ROENTGENOGRAPHIC SPECTRUM OF CYSTIC FIBROSIS.
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Child↗
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Results for “CYSTIC FIBROSIS OF PANCREAS”
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Disorders of the pancreas in children.
Diseases of the pancreas in children are often congenital; the genetic basis of some conditions such as cystic fibrosis have been elucidated using molecular biologic techniques. Anatomic abnormalities in childhood that cause symptomatic pancreatic disease are usually treated surgically. The biochemical abnormalities found with enzyme deficiencies, CF, and Shwachman's syndrome are best treated with medical therapy. The most common cause of acute pancreatic injury in children is trauma. Chronic pancreatic insufficiency usually occurs secondary to CF.
Acute Disease↗
Meconium ileus secondary to partial aplasia of the pancreas: report of a case.
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Abnormalities, Multiple↗
Inherited causes of exocrine pancreatic dysfunction.
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.
Adult↗
Cyclic AMP-related and cation-affected human platelet chloride transport regulation.
Cystic fibrosis has been characterized as a defect in the regulation of cyclic AMP-dependent transepithelial chloride transport. The activation of cyclic AMP-dependent protein kinase A by cyclic AMP occurs normally in cystic fibrosis cells, but they fail to transport chloride ions in response to protein kinase A stimulation. Defective chloride secretion and abnormal electrolyte transport occurs in several organs including the lung, sweat glands, intestine and pancreas. The present work was aimed at exploring whether the same or similar regulatory systems are functional in platelets, and if they are altered or deficient in individuals with cystic fibrosis. Chloride transport in platelets from normal subjects and from cystic fibrosis patients was measured by cell sizing techniques where chloride permeability is the limiting factor. In platelets from healthy volunteers, the chloride channel blocker, 5-nitro-2-(3-phenylpropylamino) benzoic acid, inhibits the transport in a dose-dependent manner. The preservation of chloride transport capability is shown to be dependent upon the presence of either Ca2+ or two divalent cation substitutes, Cd2+ or Cu2+. It is also shown that in normal subjects 0.1 mumol/l prostaglandin E1, which elevates cyclic AMP 6 times and abolishes platelet aggregation, significantly enhances the rate constant of the transport. Furthermore, in five out of nine cystic fibrosis patients studied, platelet chloride transport did not respond to stimulation by prostaglandin E1.
Adolescent↗
Intestinal atresia or stenosis in the newborn associated with fibrocystic disease of the pancreas.
In a review of cases of intestinal atresia or stenosis in the newborn at Children's Hospital of Los Angeles it was noted that in approximately 10 per cent there was clinical or anatomic evidence of fibrocystic disease of the pancreas. Histologic sections of the bowel in all these cases showed the alterations of the mucosa commonly found in fibrocystic disease. Extensive ulceration, foreign body reaction and calcium deposition in the bowel wall were observed in five cases. Sections through the site of obstruction in one patient showed narrowing of the diameter of the bowel with preservation of muscular layers, but replacement of the central portion by a vascular, fibrous diaphragm in which giant cells, hemosiderin and calcium were prominent. This suggested that in certain instances obstruction of the intestine may be caused by fibrosis secondary to injury of the mucosa by abnormal meconium. Awareness of the common association of intestinal obstruction and fibrocystic disease of the pancreas and recognition of the histologic change in the bowel in fibrocystic disease may lead to early diagnosis of this disorder in some infants with intestinal obstruction.
Child↗
[Physiopathology of the exocrine pancreas in children].
The mechanism leading to exocrine pancreatic disease in children differs from those encountered in adult patients: I. In acute pancreatitis autodigestion of the gland by proteolytic enzymes may occur and two mechanisms may play a role. 1. Reflux of biliary secretions (e.g. in malformations of the duct system) facilitates activation of trypsinogen by enteropeptidase and leads to the presence of active proteolytic enzymes in the gland (exogenous activation). 2. Lysosomal enzymes may play a role in the intracellular activation of zymogens if inflammation leads to a fusion of lysosomes with zymogen granules (endogenous activation). II. In chronic relapsing and hereditary pancreatitis malformations of the pancreatico-biliary duct system must be sought because surgery may be indicated (common channel syndrome and choledochal cysts). III. Among the hereditary diseases leading to pancreatic insufficiency cystic fibrosis (CF) plays the main role. Haplotype analysis has shown that two genetically different types of CF exists (PS and PI). The pancreas shows manifest insufficiency only in the PI-types which occur in more than 70% of cases but the distribution of haplotypes is different in different ethnic groups. In spite of the recent discovery of the cystic fibrosis gene the exact mechanism leading to exocrine pancreatic dysfunction in CF is not clear, but diminished chloride and bicarbonate secretion, may be the result of a disturbance in the regulation of chloride channels, on acinar or ductular level. In the Shwachman-Diamond syndrome a very severe type of exocrine insufficiency with unknown etiology is encountered at birth.
