Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UNIPROT:P01350 (gastrin)
 9,683 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Mean fasting levels of pancreatic polypeptide (PP) in 24 patients with Zollinger-Ellison syndrome (ZES) and in 12 patients with hyperparathyroidism originating from families with multiple endocrine adenomatosis type I (MEAI-HPT) were significantly higher than in 72 normal controls. The overlap between the 3 groups, however, was large. In patients with ZES, increased PP levels were not related to the presence of MEAI or metastases; nor was there a correlation between serum PP and gastrin concentrations. The post-prandial PP release in 10 ZES patients and in 10 patients with MEAI-HPT was lower than in 9 normal controls. The physiological significance of the present findings is unclear.
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PMID:Serum levels of pancreatic polypeptide in Zollinger-Ellison syndrome, and hyperparthyroidism from families with multiple endocrine adenomatosis type I. 3 34

We report the case of a 43 year old man with multiple endocrine neoplasia (Zollinger-Ellison syndrome-hyperparathyroidism) diagnosed during investigations for a severe ulcer with diarrhoea. The history suggested Zollinger-Ellison syndrome, especially as basal acid production and blood gastrin levels were high. Hypercalcaemia and low blood phosphate levels suggested hyperparathyroidism, which was confirmed by ultra-sound of the neck, showing two parathyroid adenomas. The aim of treatment was two-fold: suppress the hyperparathyroidism and treat the Zollinger-Ellison syndrome. The approach is surgical in most cases, but the tumour is often impossible to remove and total gastrectomy has become rare with the progress in anti-secretory therapy. When the tumour behind a case of Zollinger-Ellison syndrome cannot be found, complementary chemotherapy is indicated. Other endocrine lesions must always be sought and the family investigated to exclude Wermer syndrome.
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PMID:[Multiple endocrine neoplasms associated with Zollinger-Ellison syndrome and hyperparathyroidism]. 615 41

When the Zollinger-Ellison syndrome was first identified, total gastrectomy was proposed as the most effective treatment for the secretory manifestations of the syndrome. Recently, however, great enthusiasm has developed for medical treatment by means of H2-receptor antagonists. The authors have cared for 27 patients with the Zollinger-Ellison syndrome at The University of Texas Medical Branch in the past 12 years and have been pleased with the results of total gastrectomy, which was performed in 23 of the 27 patients (one patient refused operation and three patients had lesser gastric operations). Twenty-three patients underwent total gastrectomy with Roux-en-Y esophagojejunostomy. There were no operative deaths. Primary tumors were found in 17 patients, seven of whom also had metastatic tumors. No tumors were found in nine patients. Nine patients are dead; the actuarial survival rate for all patients was 75% at 5 years and 52% at 10 years. Eleven of the 27 patients had the multiple endocrine neoplasia I syndrome. Of the 18 survivors, only three have normal serum gastrin levels, and all three had extrapancreatic gastrinomas, one in peripancreatic lymph nodes, one in the liver, and one in a cystic tumor attached to the stomach. Nutritional results were good to excellent, with a mean postoperative weight loss of 14.7% (mean follow-up period was 45 months). The authors conclude that treatment of the hypersecretory problems of the Zollinger-Ellison syndrome by total gastrectomy is safe and dependable. Results compare well with those of long-term medical management, whose success is dependent upon serial favorable responses to a lifetime of repeated challenges.
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PMID:The role of surgery in the Zollinger-Ellison syndrome. 684 79

Mutations in the MEN1 gene correlate with multiple endocrine neoplasia I (MEN1). Gastrinomas are the most malignant of the neuroendocrine tumors associated with MEN1. Because menin and JunD proteins interact, we examined whether JunD binds to and regulates the gastrin gene promoter. Both menin and JunD are ubiquitous nuclear proteins that we showed colocalize in the gastrin-expressing G cells of the mouse antrum. Transfection with a JunD expression vector alone induced endogenous gastrin mRNA in AGS human gastric cells, and the induction was blocked by menin overexpression. We mapped repression by menin to both a nonconsensus AP-1 site and proximal GC-rich elements within the human gastrin promoter. Chromatin immunoprecipitation assays, EMSAs, and DNA affinity precipitation assays documented that JunD and Sp1 proteins bind these two elements and are both targets for menin regulation. Consistent with menin forming a complex with histone deacetylases, we found that repression of gastrin gene expression by menin was reversed by trichostatin A. In conclusion, proximal DNA elements within the human gastrin gene promoter mediate interactions between JunD, which induces gastrin gene expression and menin, which suppresses JunD-mediated activation.
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PMID:Menin and JunD regulate gastrin gene expression through proximal DNA elements. 2185 62

Pancreatic neuroendocrine tumors (PNETs) are rare and characterized by widely variable clinical presentation and often challenging surgical management. Methods: Retrospective study conducted during the last 15 years at the First Surgical Clinic of the Iasi St Spiridon University Hospital, and which included all the patients diagnosed with pancreatic endocrine tumors by immunohistochemistry. Results: There were 26 cases diagnosed with PNET. The male/female ratios was 7/19 and mean age 41.93 +- 2.48 years (range 20-79 years). Of the PNET cases 13 were insulinomas, 5 gastrinomas, 2 gastrinomas associated with other endocrine neoplasms (Wermer syndrome), 5 non-functional endocrine pancreatic tumors and 1 ACTHoma. Clinical manifestations depended on tumor type: hypoglycemia and Whipple triad for insulinoma, Zollinger Ellison syndrome and complicated peptic ulcer (hemorrhage, perforation) for gastrinoma, Cushing syndrome for ACTHoma. Biological diagnosis included biological markers (e.g. insulin, gastrin and cortisol). Tumor site and size at diagnosis were determined by ultrasound, CT-scan, angiography, PETscan, octreoscan and intraoperative ultrasound. Surgical procedures for PNET insulinomas were: tumor resection - 6 cases; left splenopancreatectomy - 3 cases; left spleen-preserving pancreatectomy - 2 cases; pancreaticoduodenectomy - 2 cases. We also present 4 cases of gastrinoma with multiple ulcers and multiple surgical interventions for hemorrhage and perforation with peritonitis. The two patients with Wermer syndrome also had ulcers complicated with hemorrhage and peritonitis and parathyroid adenoma. Nonfunctional pancreatic endocrine tumors were diagnosed in 5 women of which in 3 the tumors were located in the pancreatic tail (in which splenopancreatectomy and left pancreatectomy with spleen preservation were performed) and in 2 in the pancreatic head (in which pancreaticoduodenectomy and Beger type operation were performed). Conclusions: Knowledge of clinical signs of secreting tumors and exploring the patients are of crucial importance for management of PNETs. Immunohistochemistry is mandatory for confirming the diagnosis and assessing the proliferation and biological behavior of the tumor, thus facilitating the administration of specific therapy. Aggressive surgical treatment is indicated, even in advanced stages.
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PMID:The Pancreatic Endocrine Tumors - Experience of First Surgical Clinic Iasi. 3167 Jun 40