1/8. Long-term survival after diagnosis of hepatic metastatic vipoma: report of two cases with disparate courses and review of therapeutic options.This report describes two patients with pancreatic cholera caused by vasoactive intestinal polypeptide (VIP)-producing tumors, which originated in the pancreas and showed metastases in both hepatic lobes at time of diagnosis. However, the two tumors displayed remarkably disparate clinical courses. Due to the protracted but progressive course over more than 10 years, a multifaceted therapeutic approach was performed to control symptoms and to improve quality of life. The long-acting somatostatin analog octreotide was the most effective treatment for relieving symptoms and correcting fluid and electrolytes disturbances. The effects of complementary treatments, including systemic chemotherapy and hyperselective chemoembolization, as well as concurrent application of octreotide and prednisolone or interferon with respect to clinical symptoms, VIP levels, and tumor growth are reviewed. Our experience, although small, emphasizes the need for an expert, well-planned, adaptive, and multidisciplinary approach in the care of these complex patients.- - - - - - - - - - ranking = 1keywords = cholera (Clic here for more details about this article) |
2/8. Pancreatic cholera syndrome due to a vasoactive intestinal polypeptide-producing tumor: further insights into the pathophysiology.This case report describes a patient with pancreatic cholera caused by a vasoactive intestinal polypeptide-producing pancreatic tumor. The case presents several unusual characteristics of this disease. The primary tumor was a mucinous adenocarcinoma of the pancreas. The serum vasoactive intestinal polypeptide level of 2400 pmol/L is the highest reported. At this vasoactive intestinal polypeptide level, the somatostatin analogue SMS 201-995 at doses up to 2 mg/24 h did not control the 21 L/24 h stool output. fecal incontinence due to a manometrically documented hypotonic internal anal sphincter occurred. Using surgically created stomas, the segmental gastrointestinal fluid and sodium losses were shown to be greatest from the jejunum, whereas potassium losses from the colon and small intestine were equal. The cellular mechanism for the small intestinal potassium secretion is not known.- - - - - - - - - - ranking = 5keywords = cholera (Clic here for more details about this article) |
3/8. Successful treatment of therapy-resistant pancreatic cholera with human leucocyte interferon.The pancreatic cholera syndrome is a serious and potentially fatal disease found in patients with endocrine pancreatic tumours and ganglioneuromas. Two patients with therapy-resistant pancreatic cholera syndrome were successfully treated with human leucocyte interferon given intramuscularly in a dose of 3 X 10(6)-6 X 10(6) IU per day. This produced a reduction in stool volume and plasma vasoactive intestinal polypeptide (VIP) within 3-5 days of the start of treatment. Tumour mass decreased in one of the patients after 3 months of treatment but some tumour tissue remained after 15 months' observation, although circulating concentrations of VIP are normal. The mechanisms of action of interferon are not known but a direct inhibition of tumour-cell hormone production and perhaps of tumour-cell proliferation might account for the rapid clinical response.- - - - - - - - - - ranking = 6keywords = cholera (Clic here for more details about this article) |
4/8. Pancreatic cholera syndrome: effect of a synthetic somatostatin analog on intestinal water and ion transport.The effect of a synthetic somatostatin analog was studied in a patient with severe secretory diarrhea due to pancreatic cholera syndrome. Basal intestinal perfusion studies indicated an absence of water and sodium absorption, and active chloride secretion in the small bowel. Intravenous administration of the somatostatin analog (1 microgram/kg.h) changed zero net water movement to absorption (122 mL/30 cm of the jejunum per hour). Chloride secretion changed to absorption (5.0 to 7.9 meq/30 cm.h), and plasma vasoactive intestinal polypeptide concentration was reduced from 330 to 45 pmol/L (normal, less than 51). When the analog was given subcutaneously, 100 micrograms twice daily, stool weight decreased, and plasma vasoactive intestinal polypeptide concentration fell toward the normal range (67 pmol/L). plasma concentration of pancreatic polypeptide was initially elevated and dropped during intravenous infusion of somatostatin analog but returned to baseline on maintenance therapy with the analog delivered subcutaneously. The patient has not had further diarrhea during 9 months of therapy.- - - - - - - - - - ranking = 5keywords = cholera (Clic here for more details about this article) |