Acute Disease↗
Cystic fibrosis: its characteristic appearance on abdominal sonography.
The abdominal sonograms of 24 patients with cystic fibrosis aged 8-30 years were reviewed. Most patients had abnormal scans; the number and severity of organs involved tended to increase with age. When the pancreas was identified, it was usually echogenic and small. Cholelithiasis, microgallbladder, and obstruction of the common bile duct were observed. Textural changes in the liver reflected underlying parenchymal disease; splenomegaly and portal hypertension occurred in responses to severe hepatic involvement. A few patients had thick gastric or duodenal wall.
Abdomen↗
An approach for treating the hepatobiliary disease of cystic fibrosis by somatic gene transfer.
Cystic fibrosis (CF) is an inherited disease of epithelial cell ion transport that is associated with pathology in multiple organ systems, including lung, pancreas, and liver. As treatment of the pulmonary manifestations of CF has improved, management of CF liver disease has become increasingly important in adult patients. This report describes an approach for treating CF liver disease by somatic gene transfer. In situ hybridization and immunocytochemistry analysis of rat liver sections indicated that the endogenous CFTR (cystic fibrosis transmembrane conductance regulator) gene is primarily expressed in the intrahepatic biliary epithelial cells. To specifically target recombinant genes to the biliary epithelium in vivo, recombinant adenoviruses expressing lacZ or human CFTR were infused retrograde into the biliary tract through the common bile duct. Conditions were established for achieving recombinant gene expression in virtually all cells of the intrahepatic bile ducts in vivo. Expression persisted in the smaller bile ducts for the duration of the experiment, which was 21 days. These studies suggest that it may be feasible to prevent CF liver disease by genetically reconstituting CFTR expression in the biliary tract, using an approach that is clinically feasible.
Adenoviridae↗
[Acute pancreatitis in cystic fibrosis].
Cystic fibrosis is the most prevalent hereditary disease in the Caucasian race. It is a multisystemic alteration that affects the quality and quantitative properties of exocrine secretions. The pancreas develops a progressive atrophy causing steatorrhoea and nutritive deficiencies. Acute pancreatitis is an unusual complication. The pancreatic atrophy prevents the inflammatory response. Published series suggest that pancreatitis in 0.5%, including patients without pancreatic insufficiency. We present two cases with cystic fibrosis, with and without pancreatic insufficiency, who developed acute pancreatitis.
Acute Disease↗
Coordinate upregulation of guanylin and uroguanylin expression by hypertonicity in HT29-18-N2 cells.
Guanylin and uroguanylin are particulate guanylate cyclase-activating peptides that are secreted from the epithelia of the intestine, kidney, pancreas, and salivary gland. These peptides elicit chloride and bicarbonate secretion via the cystic fibrosis transmembrane conductance regulator. To test the hypothesis that hypertonicity mediates an increase in guanylin and uroguanylin mRNA, we subjected HT29-18-N2 to osmotic stress. Guanylin and uroguanylin RNA were increased substantially in the presence of hypertonicity but only with solutes that were relatively impermeable to the cell membrane. This hypertonicity-mediated increase was transcriptional and did not require protein synthesis. Herbimycin A and mitogen-activated protein kinase inhibitors SB-203580 and PD-98059 had no effect on basal or induced levels of guanylin or uroguanylin. Both staurosporine and prolonged exposure to phorbol ester reduced basal levels and completely blocked hypertonicity-related increases in guanylin or uroguanylin RNA. These data suggest that serine/theonine protein kinases, possibly protein kinase C (PKC), mediate the hypertonicity-associated increase in guanylin and uroguanylin RNA. We conclude that guanylin and uroguanylin are released in response to hypertonic stress and that regulation of these genes may be mediated by PKC isoforms.
