Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0030305 (pancreatitis)
 16,014 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Intraductal papillary mucinous tumor is a rare pancreatic tumor originating from the epithelium of the pancreatic duct and exhibiting papillary proliferation of tall columnar epithelial cells. The usual clinical presentation is recurrent episodes of pancreatitis due to hypersecretion of mucin and obstruction of a markedly dilated pancreatic duct. We describe a 74-year-old man who presented recurrent attacks of cholangitis, due to a common bile duct obstruction from thick pancreatic mucus reflux, as the first manifestation of intraductal papillary mucinous tumor.
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PMID:Recurrent cholangitis as the first manifestation of an intraductal papillary mucinous tumor of the pancreas. 1599 Sep 38

Acute biliary pancreatitis, caused by macroscopic cholesterol gallstones or microlithiasis, is often a severe disease with considerable morbidity and mortality. Formation of cholesterol gallstones and microlithiasis is caused by cholesterol crystallization from cholesterol supersaturated gallbladder bile. Particularly patients with fast and extensive crystallization, due to highly concentrated bile, low biliary phospholipid contents and gallbladder mucin hypersecretion seem at risk for pancreatitis. Patients who suffered from acute biliary pancreatitis should undergo cholecystectomy as secondary prevention strategy. For patients at high surgical risk, endoscopic sphincterotomy may be an appropriate alternative. Pharmacological manipulation of biliary lipids by the hydrophilic bile salt ursodeoxycholic acid is reserved for patients with recurrent pancreatitis despite previous cholecystectomy or sphincterotomy, or with contraindications to surgical and endoscopic treatment. Maintenance therapy with ursodeoxycholic acid is however a very effective secondary prevention strategy. Potentially, secondary prevention of acute biliary pancreatitis could also be achieved through decreasing biliary mucin contents by UDCA, NSAIDs or N-acetylcystein, or through achieving bile dilution (currently not feasible).
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PMID:Pharmacological manipulation of biliary water and lipids: potential consequences for prevention of acute biliary pancreatitis. 1608 52

Intraductal papillary mucinous neoplasms include a large spectrum of lesions communicating with the Wirsung duct, having a variable invasiveness from benign or borderline, to malignant (carcinoma in situ and invasive cancer). Final diagnosis is based on endoscopic ultrasound (EUS)-guided fine needle aspiration and histopathologic exam of surgical specimens. We present the case of a 28-year-old woman, with several episodes of acute recurrent pancreatitis in the past 6 months, admitted for dyspepsia, nausea and loss of appetite. Imaging studies (transabdominal ultrasonography, CT scanning, MR cholangiopancreatography) showed a macrocystic, multilocular, corporeal tumor, communicating with the retrograde dilated Wirsung duct. EUS revealed hypoechoic material inside the cysts, raising the suspicion of an intraductal papillary mucinous neoplasm. Diagnosis was confirmed by EUS-guided fine needle aspiration, which found columnar mucinous cells within a mucin-rich fluid. The imaging evaluation was repeated after two years, showing a rapid evolution of the tumor. The patient refused surgical exploration and caudal pancreatectomy. In the context of the absence of clinical symptoms, the indolent evolution of these tumors and the excellent prognosis after resection, we consider that early identification and regular follow-up by EUS with fine needle aspiration is imperative, especially because of the limited success of other imaging methods.
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PMID:Natural evolution of an intraductal papillary mucinous neoplasm of the pancreas. A case report. 1741 Feb 97

Pancreatic cystic lesions are being increasingly identified. Clinical decision making is driven by the differential diagnosis of the cyst and, for the asymptomatic patient, its likelihood of causing harm. The fundamental issue is whether the cyst is neoplastic, and, if so, what is its risk for malignant degeneration. High-resolution computed tomography provides detailed information about cyst structure and may facilitate differentiation from mucin-secreting tumors of the pancreas. Magnetic resonance imaging has the potential added advantage of determining communication between the cyst and pancreatic duct. Endoscopic ultrasound (EUS) imaging provides additional characterization of the lesion. While EUS morphology alone has limitations regarding definitive diagnosis, aspiration, and characterization of cyst, fluid contents may provide incremental information. Aspiration is well tolerated and safe, with a complication rate of less than 1%. In the absence of a history of pancreatitis, pseudocyst is quite unlikely, and the concern of a cystic neoplasm is paramount. In general, all symptomatic lesions should proceed to appropriate surgical resection. If preoperative characterization of the lesion will change management, EUS+FNA for cytology and fluid analysis (CEA) may characterize the lesion as mucinous, although cytology alone is rarely definitive. For those patients with benign-appearing lesions, such as classic appearance of a serous cystadenoma, observation alone seems appropriate. In some circumstances, EUS+FNA confirmation of a negative cytology and low fluid CEA can further provide evidence to support a monitoring approach and deferral of surgical intervention.
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PMID:Management of cystic lesions of the pancreas. 1795 37

