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At least 793 records · Page 44Linked to original sources
PubMed1983

Pancreatic structure and function in the immature reserpinized rat.

Immature rats were reserpinized to determine whether the model used for adults may be suitable for the study of pancreatic exocrine insufficiency seen in infants with cystic fibrosis. Rats were reserpinized by injections either into pregnant dams or into newborn rats. The dose of reserpine used by others was lethal to immature rats, so lower doses were used. Pancreas from 1-day-old fetal-treated pups was hypoplastic, but concentration of chymotrypsinogen was elevated. At age 7 days hyperplasia was seen. When rats were reserpinized as neonates, hypoplasia and decrease in all parameters measured was observed at age 7 days. Progressive recovery occurred during the following 2 weeks in both groups. Electron microscopic study of the fetal-treated 24-hr-old pancreas revealed evidence of acinar cell degeneration with the presence of abnormal zymogen granules. At age 7 days the pancreas from neonatal-treated rat pups appeared to have a reduced number of granules. At ages 14 and 21 days the pancreas was similar to that seen at age 7 days except that the granules were larger and some acinar lumina were filled with a finely granular, homogeneously dense material. It is concluded that prenatal and neonatal reserpinization of rats induces changes in pancreas similar to those found in cystic fibrosis.

Amylases↗
PubMed2006

MUC4 expression is regulated by cystic fibrosis transmembrane conductance regulator in pancreatic adenocarcinoma cells via transcriptional and post-translational mechanisms.

MUC4 mucin is a high molecular weight transmembrane glycoprotein that plays important roles in tumour biology. It is aberrantly expressed in pancreatic adenocarcinoma, while not being detectable in the normal pancreas. Previous studies have demonstrated that the cystic fibrosis transmembrane conductance regulator (CFTR), a chloride channel that is defective in CF, is implicated in multiple cellular functions, including gene regulation. In the present study, using a CFTR-defective pancreatic cancer cell line and its derived subline expressing functional CFTR, we report that MUC4 expression is negatively regulated by CFTR. Short-interfering RNA (siRNA)-mediated silencing of CFTR also leads to an increased expression of MUC4. Additionally, our results suggest that CFTR-mediated regulation of MUC4 is cell density-dependent and is achieved by transcriptional and posttranslational mechanisms. Moreover, in a panel of pancreatic cancer cell lines and normal pancreas, we observed that CFTR was downregulated in pancreatic cancer cells and negatively correlated with MUC4 in most cases. An aberrant expression of MUC4 was also detected in the CF pancreas. Downregulation of CFTR in pancreatic adenocarcinoma and its inverse association with the tumour-linked mucin, MUC4, indicate novel function(s) of CFTR in pancreatic tumour biology and suggest the implication of new signalling pathway(s) in MUC4 regulation.

Adenocarcinoma↗
PubMedDate not supplied

Nasal and paranasal sinus surgery in children with cystic fibrosis.

Cystic fibrosis (mucoviscidosis; fibrocystic disease of the pancreas) is an inherited autosomal recessive disorder that results in generalized dysfunction of exocrine glands. Chronic pansinusitis with nasal polyposis occurs frequently in this disease. Polypectomy and sinus drainage procedures are often required in those cases refractory to medical therapy. Recurrence of nasal polyps following removal is common. Controversy exists regarding the timing and extent of surgery. The management of 40 children with cystic fibrosis who underwent nasal polypectomy, intranasal ethmoidectomy, and Caldwell-Luc procedures to treat extensive sinusitis and nasal polyposis forms the basis for this report. Follow-up data indicate that fewer recurrences and longer symptom-free intervals result when intranasal ethmoidectomy and Caldwell-Luc procedures are combined with polypectomy.

Child↗
PubMed1989

Light and electron microscopy of the exocrine pancreas in the chronically reserpinized rat.

The effects of reserpine injections were studied on the morphology of the pancreas in an experimental model for cystic fibrosis, the chronically reserpinized rat. A detailed examination of the tissue was carried out at the light and electron microscopic levels. The nonspecific effects of secondary malnutrition induced by the drug were assessed with a group of animals pair fed with the treated animals. In a companion paper, we show that pancreatic wt, lipase, and GP-2 contents also are affected by reserpine treatment. In this study, we report that no morphologic differences were observed between the exocrine pancreatic tissue of control and pair-fed animals. By contrast, reserpine induced an accumulation of zymogen granules in 60% of the treated animals and a concomitant decrease of the area occupied by the rough endoplasmic reticulum in the same cells. Finally, in all treated animals, at the light and electron microscopic levels, it was observed that some particular regions of the pancreatic tissue were strongly affected. In these regions, numerous autophagic bodies and lysosomes were observed. Cisternae of the Golgi complex were also more distended. Some acinar cells were in the process of lysis. Several vacuolar inclusions were present in some intralobular duct cells. Cellular material was seen in acinar and intralobular duct lumina. In these same regions, distended intralobular ducts and acinar lumina were observed. These last two features have never been reported in the reserpinized rat but are important manifestations of the pathology in cystic fibrosis patients where obstructions of ducts are believed to trigger focal destruction of the pancreatic tissue.(ABSTRACT TRUNCATED AT 250 WORDS)

Animals↗
PubMedDate not supplied

Cystic fibrosis and the gastrointestinal tract.