Betaine↗
[Pancreatic extract therapy in exocrine pancreatic insufficiency].
The authors consider the different strategies in long term enzyme replacement therapy in relation to the complex mechanics at the basis of pancreas exocrine insufficiency. This condition occurs in chronic pancreatitis and is present in Cystic Fibrosis, the most common potentially lethal inherited disorder of Caucasians. Pancreatic exocrine insufficiency occurs in the vast majority of cystic fibrosis affected children and is now becoming a frequent pathology in adults for the better life expectancy and the recent acknowledgements in this disease. The principal aims of research in enzyme replacement therapy have been directed at the formulation of products with high enzyme concentration, to the prevention of gastric acid inactivation of enzymes and to the better mixing of the preparations with meals. The authors consider all the different enzyme preparations from pancreatin powder to the 1st. generation of enteric coated tablets and examine the advantages of administering H-2 receptor antagonists or antacids and the possibility of stimulating bicarbonate secretion as an adjunct to pancreatic enzyme replacement therapy. Significant benefits in pancreatic insufficiency therapy have derived from the introduction of enteric coated microspheres which ensure a consistent level of enzymes to reach the duodenum mixed with the meal and which are resistant to gastric acid inactivation as well.
Exocrine Pancreatic Insufficiency↗
Serum lipase levels pre and post Lundh meal: evaluation of exocrine pancreatic status in cystic fibrosis.
Determination of pancreatic function is essential in cystic fibrosis. The most-reliable method is by measuring pancreatic enzymes in the duodenum following intravenous or oral stimulation. However, this is invasive, time consuming, and expensive. Indirect tests are non-invasive but lack accuracy. This study examines a simple test which combines pancreatic stimulation by Lundh meal and sequential serum lipase measurements. The test was performed on three groups: group A, 36 cystic fibrosis patients carrying two mutations associated with severe disease and pancreatic insufficiency (delta F508, W1282X, G542X, N1303K, S549R); group B, 8 compound heterozygote cystic fibrosis patients carrying one mutation causing mild disease with pancreatic sufficiency (3849 + 10 kb C-->T); group C, 17 healthy individuals. Basal lipase levels were 2-16.5, 16.4-73, and 8.5-27.8 U/l in groups A, B, and C, respectively, with some overlapping between groups. There were three patterns of lipase activity (1) consistently low levels (group A) suggested a severely affected insufficient pancreas; (2) normal basal levels followed by a linear rise peaking 30 min after the meal (found in 16 of 17 healthy individuals and 3 patients of group B) reflecting an unaffected sufficient pancreas; (3) elevated lipase levels not influenced by the meal (5 patients of group B). This reflects an ongoing destructive process in the pancreas which will eventually result in conversion from pancreatic sufficiency to pancreatic insufficiency. Hence serum lipase activity prior to and 30 min after Lundh meal is a good indicator of pancreatic status allowing categorization of cystic fibrosis patients as pancreatic insufficient, pancreatic sufficient, or pancreatic sufficient with late conversion to insufficiency.
Adolescent↗
[The male genital tract in fibrocystic disease of the pancreas].
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Child↗
Pancreatic diseases (excluding cystic fibrosis).
Except for cystic fibrosis, which is the most frequent genetic disorder in the Caucasian population, diseases of the exocrine pancreas are relatively uncommon in children. However, they are many and varied in terms of their pathogenesis and clinical manifestation. They can be classified as: (1) congenital anatomical abnormalities, (2) congenital secretory insufficiencies, and (3) pancreatitis. In all of these diseases, when pancreatic insufficiency (whether partial or complete) is present, the nutritional status of the patients must be investigated regularly, and pancreatic enzymes as well as nutritional supplementations must be prescribed as soon as malnutrition is present, or even prophylactically. The preservation of good nutritional status is the guarantee of a better prognosis.
Child↗
Immunocytochemical localization of elastase 1 in human pancreas.