Intraductal papillary mucinous tumor (IPMN) of the pancreas is a rare pancreatic tumor characterized by intraductal proliferation of mucin producing cells with hypersecretion of mucin that leads to cystic dilatation of the involved ducts. The usual clinical presentation is recurrent episodes of pancreatitis due to hypersecretion of mucin and temporary obstruction of the main pancreatic duct.
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PMID:Acute recurrent pancreatitis curtaining an intraductal papillary mucinous tumor of the pancreas. 1819 31

Cystic lesions of the pancreas, with an estimated prevalence of 20%, frequently are incidental findings at imaging on asymptomatic patients. Pseudocysts, typically in a setting of pancreatitis, should first be excluded. Characterization of cystic tumors is more complicated. Still, it is important to differentiate between benign and malignant lesions. Multi-detector row CT and MRI allow characterization of such lesions in over 75% of cases. Indeterminate lesions should undergo endoscopic US with biopsy/aspiration and fluid analysis, especially for mucin producing tumors (rounded with thick enhancing wall). When imaging fails to fully characterize a lesion, follow-up may be proposed for lesions less than 3 cm in size, that are either unilocular with thin nonenhancing wall (simple cyst) or lobulated multilocular with thin nonenhancing wall (serous cystadenoma, isolated side branch IPMTP). Follow-up imaging shows that these tumors usually show very little change over time. Management is based on comparing estimated patient survival without treatment to surgical risks (morbidity, mortality, functional sequelae from the procedure).
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PMID:[Imaging of incidental cystic lesions of the pancreas]. 1835 50

Protease-activated receptor-2 (PAR-2) is activated when trypsin cleaves its NH(2) terminus to expose a tethered ligand. We previously demonstrated that PAR-2 activates ion channels in pancreatic duct epithelial cells (PDEC). Using real-time optical fluorescent probes, cyan fluorescence protein-Epac1-yellow fluorescence protein for cAMP, PH(PLC-delta1)-enhanced green fluorescent protein for phosphatidylinositol 4,5-bisphosphate, and protein kinase Cgamma (PKCgamma)-C1-yellow fluorescence protein for diacylglycerol, we now define the signaling pathways mediating PAR-2 effect in dog PDEC. Although PAR-2 activation does not stimulate a cAMP increase, it induces phospholipase C to hydrolyze phosphatidylinositol 4,5-bisphosphate into inositol 1,4,5-trisphosphate and diacylglycerol. Intracellular Ca(2+) mobilization from inositol 1,4,5-trisphosphate-sensitive Ca(2+) stores and a subsequent Ca(2+) influx through store-operated Ca(2+) channels cause a biphasic increase in intracellular Ca(2+) concentration ([Ca(2+)](i)), measured with Indo-1 dye. Single-cell amperometry demonstrated that this increase in [Ca(2+)](i) in turn causes a biphasic increase in exocytosis. A protein kinase assay revealed that trypsin also activates PKC isozymes to stimulate additional exocytosis. Paralleling the increased exocytosis, mucin secretion from PDEC was also induced by trypsin or the PAR-2 activating peptide. Consistent with the serosal localization of PAR-2, 1 microm luminal trypsin did not induce exocytosis in polarized PDEC monolayers; on the other hand, 10 microm trypsin at 37 degrees C damaged the epithelial barrier sufficiently so that it could reach and activate the serosal PAR-2 to stimulate exocytosis. Thus, in PDEC, PAR-2 activation increases [Ca(2+)](i) and activates PKC to stimulate exocytosis and mucin secretion. These functions may mediate the reported protective role of PAR-2 in different models of pancreatitis.
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PMID:Protease-activated receptor-2 increases exocytosis via multiple signal transduction pathways in pancreatic duct epithelial cells. 1844 25

