sonography of the upper abdomen in cystic fibrosis (author's transl)].
Ultrasound scans of the upper abdomen were carried out in fifty patients with cystic fibrosis (age 2 to 26 years). 38% showed an abnormal hepatic sonogram. On eight occasions it was possible to confirm these changes by a liver biopsy. In 14% there was homogenous increase in liver echoes. 22% showed an non-homogeneous liver structure due to fibrotic and cirrhotic changes and cholangitis. Four of these patients had splenomegaly due to portal hypertension. 85% of the pancreatic scans which could be evaluated showed small pancreases with high echoes. Cholelithiasis was not present in any. Sonography of the upper abdomen is the method of choice for evaluating the liver, biliary system, pancreas and spleen.
Cystic fibrosis in two Chinese infants in Taiwan.
Cystic fibrosis was diagnosed in two female infants, respectively nine months old (Case 1) and six months old (Case 2). Clinical presentation of these two infants showed frequent episodes of pneumonia, dehydration and poor weight gain, despite fair intake of food since birth. Hyponatremic, hypochloremic metabolic alkalosis had been found during their previous hospitalizations. In Case 1, stool fat was positive with negative trypsin test. Both cases had abnormal findings on chest X-ray films, but the pancreas showed no remarkable cystic changes from sonography. Plastic-bag method of sweat test showed Na/Cl: 155/185 nmol/L in Case 1, Na/Cl: 127/135 nmol/L in Case 2. A family history was suggested, since each one had an elder brother who had died in early infancy with similar clinical presentations. Both patients were put on special diet therapy as well as chest physical therapy. Case 1 died suddenly at 16 months of age. Autopsy revealed mucusplugged dilated ducts with atrophy of the exocrine portion of the pancreas. DNA analysis in Case 2 showed abnormal mutation point at 1898+5 G-->T on chromosome 7.
Establishment and characterization of a rat pancreatic stellate cell line by spontaneous immortalization.
AIM: Activated pancreatic stellate cells (PSCs) have been implicated in the pathogenesis of pancreatic fibrosis and inflammation. Primary PSCs can be subcultured only several times because of their limited growth potential. A continuous cell line may therefore be valuable in studying molecular mechanisms of these pancreatic disorders. The aim of this study was to establish a cell line of rat PSCs by spontaneous immortalization. METHODS: PSCs were isolated from the pancreas of male Wistar rats, and conventional subcultivation was performed repeatedly. Telomerase activity was measured using the telomere repeat amplification protocol. Activation of transcription factors was assessed by electrophoretic mobility shift assay. Activation of mitogen-activated protein (MAP) kinases was examined by Western blotting using anti-phosphospecific antibodies. Expression of cytokine-induced neutrophil chemoattractant-1 was determined by enzyme immunoassay. RESULTS: Conventional subcultivation yielded actively growing cells. One clone was obtained after limiting dilution, and designated as SIPS. This cell line has been passaged repeatedly more than 2 years, and is thus likely immortalized. SIPS cells retained morphological characteristics of primary, culture-activated PSCs. SIPS expressed alpha-smooth muscle actin, glial acidic fibrillary protein, vimentin, desmin, type I collagen, fibronectin, and prolyl hydroxylases. Telomerase activity and p53 expression were negative. Proliferation of SIPS cells was serum-dependent, and stimulated with platelet-derived growth factor-BB through the activation of extracellular signal-regulated kinase. Interleukin-1beta activated nuclear factor-kappaB, activator protein-1, and MAP kinases. Interleukin-1beta induced cytokine-induced neutrophil chemoattractant-1 expression through the activation of nuclear factor-kappaB and MAP kinases. CONCLUSION: SIPS cells can be useful for in vitro studies of cell biology and signal transduction of PSCs.