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)

In 4 patients with familial adenomatous polyposis (FAP) and multiple severe dysplastic adenomas in the duodenum (a 42-year-old woman and 3 men aged 44, 53 and 33 years, respectively), pancreas-preserving total duodenectomy (PPTD) was carried out. In 2 of the patients, serious early post-operative complications arose (leakage and haemorrhage of the gastrojejunostomy, respectively), and 1 patient developed a late complication (attacks of pancreatitis). During the 1-5-year follow-up period, small villous adenomas were seen in the jejunum (neoduodenum) of 1 of the patients. PPTD is a technically possible procedure which allows targeted treatment to be carried out if duodenum polyps are found upon endoscopic examination.
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PMID:[Pancreas-preserving total duodenectomy due to familial adenomatous polyposis in 4 patients]. 1195 83

Tumors of the papillary region are an unusual and heterogeneous group of neoplasms that arise from the major papilla, the ampulla of Vater, and the peripapillary duodenum. Benign adenomas of the papilla of Vater are an increasingly recognized condition in those with familial adenomatous polyposis syndromes as well as sporadic cases. Papillary adenoma is a recognized but rare cause of acute pancreatitis. We describe a patient who presented with acute recurrent pancreatitis that was attributed to an intrapapillary pedunculated villous adenoma. Following diagnosis by endoscopic needle knife sphincterotomy and endoscopic retrograde cholangiopancreatography, endoscopic snare resection of the adenoma resulted in symptomatic improvement.
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PMID:Endoscopic snare resection of an intrapapillary pedunculated villous adenoma presenting as acute recurrent pancreatitis. 1510 28

Pancreatic cancer (PC) is the most fatal of all gastrointestinal cancers, wherein its mortality compares strikingly with its incidence. Unfortunately, 80-90% of PCs are diagnosed in the nonresectable stage. While the lifetime risk of PC in developed countries is approximately 1-3%, it is the fifth most common cause of cancer deaths among both males and females in Western countries. It occurs in excess in Jews. Approximately 5-10% of PC shows familial clustering. Examination of such familial clusters must take into consideration cancers of diverse anatomic sites, such as malignant melanoma in the familial atypical multiple melanoma (FAMMM) syndrome due to the CDKN2A (p16) germline mutation, and combinations of colorectal and endometrial carcinoma, ovarian carcinoma, and several other cancers in hereditary nonpolyposis colorectal cancer (HNPCC), which are due to mismatch repair germline mutations, the most common of which are MSH2 and MLH1 . Other hereditary disorders predisposing to PC include Peutz-Jeghers syndrome, due to the STK11 mutation, familial pancreatitis due to the cationic trypsinogen gene, site-specific familial pancreatic cancer which may be due to the 4q32-34 mutation, hereditary breast-ovarian cancer (HBOC) syndrome that is due to BRCA2 and possibly some families with HBOC that is due to BRCA1 , familial adenomatous polyposis due to the ATP gene, and ataxia telangiectasia due to the ATM germline mutation. This extant heterogeneity mandates that the physician be knowledgeable about these PC-prone syndromes which play such an important role when considering the differential diagnosis of hereditary PC. Unfortunately, there are no PC screening programs with acceptable sensitivity and specificity. However, the gold standard for screening at this time is endoscopic ultrasound. Clearly, there is a great need for the development of novel screening approaches with acceptable sensitivity and specificity. Further research is needed to elucidate those etiologic factors that contribute to the apparent excess of PC in Ashkenazi Jews. Attention should also be given to the search for mutations predisposing to PC in Jews so that opportunities to learn more about the disease's pathogenesis, as well as screening and control, may take place.
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PMID:Familial pancreatic carcinoma in Jews. 1551 47

Most ampullary adenomas (80%) are common benign ampullary tumors; however, they can range from mild dysplasia to high-grade dysplasia to invasive carcinoma. They are considered premalignant lesions found in the setting of familial polyposis syndromes or found sporadically, usually manifested by vague abdominal pain, liver enzyme elevation, jaundice, recurrent pancreatitis, or with uncommon symptoms such as gastrointestinal bleeding or duodenal obstruction. Endoscopic retrograde cholangiopancreatography with biopsy is a minimally invasive technique used to visualize these tumors directly and to evaluate their histologic characteristics. Definitive treatment primarily depends on these histologic results. Local resection has a high rate of recurrence (5% to 30%) and requires postoperative endoscopic surveillance, which is the reason it is not considered as a first choice in the management of ampullary tumors. The operative mortality is 10% or less for pancreaticoduodenectomy, a procedure of choice at most experienced centers for frank carcinoma, foci papillary adenocarcinoma in pre-excisional biopsies, or high-grade dysplasia ampullary adenomas. Endoscopic interventions for presumed benign ampullary adenomas have resolved symptoms of obstruction, but long-term follow up is necessary to detect early malignant transformation. In summary, the choice of treatment depends on level of surgical skill available, patient tolerance of long-term endoscopic surveillance versus radical surgery, and the presence or absence of coexisting familial adenomatous polyposis.
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PMID:Ampullary tumors: endoscopic versus operative management. 1575 95