Herein is reported a case of mucin-producing carcinoma of the gallbladder in a 55-year-old Japanese woman. Although the patient's status and laboratory data initially suggested biliary pancreatitis due to gallstone, radiography and endoscopy confirmed the presence of pancreaticobiliary maljunction and a gallbladder tumor with excessive mucin, in which the duodenal papilla and the common bile duct were impacted. Following surgery, the gallbladder tumor was histopathologically diagnosed as a mixed endocrine-exocrine carcinoma. The carcinoma predominantly consisted of papillary, but also contained some tubular adenocarcinomatous components. Additionally, small foci of small cell and large cell neuroendocrine carcinomatous components were observed. There was no evidence of lymph node metastasis, distant metastasis, or direct invasion outside the gallbladder. Thus, the final classification of pT2N0M0 stage II was given to this lesion, according to the Union Internationale Contre le Cancer guidelines. The postoperative course was uneventful, and the carcinoma had not recurred in the absence of chemoradiotherapy for a period of 20 months. Mucin-producing gallbladder carcinoma is a rare clinical condition that can occur in patients with pancreaticobiliary maljunction. Detailed investigation of this condition is important to develop and refine effective therapeutic strategies.
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PMID:Mucin-producing gallbladder adenocarcinoma with focal small cell and large cell neuroendocrine differentiation associated with pancreaticobiliary maljunction. 1906 53

Preoperative diagnosis of pancreatic cysts benefits from integrating the clinical, radiological, and cytological features. As patient management algorithms evolve to increasingly nonsurgical options, accuracy in distinguishing mucinous from nonmucinous and benign from malignant mucinous cysts is important. This review focuses on pseudocysts, serous cystadenomas, intraductal papillary mucinous neoplasms (IPMNs), and mucinous cystic neoplasms. Patients with pseudocysts almost always present with pancreatitis and are usually medically managed. Radiological studies reveal a unilocular cyst mostly in the pancreatic tail. Cyst fluid is thin, with high amylase but low carcinoembryonic antigen (CEA) levels. DNA mutations are absent. Serous cystadenomas are benign and do not require resection. Patients are usually asymptomatic and have microcystic or macrocystic masses anywhere in the pancreas. Cytology is frequently nondiagnostic. CEA and amylase levels are low. DNA analysis may reveal loss of heterozygosity (LOH) at 3p if associated with Von Hippel-Lindau disease. Neoplastic mucinous cysts are highly variable in their presentation. Most are resected. Mucinous cystic neoplasms typically arise in the body or tail of the pancreas of middle-aged women and demonstrate a septated cyst without dilatation of the main pancreatic duct. Branch duct IPMNs are more common in the pancreatic head of elderly men. Main duct dilatation correlates with main duct or combined type IPMN. Both types of mucinous cysts produce variable amounts of mucin. Cytologically nonmalignant but atypical epithelial cells, even when scant, are an indication of a high risk for malignancy. High CEA level supports a mucinous cyst, as do KRAS mutation and good quality DNA levels. KRAS mutation and multiple LOH support malignancy.
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PMID:Pancreatic cysts: preoperative diagnosis and clinical management. 2004 27

The paper presents the data available in the literature on mutations in known genes in pancreatitis, such as cationic trypsinogen (PRSS1), pancreatic secretory trypsin inhibitor (PSTI/SPINK1), cystic fibrosis (CFTR), and apolipoprotein E (APOE) genes, as well as the new candidate gene--chymotrypsinogen (CTRC). It also gives the results of the authors studies estimating the spread of the mutations in the PRSS1 (2.5%), PSTI/SPINK1 (3.3%), and CFTR (0.8%) genes, as well as APOE polymorphism in patients with pancreatitis. It is shown that the E4 allele of the APOE gene was more frequently identified in patients with acute pancreatitis than in those with chronic pancreatitis (0.143 +/- 0.05 and 0.026 +/- 0.02, respectively; p < 0.05). An overview is given of 7 major classes of candidate genes implicated in the pathogenesis of cholesterol cholelithiasis (CL): hepatic enzymes regulating blood lipid composition; receptors of lipoproteins, hepatic and intestinal membrane and intracellular transport proteins; factors regulating the transcription of lipids and bile salts, cholecystokinin and its receptors, and mucin. In the authors' epidemiological study, the spread of APOE alleles and genotypes did not differ in women with and without CL; low molecular-weight apolipoprotein(a) isoforms (B, S2) were significantly found in patients with CL than in those without CL; the spread of the CG genotype in the TRPM8 gene was significantly lower in women with cholesterol CL than that in the Novosibirsk population. These polymorphisms have been proved to be associated with bile cholesterol concentrations in women with cholesterol CL. The opposite effect of the APOE4 allele on gallbladder stone formation processes is demonstrated, by using the APOE polymorphism as an example, which shows it necessary to examine each specific population to elicit a possible association between the polymorphism of different genes and gastrointestinal tract diseases.
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PMID:[Genetic aspects of digestive diseases. Part 1]. 2038 81


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