The late intestinal and hepatic complications of fibrocystic disease of the pancreas (mucoviscidosis).
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Adult↗
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Results for “CYSTIC FIBROSIS OF PANCREAS”
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Sweat test for the diagnosis of fibrocystic disease of the pancreas; report of a fatality.
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Cystic Fibrosis↗
Cimetidine and fat malabsorption in children with cystic fibrosis.
Under controlled clinical observation we administered Cimetidine (600 mg/m2/day) in 10 children with cystic fibrosis. All patients received identical diets (40% fat, 45% carbohydrate, 15% protein) as well as same standardized enzyme preparation (lyophilised whole pancreas). 3-day fecal fat and nitrogen excretion analyses were performed. After 6 days of Cimetidine therapy, an improvement of fat and nitrogen absorption could be detected in 5 patients. Statistical analysis of the group as a whole showed no improvement in fat absorption coefficients. We conclude that this drug therapy should only be recommended for routine clinical use after long-term trials have shown a real improvement of absorption.
Adolescent↗
Skipping of exon 9 in CFTR mRNA of human adult and fetal pancreas from non-CF individuals.
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Adult↗
Progress in the study of pancreatic cancer and its pathology.
Recent advances in diagnostic techniques allow detection of relatively small ductal cancers and mucinous cystic neoplasms of the human pancreas. The histopathological characteristics of a series of such small pancreatic carcinomas encountered in our hospital were: 1) all were invasive ductal carcinomas accompanied by fibrosis, 2) four of seven carcinomas were thought to originate in the branch ducts, 3) no precancerous lesions were found, and 4) lymph node metastasis was detected in only one patient. Mucinous cystic neoplasms were of two types: megacystic and ductectatic. The megacystic type is the classical mucinous cystic neoplasm. The ductectatic type is characterized by the presence of multilocular cysts (reminiscent of bronchiectasia of the lung), and papillary proliferation of the lining epithelium-differing from typical ductal carcinomas as well as from megacystic lesions in terms of morphological features, intrapancreatic location, patient age, and sex. The ductectatic type occurs exclusively in the head and body of the pancreas, and is found predominantly in aging males.
Adult↗
Cystic fibrosis.
Cystic fibrosis, an autosomal recessive disorder, is the most common genetic disease of Caucasians. One in 25 Caucasians are carriers of the gene. The gene is found far less commonly in other races. There are over 230 different alleles of the gene, located on the 7th chromosome. The gene encodes for a membrane protein that functions as an ion channel. The survival of cystic fibrosis patients has been gradually increasing, with a mean survival in 1990 of 28 years. If the current trend of improved survival continues, it is estimated that half of cystic fibrosis patients will be over 18 years old by 1996. Disease is found in many organs including the lungs, sinuses, pancreas, gastrointestinal tract, hepatobiliary system, sweat glands and reproductive tract. The majority of patients die of pulmonary disease. The airways become chronically colonized with bacteria that cannot be eradicated, leading to bronchitis, bronchiectasis, and finally, pulmonary fibrosis with respiratory failure. The pulmonary disease may be complicated by massive hemoptysis and pneumothorax. Patient survival rates have increased because of antibiotic therapy and improved nutrition with pancreatic enzyme replacements. New treatments for the pulmonary disease are under clinical trial and include antiproteases, amiloride, a sodium channel blocker, and DNase. The insertion of the normal cystic fibrosis allele into an animal model using a modified adenovirus with effective transcription suggests that gene therapy may be possible in the future, but safety and technical problems have to be addressed.
Cystic Fibrosis↗
Disturbance of fluid and electrolyte transport in cystic fibrosis epithelia.
Studies of the disease CF suggest that the basic defect is related to impaired electrolyte movement in the epithelia of a variety of organs with exocrine function. The disturbances of electrolyte secretion in the organs classically involved in CF range from 1) a decrease in secretion- or uptake of chloride ions in all the organs studied; 2) an increase in sodium uptake in nasal airway epithelium and 3) a decrease in bicarbonate output of the pancreas. In this review an overview is presented of the expression of the CF defect, the abnormalities of fluid and electrolyte secretion in each CF affected organ are considered in more detail with particular emphasis on the hormonal and neuronal (dys)regulation of iron transport systems in epithelial cells.
Biological Transport↗
Renal dysplasia and asplenia in two sibs.
