Serum pepsinogen I in familial multiple endocrine neoplasia type I

An increased serum pepsinogen I (PG I) concentration has been reported to be a marker of inherited peptic ulcer disease. Since both the Zollinger-Ellison syndrome and hyper-parathyroidism, components of the familial multiple endocrine neoplasia type I syndrome (MEN I), are often associated with peptic ulcer, we have studied serum PG I concentrations in members of six well-defined families with MEN I. Serum PG I concentrations in 20 family members with hyperparathyroidism ranged from 35 to 864 ng/ml compared to 21–92 ng/ml in 16 nonaffected MEN I members. However, serum PG I levels were significantly higher (P<0.01) in the hyperparathyroid patients with hypergastrinemia (PGI median 192, range 75– 864 ng/ml) than in those with normogastrinemia (PGI median 75, range 35–139 ng/ml). In fact, five of seven patients with hyperparathyroidism and hypergastrinemia compared to only one of 13 hyperparathyroid patients without hypergastrinemia had increased serum PG I levels above 130 ng/ml. We conclude that in the MEN I syndrome, increased serum PG I levels are found in patients with Zollinger-Ellison syndrome but not in hyperparathyroid patients with normogastrinemia and not in nonaffected MEN I members. The results indicate that in familial MEN I, hyperpepsinogenemia I is not inherited as a genetic trait but suggest that the elevated serum PG I levels are secondary to chronic hypergastrinemia.Supported in part by Research Grant DK 32015 from the National Institutes of Health, Bethesda, Maryland.     

Prospective study of efficacy and safety of lansoprazole in Zollinger-Ellison syndrome

Lansoprazole, a new substituted benzimidazole H⁺, K⁺-ATPase inhibitor, profoundly inhibits gastric acid secretion and has potential use in the management of diseases such as Zollinger-Ellison syndrome (ZES). In the present study we evaluated the efficacy and safety of lansoprazole in controlling acid hypersecretion in 20 patients with ZES. The starting dose was 60 mg once daily. Control of acid hypersecretion was defined as the dose required to reduce acid secretion to <10 meq/hr in the last hour before the next dose. Doses were adjusted upwards until effective control was achieved. Patients not controlled with 120 mg once daily were placed on twice daily lansoprazole. Most patients (90%) required lansoprazole once daily. During long-term follow-up (mean 18.5 months), 25% of patients required upward dose adjustments and 25% of patients required twice daily lansoprazole. Following cessation of therapy, the mean time for gastric acid output to reach half basal acid output was 39.1 hr. Lansoprazole was well-tolerated without side effects. Clinical chemistry and hematological studies were unchanged, and no gastric carcinoids developed. These results demonstrate that lansoprazole is a safe and effective inhibitor of gastric acid hypersecretion in patients with Zollinger-Ellison syndrome. Because it has a long duration of action, lansoprazole can be used to control gastric acid hypersecretion in most patients with Zollinger-Ellison syndrome using a once daily dosing schedule.     

A meal stimulation test in the diagnosis of pancreatic endocrine tumors in multiple endocrine neoplasia type 1

Background: The diagnostic value of the determination of the serum pancreatic polypeptide (PP) and gastrin concentrations after a standard meal for early diagnosis of patients with multiple endocrine neoplasia type 1 (MEN 1) is controversial. The aim of this study was to clarify this issue. Thirteen patients with MEN 1, seven healthy family members, and eight healthy controls were studied. Plasma PP and serum gastrin were measured before and after the ingestion of a standardized meal. The meal caused a statistically significant (p<0.05) increase of both PP and gastrin in all three groups studied. Concerning PP, no statistically significant difference was observed between patients and controls. In family members, the values were significantly (p<0.05) lower than in the other two groups. On the whole, no significant differences in gastrin levels were noted between patients and controls; in family members, the values were significantly (p<0.05) lower than in patients. All patients who had abnormally high postprandial values of PP and gastrin also had abnormally high basal values of these two peptides. The determination of serum PP and gastrin levels after a meal stimulation test in patients with MEN 1 adds no information about the presence of pancreatic endocrine tumors over that provided by basal values of the two peptides.     