Cystic fibrosis is known to be a serious genetic disease among those of northern European ancestry. Well known to nurses are the pulmonary insults to survival for children with cystic fibrosis. An aspect of health care not often found in the nursing literature is a focus on cystic fibrosis gastrointestinal presentation. Because of the chronic nature of cystic fibrosis, those providing health care to children must have an understanding of the disease in the growing infant and child. An overview of cystic fibrosis and the possible effect on the pancreas, gut, and liver are reviewed. Areas of developmental concern to the pediatric nurse practitioner are listed.

Adolescent↗
PubMed1998

Cystic fibrosis: present and future.

Cystic fibrosis (CF) is an inherited disorder of epithelial chloride transport affecting primarily pancreas, lungs, gut, liver and exocrine glands. The defect is caused by defects of the cystic fibrosis transmembrane regulation gene on chromosome 7. Genotyping has proved useful in identifying gene carriers, a definitive diagnosis, and in antenetal diagnosis. Genotype/phenotype relationships have shown that the commonest cause of pancreatic insufficiency is the D F508 mutation. Clinical trials are exploring the use of somatic gene therapy but this is not yet a viable treatment option. Liver, lung and intestinal disease result in malnutrition which causes further dysfunction of these organs. Aggressive nutritional and pancreatic enzyme therapy results in improved disease, normal growth and increased survival. However, high-dose enzyme therapy may in some individuals cause a fibrosing colonopathy. For those with end-stage liver and lung disease, transplantation holds out some hope.

Chromosome Aberrations↗
PubMed2006

Delayed response toward activation stimuli in pancreatic stellate cells.

OBJECTIVES: Pancreatic stellate cells (PSCs) are known to be crucially involved in the development of pancreatic fibrosis, a characteristic feature of chronic pancreatitis and pancreatic cancer. The key event in the pathogenesis of fibrosis represents a transition process of quiescent PSCs into a myofibroblastlike phenotype associated with cell activation in terms of proliferation and synthesis of profibrogenic substances. There is little information available regarding the dynamics of the complex processes initiated by an activating stimulus in quiescent stellate cells. METHODS: Using microarray analysis, we characterized the expression profiles during PSC activation caused by in vitro cultivation on days 2, 4, 7, and 14 after cell isolation. Activation status has been identified by the expression of the activation marker alpha-smooth muscle actin. Genes of interest were subjected to reverse transcription-polymerase chain reaction. To test biologic functions, the responsiveness of stellate cells toward the activators activin A and transforming growth factor-beta1 was investigated in dependence on the cell transition status. RESULTS: Our results revealed that freshly isolated (=quiescent) PSCs were refractory to stimuli for several days, a phenomenon that we referred to as delay phase. CONCLUSION: The retarded response could be considered a protection mechanism to prevent inappropriate stellate cell activation.

Animals↗
PubMedDate not supplied

[Fibrocystic disease of the pancreas: a presentation feature. Anatomopathological report].

Five patients with CF (cystic fibrosis) dead between 1974 to 1982 at ages ranging from one to six months are presented. All of them showed edema, hypoalbuminemia and anemia in a severely compromised clinical situation, and failure to gain weight in spite of being breast-fed in the first weeks of life, in four of them. All of them were second or third degree malnourished babies (Gomez classification) at admission. Five children presented edema, two severe, two moderate and one mild. Hematocrit values ranged from 19% to 39% (means 26.4%), and albuminemia from 1.60 to 3.00 g/% (means 2.14 g/%). Two patients presented antecedents of dead brothers. All of them received substitution therapy with pancreatic enzymes. The children dead within seven and seventeen days of admission (means ten days) of broncho-pulmonar disfunction. In this work, we wish to call the pediatrician's attention about the importance of making this diagnostic presumption in the first months of the life.

Bronchi↗
PubMedDate not supplied

[Cystic fibrosis in adolescent patients].

Summary--An autopsy observation of mucoviscydosis in a female who died at the age of 17 with a prevalent affection of the bronchopulmonary system is reported. Pronounced cystic fibrosis with atrophy and lipomatosis of the pancreas was found. The cause of death was respiratory failure in spite of intensive antibacterial therapy. Bullous emphysema with frequent pneumothorax in adults with this disease considerably worsens the prognosis and requires urgent therapy which in this case was not conducted because of late hospitalization.

Adolescent↗
PubMed1985

Prenatal diagnosis of cystic fibrosis by trehalase enzyme assay in amniotic fluid.

Amniocentesis and amniotic fluid trehalase enzyme assay were offered to 14 pregnant women at a 1 in 4 risk for a child with cystic fibrosis. Twelve of these pregnancies were screened at the 18th week of gestation; ten proceeded to term, seven following the finding of a normal trehalase activity and three despite the low enzyme level in amniotic fluid. In all ten cases prenatal diagnosis proved to be correct. In two cases with low enzyme activity parents opted for termination at the 19th week, and with PAS-Alcian Blue staining some slight histochemical lesions characteristic of cystic fibrosis were seen in the exocrine glands, including the pancreas and intestinal mucosa, of both fetuses. The total protein content in the meconium of these fetuses was significantly higher than in the controls. Results suggest that trehalase assay in the amniotic fluid is a potential prenatal test for cystic fibrosis and it appears that in fetuses with cystic fibrosis some histochemical and biochemical abnormalities can be observed as early as the 19th week of gestation. The role of ultrasound examination as an additional procedure for the prenatal diagnosis of cystic fibrosis is also discussed.

Amniotic Fluid↗