A family is reported in which two sibs, one male and the other female, both died within 24 hours of birth with enlarged polycystic kidneys. Postmortem histology in the second child showed gross renal dysplasia. In both children the pancreas was enlarged, nodular and cystic but the liver appeared macroscopically normal. In the second child, histological examination confirmed pancreatic fibrosis with cystic dilation of ducts, but showed portal fibrosis with bile duct proliferation in the liver. This combination of findings is very reminiscent of those in a girl and her brother reported by Ivemark et al. (1959). The children reported here also showed absence or hypoplasia of the spleen, cardiac anomalies and other features of the Ivemark syndrome (Ivemark 1955), a quite different, usually sporadic, congenital disorder. It is suggested that the children described here have a distinct lethal congenital disorder, probably inherited in an autosomal recessive manner.
Abnormalities, Multiple↗
Normal human pancreas cultures display functional ductal characteristics.
BACKGROUND: Normal cell cultures are invaluable in the analysis of cell differentiation and neoplastic transformation. EXPERIMENTAL DESIGN: We have developed methods to reproducibly culture normal pancreas epithelial cells. The characteristics of the cultures were analyzed using ultrastructural methods, antibodies, and cDNA probes detecting epithelial differentiation markers. RESULTS: Normal pancreas tissue (N = 56) was obtained from organ donors; isolation of highly enriched exocrine fraction consistently yielded epithelial cultures. In vitro proliferation assays revealed a lag growth phase of 2 days followed by a proliferative phase until 8. Epithelial cells formed a polarized monolayer displaying apical microvilli, tight junctions, and desmosomes. Zymogen granules were not observed. A panel of mouse monoclonal antibodies detecting differentiation antigens of epithelial cells was used to determine the phenotype of the cultures: all cells expressed cytokeratin polypeptides of simple epithelial (CK 7, CK 8, CK 18, and CK 19), whereas polypeptides typical of stratified epithelial (CK 5, CK 10, CK 13, and CK 16) were not detected. Cultured cells expressed the MUC1 apomucin as well as mucin-associated carbohydrate epitopes. Expression of the cystic fibrosis transmembrane regulator was demonstrated at the RNA level. Secretin induced a very high stimulation of cAMP levels. CONCLUSIONS: The ultrastructural characteristics, molecular markers, and hormone responsiveness of the cultures suggest a ductal cell phenotype. These cultures should be useful in the analysis of pancreas growth and differentiation.
Adult↗
Early changes in the exocrine pancreas of the dog and rat after ligation of the pancreatic duct. A light and electron microscopic study.
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Acid Phosphatase↗
The chronically reserpinized rat as a model for cystic fibrosis: alterations in pancreatic enzyme secretion and storage.
Alterations in the pancreatic secretion of fluid and of enzymes in response to either pilocarpine (15 mg/kg) or an octapeptide of cholecystokinin (0.1 microgram/kg) have been found in rats that received daily injections of reserpine (0.5 mg/kg) for 7 days. During a 3-hr secretory period, significant reductions in the volume of pancreatic juice and in the total output of protein, amylase, and trypsin were observed in these animals. In the first hour of the secretory response, however, protease output was increased in the treated animals, particularly that of chymotrypsin, which was also increased in the longer secretory period following pilocarpine, but not cholecystokinin, stimulation. Zymogen granules isolated from the pancreas of the treated rats by differential centrifugation in a 0.3 M sucrose buffer had increased specific activities of the proteases when compared to those of untreated controls. Ultrastructurally, zymogen granules isolated from the pancreas of the treated rats showed changes in density, with bizonal and trizonal configurations being frequently observed, and had less distinct limiting membranes. In some, the membrane appeared broken at intervals, and there was granular material, presumably derived from the granule contents, lining the surface of the granule. It is concluded that pretreatment with reserpine inhibits fluid secretion and alters enzyme secretion in the rat exocrine pancreas. The latter effect is related to a nonparallel storage of amylase and proteases in the secretory granules induced by the drug treatment, probably through an action on protein synthesis or intracellular transport. An accumulation of proteases may lead to activation of these enzymes and to granule lysis. Inasmuch as the reserpine-treated rat has been proposed as an experimental model for cystic fibrosis, these findings are relevant in terms of possibly pathogenetic mechanisms in this disease.