By light and electron microscopic immunocytochemistry the distribution is described of human pancreatic elastase 1 (E1) during ontogenesis, in adults, in cases of acute and chronic pancreatitis, acute pancreatic ischaemia as well as pancreatic tumours. E1-positive cells were first detected in ductal sprouts in the 14th gestational week. Complete acini expressing E1 could be found from the 17th to the 20th week of gestation onwards. Scattered distinct E1-positive epithelia could be found in the ducts of fetal and adult pancreas. By immunoelectron microscopy, E1 was localized in rough endoplasmic reticulum, condensing vacuoles, zymogen granules of acinar epithelia and in acinar lumina. E1 appeared to be distributed homogeneously in zymogen granules. As specific markers of acinar cells, both monoclonal antibodies under study identified heterotopic pancreatic acini in peribiliar glands of the liver and also helped to visualize different damage patterns in pancreatitis. The acinar epithelia surrounding acute lipolytic necroses initially reacted more intensely with the E1-antibodies than undamaged pancreatic tissue. In acute ischaemia, acinar cells which are dissociated from intercalated ducts lost their immunocytochemical reactivity for E1. Pancreatic parenchyma involved in advanced acute pancreatitis as well as in chronic inflammation was detected only weakly by both E1-antibodies. However, atrophic lobules in post-inflammatory scars were stained more intensely by the E1-antibodies than normal parenchyma. Pancreatic tumours (adenomas, adenocarcinomas, solid-cystic tumours and islet cell tumours) were not labelled by these antibodies.
Acute Disease↗
Alterations of pancreatic growth and of GP-2 content in the reserpinized rat model of cystic fibrosis.
The chronically reserpinized rat is an experimental model for cystic fibrosis. In this study, we report the effects of two doses of reserpine (0.5 and 1.0 mg.kg-1.d-1) on the growth of the pancreas and on its content of the glycoprotein GP-2, a characteristic protein of the zymogen granule. An assessment of the effects of secondary malnutrition induced by the drug was also performed by adding a group of pair-fed animals. During the 7 d of treatment, body wt and food intake were monitored. These two parameters were significantly affected from the 4th d on. Pancreatic wt, DNA, protein, and activity of amylase and chymotrypsinogen were measured after 4 and 7 d of treatment; lipase activity and GP-2 content, after 7 d. Although the DNA content never did change, total protein diminished by 27% at the higher dose of reserpine. Pancreatic wt, amylase activity and GP-2 content were reduced by the treatment, while chymotrypsinogen and lipase activities were increased. Effects on pancreatic wt, amylase, chymotrypsinogen, and GP-2 were dose-dependent. Malnutrition had effects similar to reserpine on body wt, protein, amylase, and chymotrypsinogen. Pancreatic wt, lipase, and GP-2, however, were specifically altered by the chronic reserpine treatment. It is concluded from these results that reserpine induces, in the pancreas, specific alterations that are distinguishable from the accompanying malnutrition. These findings support the use of pancreatic wt, lipase, and GP-2 as specific markers of the effects of the drug on the pancreatic tissue in the chronically reserpinized rat model for cystic fibrosis.(ABSTRACT TRUNCATED AT 250 WORDS)
Animals↗
Cystic fibrosis - ultrastructural and microanalytical studies.
This review summarizes the contribution of electron microscopy and X-ray microanalysis to the study of the pathogenesis and the diagnosis of cystic fibrosis (CF). Light and electron microscopical studies of the most severely affected exocrine glands of CF patients (pancreas, tracheobronchial submucosal glands) generally show dilatation of the ducts and inspissation of mucous material. The ultrastructure of the acinar cells, however, is normal. Functional abnormalities in the gland can be demonstrated by X-ray microanalysis of the secreted fluid. In the search for the basic defect in CF use is often made of cultured fibroblasts. The ultrastructure of CF fibroblasts is basically normal. X-ray microanalysis shows significantly lower Na and higher Ca levels in CF fibroblasts. Several of the biological effects of the 'CF factor(s)', supposedly present e.g. in the serum of CF patients or in CF fibroblast culture medium, can be demonstrated by electron microscopy or X-ray microanalysis. Ultrastructural and X-ray microanalytical studies of 'animal models' for CF have shown that pathological changes in exocrine glands resembling some of those seen in CF patients, can be induced by interference with the nervous regulation of exocrine secretion.
Animals↗