The conditions of the diagnosis of the Zollinger-Ellison syndrome.
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Biomedical subjects
Publications and source records attributed to S Bonfils.
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The large intestine resected from 6 Hirschsprung's patients and surgical colonic biopsies from 6 control children were examined with light and electron microscopy. The presence and the relative distribution of various endocrine cell types in both groups of mucosa were determined. In light microscope studies endocrine cell data were expressed as number of cells per unit area of mucosa using a quantitative method after argentaffin and Grimelius's argyrophilic techniques and an immunoperoxidase reaction with glucagon and somatostatin (SRIF) antisera. The results indicate that endocrine cells are apparently not involved in Hirschsprung's disease, since their number and frequency did not differ significantly between the ganglionic and aganglionic segments of Hirschsprung's patients nor between the latter and control children. Glucagon immunoreactive cells were, on the average, 5-6 times and 7-9 times more numerous that SRIF cells in the rectum and the sigmoid, respectively. Ultrastructurally, five endocrine cell types could be distinguished. The fifth type, probably a transition type, apparently disappears in adults.
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Cimetidine was studied in 8 cases of Zollinger-Ellison syndrome. The average dose was 1,2 g a day for 14 to 195 days. The treatment was remarkably effective in 2 cases, but only partial or temporary improvement was noted in 3 other cases. The last 3 patients failed to respond to treatment. The possibility of a rebound phenomenon requires strict clinical and biological supervision.
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Six patients each with an extensive ulcerating colitis are reported. Common features were a febrile nonbloody diarrhea; radiological involvement of the whole colon with superficial ulcerations and preservation of haustra, and integrity of the terminal ileum and rectum; the absence of any specific pathological finding; and, most important, the spontaneous recovery of the lesions in the absence of any treatment except sympatomatic measures. The present follow up after recovery range from 18 months to 5 years. Four cases were young women taking oral contraceptives and two patients were older. These patients with colitis could represent a new entity or could be a particular form of Crohn's disease of the colon, with delayed diagnostic pathological features.
Five patients with Zollinger-Ellison syndrome received metiamide per os in doses ranging from 600 to 1,200 mg/day for a minimum period of 2 weeks. Drug produced an inhibition of basal gastric acid secretion ranging from 5 to 100% with relief of symptoms. Survey of patients during and after metiamide course showed sometimes a prolonged antisecretory effect up to 26 days after the end of treatment or, on the contrary, a reduced drug activity in spite of increasing doses.
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