Amylases↗
Distribution of aquaporin water channels AQP1 and AQP5 in the ductal system of the human pancreas.
BACKGROUND: The exocrine pancreas secretes large volumes of isotonic fluid, most of which originates from the ductal system. The role of aquaporin (AQP) water channels in this process is unknown. METHODS: Expression and localisation of known AQP isoforms was examined in normal human pancreas, pancreatic adenocarcinoma, and pancreatic cell lines of ductal origin (Capan-1, Capan-2, and HPAF) using reverse transcriptase-polymerase chain reaction and immunohistochemistry. RESULTS: Messenger RNAs for AQP1, -3, -4, -5, and -8 were detected in normal pancreas and in pancreatic adenocarcinoma. The cell lines expressed AQP3, -4, and -5 but lacked AQP1 and AQP8. Immunohistochemistry of normal pancreas revealed that AQP1 is strongly expressed in centroacinar cells and in both the apical and basolateral domains of intercalated and intralobular duct epithelia. AQP1 expression declined with distance along the small interlobular ducts and was not detectable in larger interlobular ducts. AQP3 and AQP4 were not detectable by immunohistochemistry. AQP5 was observed at the apical membrane of intercalated duct cells and also in duct associated mucoid glands. AQP8 was confined to the apical pole of acinar cells. Both AQP1 and AQP5 were colocalised with cystic fibrosis transmembrane conductance regulator (CFTR) at the apical membrane of intercalated duct cells. CONCLUSIONS: AQP1 and AQP5 are strongly expressed in the intercalated ducts of the human pancreas. Their distribution correlates closely with that of CFTR, a marker of ductal electrolyte secretion. This suggests that fluid secretion is concentrated in the terminal branches of the ductal tree and that both AQP1 and AQP5 may play a significant role.
Adenocarcinoma↗
Cystic fibrosis: current concepts.
Cystic fibrosis (CF), a common fatal genetic disease, is a multisystem disorder whose pathogenesis has recently been linked to defects in CFTR, a newly discovered protein. CFTR is a molecular channel which controls chloride concentration in secretions of the sweat glands and the respiratory, GI, and reproductive tracts. Defective forms of CFTR, arising from various mutations in its gene, are responsible for the inadequate hydration of mucus, pancreatic juice, and other exocrine secretions. The result is dysfunction of the lungs, pancreas, and other involved organs. This article describes the diagnosis, clinical features, and approach to management of CF.
Cystic Fibrosis↗
Characterization of LPS-induced lung inflammation in cftr-/- mice and the effect of docosahexaenoic acid.
The mechanism by which Pseudomonas causes excessive inflammation in the cystic fibrosis lung is unclear. We have reported that arachidonic acid is increased and docosahexaenoic acid (DHA) decreased in lung, pancreas, and ileum from cftr-/- mice. Oral DHA corrected this defect and reversed the pathology. To determine which mediators regulate inflammation in lungs from cftr-/- mice and whether inhibition occurs with DHA, cftr-/- and wild-type (WT) mice were exposed to aerosolized Pseudomonas lipopolysaccharide (LPS). After 2 days of LPS, tumor necrosis factor-alpha (TNF-alpha), macrophage inflammatory protein-2, and KC levels in bronchoalveolar lavage fluid were increased in cftr-/- compared with WT mice and not suppressed by pretreatment with oral DHA. Neutrophil levels were not different between cftr-/- and WT mice. After 3 days of aerosolized LPS, neutrophil concentration, TNF-alpha, and the eicosanoids 6-keto-PGF1alpha, PGF2alpha, PGE2, and thromboxane B2 were all increased in bronchoalveolar lavage fluid from cftr-/- mice compared with WT controls. Oral DHA had no significant effect on TNF-alpha levels in cftr-/- mice. In contrast, neutrophils and eicosanoids were decreased in cftr-/- but not in WT mice treated with DHA, indicating that the effects of DHA on these inflammatory parameters may be related to correction of the membrane lipid defect.
Administration, Oral↗
[Anesthetic complications in sequential bipulmonary transplantation in patients with cystic fibrosis. Apropos of 6 cases].