5/8. multiple endocrine neoplasia, type 1, with pancreatic cholera.A 20-yr-old black woman presented in 1969 with headache, amenorrhea, hyperprolactinemia, hypogonadotropism, hypogonadism, and hypercalcemia due to a chromophobe adenoma. She received 5000 rads to the sella. One year later she was found to have hyperparathyroidism due to parathyroid adenoma and three and a half glands were removed. Thirteen years later she presented with 3 months of profuse watery diarrhea, hypokalemia, hypercalcemia, hyperchloremic metabolic acidosis, and a normal anion gap. A vasoactive intestinal polypeptide-producing tumor of the pancreas was found and successfully removed, after which hypercalcemia resolved. This is an unusual case of the multiple endocrine neoplasia syndrome, type 1, being associated with a vasoactive intestinal polypeptide-oma and pancreatic cholera.- - - - - - - - - - ranking = 5keywords = cholera (Clic here for more details about this article) |
6/8. Effect of somatostatin on diarrhea and on small intestinal water and electrolyte transport in a patient with pancreatic cholera.The effects of somatostatin on diarrhea and on small intestinal flow of water and electrolytes (slow-marker perfusion technique) in a patient with pancreatic cholera are reported. Continuous intravenous infusion of somatostatin (8 micrograms/kg/hr) suppressed the diarrhea, but a rebound was observed after somatostatin. Infusion of somatostatin at the same dosage decreased the ileal fluid flow rate to within control values. This effect was mainly due to a sharp reduction in the rate fluid entered the jejunum, but was also due to a suppression of the abnormal water and electrolyte secretion in the proximal jejunum. Secretion in the rest of the small bowel remained unchanged. somatostatin did not noticeably alter the high preinfusion plasma level of prostaglandin E1, but decreased the initially high plasma concentration of vasoactive intestinal peptide to normal values. These results suggest that long-acting somatostatin analogs could be of value in the symptomatic treatment of diarrhea in pancreatic cholera.- - - - - - - - - - ranking = 6keywords = cholera (Clic here for more details about this article) |
7/8. Streptozotocin treatment in pancreatic cholera (Verner-Morrison) syndrome.A case of pancreatic cholera (Verner-Morrison syndrome) associated with a pancreatic endocrine tumor and hepatic metastases is presented. VIP and HPP plasma levels, initially elevated, were accurately followed in various conditions: during corticosteroid therapy, after pancreatic tumor excision, during and after streptozotocin therapy (1.5 g/m2) by repeated intraarterial route). Only streptozotocin therapy resulted in a reduction of the stool volume with concomitant decrease in VIP plasma levels. However, the size of the hepatic metastases was unchanged and HPP plasma levels remained elevated. It is suggested that VIP represents the tumoral secretion and HPP a marker of the residual malignant tissue.- - - - - - - - - - ranking = 5keywords = cholera (Clic here for more details about this article) |
8/8. Surgical treatment of pancreatic cholera: a case report.After surgical resection for rectosigmoid carcinoma a 63-year-old man had secretory diarrhea causing severe metabolic acidosis, hypokalemia, hypercalcemia and dehydration. Subsequent investigations revealed a mass measuring 4 x 5 cm in the uncinate process of the pancreas and an elevated vasoactive intestinal polypeptide concentration. The diarrhea responded to treatment with the somatostatin analogue. Sandostatin, and remained under control during a prolonged preoperative period. The patient underwent a Whipple procedure with immediate lessening of his diarrhea. This report illustrates a classic case of vipoma and demonstrates the need to consider this condition in the differential diagnosis of secretory diarrhea, even in the presence of other gastrointestinal lesions. The effectiveness of somatostatin analogues in stabilizing the diarrhea preoperatively is also well illustrated.- - - - - - - - - - ranking = 4keywords = cholera (Clic here for more details about this article) |