Duodenal adenocarcinoma remains the leading cause of cancer death in familial adenomatous polyposis patients following colectomy. Stratification based on Spigelman's criteria provides a means for determining therapy. Spigelman stage IV patients have been selected for pancreas-sparing duodenectomy. Twenty-one patients underwent resection between 1992 and 2004, with a mean age of 58 +/- 11 years. The mean time from colectomy to duodenectomy was 27 +/- 13 years. Invasive cancer was found in the distal duodenum in one patient. Operative time averaged 327 +/- 61 minutes with a mean blood loss of 503 +/- 266 ml. There was no mortality, and eight patients (38%) had 14 complications: six (29%) with delayed gastric emptying, four (19%) with biliary/pancreatic anastomotic leak, one with pancreatitis, and one with wound infection. There were two reoperations: one for delayed gastric emptying and one for an early biliary leak. Mean length of stay was 15 +/- 10 days. Two late complications occurred: a stomal ulcer and an intestinal obstruction at 48 and 24 months, respectively. Mean follow-up was 79 months (range, 3-152 months). Two patients developed polyps in the advanced jejunal limb and were endoscopically treated. Pancreas-sparing duodenectomy represents a definitive treatment for advanced duodenal polyposis and can obviate the need for pancreaticoduodenectomy.
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PMID:Pancreas-sparing duodenectomy is effective management for familial adenomatous polyposis. 1626 79

We present an unusual case of pancreatitis secondary to a polyp obstructing the papilla, treated endoscopically. A 45-year-old woman with familial adenomatous polyposis syndrome and prior total colectomy presented with acute pancreatitis. Upper endoscopy and endoscopic retrograde cholangiopancreaticogram revealed significant periampullary tissue. Sphincterotomy and endoscopic snare resection of the polyp were performed without complications. Local, noninvasive procedures are a promising diagnostic and therapeutic modality which has significantly less morbidity and mortality than conventional surgical techniques, and may be a reasonable alternative in the management of such patients.
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PMID:Acute pancreatitis secondary to adenomatous transformation of the ampulla of Vater in a patient with familial adenomatous polyposis. 1731 55

Familial adenomatous polyposis (FAP) is characterized by the development of multiple adenomatous polyps predominantly in the colon but also in the duodenum. Scattered case reports indicate that there is a risk for pancreatitis in FAP. The most likely cause of pancreatitis in FAP is obstructing ampullary adenomas. We describe 7 FAP patients who experienced one or more episodes of pancreatitis. Two patients experienced pancreatitis after endoscopic treatment of ampullary adenoma. The cause of the pancreatitis in 5 of 7 patients could not be determined, as none of the patients had obstruction of the ampulla. Furthermore, other risk factors for pancreatitis such as pancreatic serine protease inhibitor Kazal type I (SPINK1) gene mutations were ruled out. A review of literature identified 20 FAP patients who developed the first episode of pancreatitis at a mean age of 45 years (range 23-72 years). Some 55% had recurrent episodes of pancreatitis. Eight patients had (peri) ampullary adenomas or carcinomas. In most cases, the course of pancreatitis was mild with an uneventful outcome, but one patient died after an episode of acute pancreatitis.
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PMID:Recurrent idiopathic pancreatitis in familial adenomatous polyposis: report of a case-series and review of the literature. 1731 39

Although turmeric (Curcuma longa; an Indian spice) has been described in Ayurveda, as a treatment for inflammatory diseases and is referred by different names in different cultures, the active principle called curcumin or diferuloylmethane, a yellow pigment present in turmeric (curry powder) has been shown to exhibit numerous activities. Extensive research over the last half century has revealed several important functions of curcumin. It binds to a variety of proteins and inhibits the activity of various kinases. By modulating the activation of various transcription factors, curcumin regulates the expression of inflammatory enzymes, cytokines, adhesion molecules, and cell survival proteins. Curcumin also downregulates cyclin D1, cyclin E and MDM2; and upregulates p21, p27, and p53. Various preclinical cell culture and animal studies suggest that curcumin has potential as an antiproliferative, anti-invasive, and antiangiogenic agent; as a mediator of chemoresistance and radioresistance; as a chemopreventive agent; and as a therapeutic agent in wound healing, diabetes, Alzheimer disease, Parkinson disease, cardiovascular disease, pulmonary disease, and arthritis. Pilot phase I clinical trials have shown curcumin to be safe even when consumed at a daily dose of 12g for 3 months. Other clinical trials suggest a potential therapeutic role for curcumin in diseases such as familial adenomatous polyposis, inflammatory bowel disease, ulcerative colitis, colon cancer, pancreatic cancer, hypercholesteremia, atherosclerosis, pancreatitis, psoriasis, chronic anterior uveitis and arthritis. Thus, curcumin, a spice once relegated to the kitchen shelf, has moved into the clinic and may prove to be "Curecumin".
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PMID:Curcumin as "Curecumin": from kitchen to clinic. 1790 May 36

Familial adenomatous polyposis is an archetypal disease illustrating the genetic basis of human cancer. The adenomatous polyposis coli gene functions as a tumor suppressor with hundreds of known mutations that result in a defective adenomatous polyposis coli protein. In addition to the certain fate of colon cancer without colectomy, patients with familial adenomatous polyposis are also at increased risk for other types of neoplasms, including those which affect the pancreas. This review focuses on periampullary and ampullary tumors, benign and malignant pancreatic neoplasms that are associated with familial adenomatous polyposis and Gardner syndrome and pancreatitis in these patients. An individualized surveillance regimen is suggested which for certain patients could include endoscopic ultrasound.
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PMID:The pancreas in familial adenomatous polyposis. 1818 37

Ampullary adenoma is a premalignant lesion involving the major papilla and surrounding mucosa of the second duodenum. These lesions may arise sporadically or in the setting of familial adenomatous polyposis syndrome. Ampullary adenoma may be found because of symptoms, at the time of adenomatous polyposis syndrome surveillance, or incidentally when upper endoscopy is performed for other reasons. Management options include observation, local or extended surgical resection (including pancreaticoduodenectomy), and endoscopic resection. Endoscopic resection is being performed more often and involves techniques similar to those of colonoscopic polypectomy. Preventing post-endoscopic resection pancreatitis is important and can be achieved with placement of temporary pancreatic duct stents.
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PMID:Ampullary adenoma. 1832 36


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