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PubMed1965

THE PANCREAS. IV.

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Amylases↗
PubMed2000

[Diagnosis and management of cystic fibrosis in children].

Cystic fibrosis is a genetic disease occurring more frequently in Caucasians. The cystic fibrosis gene, cloned in 1999, codes for the cystic fibrosis transmembrane conductance regulator (CFTR) protein. Dysfunction of this protein leads to the clinical manifestations of cystic fibrosis, mainly lung disease and exocrine pancreas disorders. The pathophysiology of the respiratory component is quite complex, basically related to major and excessive inflammatory processes and to early microbial colonization. Respiratory physical therapy is a key element to management of the respiratory disorder. Antibiotic treatments should be adapted to the bacterial ecology, mainly using antistaphylococcal and antihaemophilus drugs initially, then directed against Pseudomonas aeruginosa. Other drugs including inhaled antiinflammatory drugs are currently in the evaluation stage. In addition, nutritional care and correction of pancreas insufficiency are necessary. The diagnosis of this disease must be made early although systematic neonatal screening is not proposed. Early diagnosis is necessary for improved care and prognosis. Currently, median survival is 29 years. This survival time should probably improve with better understanding of the pathophysiological mechanisms and new therapeutic perspectives.

Adult↗
PubMed1996

Cystic fibrosis: MR assessment of pancreatic damage.

PURPOSE: To evaluate magnetic resonance (MR) imaging for assessment of pancreatic damage in cystic fibrosis. MATERIALS AND METHODS: Twenty-seven patients with cystic fibrosis and 12 control subjects underwent T1-weighted imaging for visual assessment for pancreatic hyperintensity and mixed spin-echo-inversion-recovery imaging for quantitative measurement of T1. Pancreatic insufficiency, pulmonary status, and genotype were recorded. Statistical correlation was conducted. RESULTS: Four patterns of pancreatic involvement were noted: diffuse hyperintensity with a lobular pattern, diffuse homogeneous hyperintensity without residual lobular pattern, hyperintensity with focal areas of sparing, and no structural or signal intensity changes. Statistically significant reduction of the T1 was noted in 21 patients compared with that of controls. Statistically significant correlation between T1 shortening and pulmonary clinical-radiologic compromise and pancreatic insufficiency was found. CONCLUSION: MR imaging may offer further indication of pancreatic and, indirectly, pulmonary damage during the clinical course of cystic fibrosis. A pattern of fibrofatty infiltration of the pancreas, of undetermined clinical significance, is reported.

Adolescent↗
PubMed2001

[From gene to disease; from defective chloride ion transport to cystic fibrosis].

Cystic fibrosis is an autosomal recessive disorder affecting the lungs, pancreas, intestines, sweat ducts and liver, due to an abnormal salt transport across the apical border of epithelial cells. Mutations in the CF underlying gene, the cystic fibrosis transmembrane conductance regulator (CFTR) gene, result in most cell types in an misprocessing so that little of the protein reaches the membranes. In case of clinical suspicion and/or doubtful sweat test results, mutation analysis can support the diagnosis of CF. Also carrier detection is offered.

Adult↗
PubMed1977

Environmental factors and diseases of the pancreas.

The five major diseases of the pancreas together make a significant contribution to morbidity and mortality among the people of the United States. These diseases are diabetes, cystic fibrosis, acute and chronic pancreatitis, and carcinoma of the exocrine pancreas. Four of these diseases can be modeled in laboratory animals by acute or chronic administration of chemical poisons or carcinogens. Human pancreatic diseases attributed to the effect of chemical agents including alcohol and drugs include many cases of chronic pancreatitis and some cases of acute pancreatitis. The cause is not known in many cases of human pancreatitis, including interstitial, acute, and chronic clinical forms. Epidemiologic studies suggest that the increasing incidence of carcinoma of the exocrine pancreas in the United States may reflect chemical carcinogenesis. On the basis of experimental observations, we know that pancreatic islet cells can be damaged directly by toxic chemicals, and that islet cell tumors can be chemically induced. Thus, there is adequate background data to conclude that several pancreatic diseases of obscure etiology may be due in part to hitherto unidentified toxic effects of chemical agents encountered in personal or general environments.

Aging↗
PubMed1995

Increased expression of sulfated gp300 and acinar tissue pathology in pancreas of CFTR(-/-) mice.

The CFTR (-/-) mouse model of cystic fibrosis (CF) has revealed that the mouse pancreatic duct has a Ca(2+)-regulated chloride conductance that allows ductal electrolyte transport to remain unaffected by loss of the cystic fibrosis transmembrane conductance regulator (CFTR). Therefore, this model provides a unique opportunity to investigate effects of CF on the acinar tissue. It has been reported that exocrine secretions contain higher levels of sulfate in CF. We discovered in CFTR(-/-) acini that gp300, the major sulfated glycoprotein of the mouse acinar cell, has increased steady-state and biosynthetic levels. However, there are no apparent changes in sulfate or carbohydrate composition of gp300, indicating that posttranslational processing of this sulfated glycoprotein is not altered in CF. In addition to the increase in gp300, the morphology of CF acinar tissue is dramatically altered: acinar lumina of CFTR(-/-) mice are greatly dilated and filled with aggregated protein. gp300, which in the normal tissue is mainly localized to the zymogen granule membrane, was found to line the distended luminal membranes in the CF tissue. These results demonstrate that the acinar tissue is affected in the CF mouse and that expression of the major sulfated glycoprotein is also increased. It is suggested that increased expression of gp300 in CFTR(-/-) mice may cause poorly soluble exocrine protein secretion, contributing to the development of CF in the pancreas.

Animals↗
PubMed1976

Galactosemia with endogenous production of galactose-1-phosphate and with cystic fibrosis-like appearance at autopsy.

In an infant with galactosemia high levels of galactose-1-phosphate in red blood cells and of blood galactose were observed under a "galactose-free'' diet. The child did not thrive and developed a liver cirrhosis. At the age of 5 months he died unexpectedly. Post mortem examination revealed in the pancreas and the small intestine changes suggestive of a cystic fibrosis. Since the exogenous administration of galactose by diet could be excluded the endogenous production of significant amounts of galactose-1-phosphate has to be considered.

Autopsy↗