Cystic fibrosis (CF) is a disease characterized mainly by altered exocrine gland function that eventually produces irreversible dysfunction of the pancreas and lungs. The respiratory insufficiency that develops in CF patients in the advanced stages of disease can only be corrected at this time by lung or heart-lung transplantation. We describe our experience with 6 terminal phase CF patients who underwent sequential double lung transplantation (SDLT). Anesthesia was intravenous, with exhaustive hemodynamic and respiratory monitoring. During surgery the most frequently encountered hemodynamic complications were low minute volume, arterial hypotension and irregular heart rate. The main respiratory complications were hypoxemia, hypercapnia and pulmonary edema of the implanted lung, which developed in all cases to varying degrees related to the organ's state of preservation and duration of ischemia. Other complications were the need for extracorporeal circulation in 1 case, oliguria and blood loss requiring multiple transfusions. The most critical moments were at the time of clamping the pulmonary artery, the period after revascularization of the donated lung, and at the start of patient ventilation through the first implanted lung so that the second could be implanted. Although our series is small, it is of interest given the limited Spanish experience with lung transplantation in CF patients, and the good early results obtained, which are similar to those reported for other diseases.
Adolescent↗
Benign extrinsic compression of the common bile duct: a case report.
Painless obstructive jaundice is usually the result of malignant compression of the distal common bile duct. Infrequently extrinsic benign lesions may also cause ductal obstruction and be mistaken for a malignant process. A case of compression of the distal common bile duct is described. Preoperative evaluation was most consistent with a cystadenocarcinoma of the head of the pancreas. At operation we found an enlarged and calcified periductal lymph node with associated fibrosis and compression and obstruction of the distal common duct. Massive dilatation of the distal common duct and the cystic duct remnant gave it the appearance of a cystic mass in the superior border of the head of the pancreas. Resection and choledochoduodenostomy were curative.
Aged↗
A Japanese adult case of cystic fibrosis causing bone demineralization.
Cystic fibrosis is an inherited, multisystem disorder characterized by an abnormality in exocrine gland function. It leads to chronic pulmonary disease in most cases and pancreatic insufficiency in 85 percent of patients. Although this disease is not uncommon in Caucasians, it has been considered very rare among Japanese. The majority of patients are diagnosed in infancy or childhood. The patient in this case report was a 45-year-old Japanese man who had not been diagnosed as having cystic fibrosis. This patient had recurrent episodes of pulmonary infection that started in childhood, and plain films of the chest showed increased interstitial markings, hyperaeration, and bronchiectasis. CT of the upper abdomen showed a generally enlarged pancreas with complete fatty replacement. Serum and urine pancreatic enzyme levels were low, suggesting pancreatic insufficiency. Repeated sweat tests were positive. A roentgenologic skeletal survey showed general demineralization, which may be multifactorial. In this case, it was concluded that vitamin D deficiency caused by vitamin D malabsorption and/or insufficient sunlight exposure was mainly responsible for the demineralization and that chronic respiratory acidosis might also be partially responsible.
Bone and Bones↗
Developmental expression of a mucinlike glycoprotein (MUCLIN) in pancreas and small intestine of CF mice.
The mucinlike glycoprotein MUCLIN, one of two protein products of the CRP-ductin gene, was used to study changes in the expression of sulfated glycoconjugates during the pathogenesis of cystic fibrosis, using the cystic fibrosis transmembrane conductance regulator (CFTR) knockout mouse (CF mouse). We assessed the appearance of dilated lumina containing protein or mucus plugs in pancreatic acini and crypts of the small intestine and quantified MUCLIN protein and CRP-ductin mRNA during postnatal development. In CF mice, the pancreatic acinar lumen was dilated by postnatal day 16 (P16), but MUCLIN protein was first significantly increased by P23 and remained elevated through adulthood compared with normal mice. Similarly, intestinal crypts had CF-like mucus plugs by P16, but MUCLIN protein was first elevated by P23 and remained elevated through adulthood compared with normal mice. In both organs, MUCLIN labeling of the luminal surface was increased concomitantly with dilation and protein or mucus plugging but before upregulation of expression. The morphological changes were then followed by upregulation of MUCLIN protein and CRP-ductin mRNA expression. This is the first direct study of CF pathogenesis and the resultant increase in glycoconjugate gene expression. The data are consistent with CF pathogenesis progressing from an initial alteration in protein secretory dynamics (increased luminal MUCLIN and protein/mucus plugs) to an upregulation of glycoprotein/mucin gene expression, which is expected to exacerbate obstruction of the luminal spaces.
Animals↗