A 7-year-old child with primary tumour localisation in the distal duodenum – new imaging procedures for an improved diagnosis

Duodenal gastrinomas in childhood are extremely rare and often missed at first medical consultation. We report on a 7-year-old child with sporadic gastrinoma of primary localisation in the distal duodenum. Small metastases in the liver and regional nodes were detected pre-operatively by somatostatin receptor scintigraphy (SRS) but not by other conventional imaging procedures. Diagnostic procedures include pre-operative SRS, endoscopic ultrasound and intra-operative endoscopic transillumination. Conclusion Gastrinomas are rare abdominal tumours in childhood. Pre-operative tumour-specific diagnosis is possible by ¹¹¹indium pentreotide SRS. Received: 9 May 1996 / Accepted: 21 October 1996     

Concurrent gastrinoma and somatostatinoma in a 10-year-old Portuguese water dog

Severe hypergastrinaemia in the dog is considered a strong indicator for a gastrin-producing pancreatic islet cell tumour. In human medicine, gastrinomas are described as enteropancreatic neuroendocrine tumours that cause the Zollinger-Ellison syndrome. However, in contrast to the supposed origin of canine gastrinomas in pancreatic islets, gastrinomas in human beings arise predominantly in the duodenum and gastric antrum, and are often a component of the syndrome of multiple endocrine neoplasia type I. This report is of a canine case of multiple endocrine neoplasms, consisting of a pancreatic islet cell somatostatinoma, and a gastrinoma in the mesenteric lymph nodes and liver. From the literature and the authors' findings, it is concluded that the clinical diagnosis of hypergastrinaemia is not pathognomonic for a gastrin-producing islet cell tumour. Furthermore, the presence of an islet cell tumour in the face of hypergastrinaemia does not warrant a diagnosis of an islet cell gastrinoma. Immunohistochemistry is necessary to confirm the diagnosis of a pancreatic islet cell gastrinoma.     

THE DIAGNOSIS AND TREATMENT OF GASTRINOMA:REPORT OF 17 CASES

Objective, To study the experience of diagnosis and treatment of gastrinoma.Methods, A retrospective study of 17 patients with gastrinoma seen from 1978 through 1998 in PUMC hospital.Reaults, Three of the 17 eases were associated with multiple endocrine neoplasia type I (MEN-I) syndrome, Tumors dispersed extensively in pancreas ( 31%), duodenum ( 25%) lymphnode ( 19%) and otherplace (25%), Ultrasoaography and computed tomography(CT)were sensitive in localizing tumor in pancreas liver, but were not so wall for small tumor in duodenum and lymph node. The angiography, percutaneous transhepatic portal sampling (PTPS), scintigraphy and endoscopic uftrasonography (EUS) also can be used. Sixteen of 17 patients underwent 38 operations and 56 percent of the 16 eases underwent several operations, Seven patients at last performed total gastreetomy that was still considered as a choice of treatment, Tumor resection was rare because the advancing of tumor in most cases. Conclusion, Diagnosing gastrinoma in its early stage and striving for the tumor reaection and reducing the metastasis of liver were the cruxes for good prognosis.     

Zollinger-Ellison phenotype in the absence of hypergastrinemia and islet-cell tumor

Patients with the Zollinger-Ellison syndrome are characterized by islet-cell tumors, striking gastric acid hypersecretion, and peptic ulcer disease. They often experience severe abdominal pain, diarrhea, and gastrointestinal bleeding with potentially life-threatening consequences. It is a rare syndrome caused by nonbeta cell islet-cell tumors (gastrinomas) located in or in proximity to the pancreas. These tumors freely secrete gastrin, a peptide hormone that serves as a powerful stimulant of gastric acid secretion. Exuberant secretion of gastrin from the gastrinomas produces severe gastric acid hypersecretion that often leads to impressive peptic ulcer disease and the constellation of symptoms listed above. We describe a patient presenting with clinical manifestations characteristic of the ZES with strikingly elevated gastric acid secretion, multiple ulcers in the first and second portions of the duodenum and diarrhea, but in absence of islet-cell tumor and/or hypergastrinemia.     

The gastrinoma/Zollinger-Ellison syndrome: Statistical evaluation of a Japanese series of 359 cases

The diagnostic modalities and management of gastrinoma/Zollinger-Ellison syndrome (ZES) have been markedly modified and improved over the past 15 years. To evaluate the present status of this disease, in terms of various clinicopathologic features, we collected 359 Japanese cases of gastrinoma/ZES from the literature. We found a decreasing incidence (from 74.7% in 1965–1980 to 34.2% in 1981–1995) of multiple surgeries and a decreasing rate (from 94.3% in 1965–1980 to 83.5% in 1981–1995) of ZES associated with gastrinoma. There was an increasing rate (from 12.6% in 1965–1980 to 48.9% in 1981–1995) of correct preoperative diagnosis. (All these differences were significant; P < 0.01). In 1981–1995, there was a high incidence (51.1%) of small tumors (20 mm or less) and a high rate (39.5%) of metastases, and a relatively favorable postoperative outcome (10-year survival rate of 63.7%); P < 0.05. The diagnosis and treatment of gastrinoma/ZES have been markedly improved by increased rates of curative surgery, and more favorable postoperative outcomes will be expected in decades to come. Received for publication on July 2, 1997; accepted on Aug. 15, 1997