[Zollinger-Ellison syndrome type II due to diffuse hyperplasia of the pancreatic islet cells (author's transl)]. 1979

M J Varas Lorenzo, and J A Curto Cardús, and M Sans Segarra, and M Carrera Plans

A 58-year-old patient with hypergastrinemia (basal and after stimulation by means of protein food, calcium, glucagon, and secretin), acid hypersecretion, recurrent anastomotic ulcer, gastrocolonic fistula, steatorrhea, and malabsortion (hypocalcemia, hypocholesterolemia and a rather elevated 5-HIAA) is reported. The definite preoperative diagnosis of Zollinger-Ellison syndrome was established after the intravenous secretin test (75 U) which produced a significant stimulation peak 5 minutes after being injected. The possible existence of a multiple endocrine adenomatosis syndrome type I was discarded. During the operation no pancreatic or extrapancreatic macroscopic tumor was found. A total gastrectomy, transverse colectomy, splenectomy, and subtotal pancreatic resection were performed; Rosanow's techniques was used to re-established the gastrointestinal continuity. The morphological study of the excised pancreatic tissue showed a diffuse hyperplasia of the Langerhans islet cells; indirect immunofluorescence in the presence of antigastrin antibodies was faintly positive and difficult to evaluate. However, gastrin levels clearly decrease after the operation may be because the inhibitory effect of total gastrectomy or because of the partial pancreatectomy. Furthermore, the inhibitory effect of tyrocalcitonine onthe pre- and postoperative gastrin levels measured by radioimmunoassay could be verified. For the moment the importance of this test in the diagnosis of Zollinger-Ellison syndrome, and especially in the diagnosis of ZES-type II, is not known.

UI MeSH Term Description Entries
D006984 Hypertrophy General increase in bulk of a part or organ due to CELL ENLARGEMENT and accumulation of FLUIDS AND SECRETIONS, not due to tumor formation, nor to an increase in the number of cells (HYPERPLASIA). Hypertrophies
D007275 Injections, Intravenous Injections made into a vein for therapeutic or experimental purposes. Intravenous Injections,Injection, Intravenous,Intravenous Injection
D007515 Islets of Langerhans Irregular microscopic structures consisting of cords of endocrine cells that are scattered throughout the PANCREAS among the exocrine acini. Each islet is surrounded by connective tissue fibers and penetrated by a network of capillaries. There are four major cell types. The most abundant beta cells (50-80%) secrete INSULIN. Alpha cells (5-20%) secrete GLUCAGON. PP cells (10-35%) secrete PANCREATIC POLYPEPTIDE. Delta cells (~5%) secrete SOMATOSTATIN. Islands of Langerhans,Islet Cells,Nesidioblasts,Pancreas, Endocrine,Pancreatic Islets,Cell, Islet,Cells, Islet,Endocrine Pancreas,Islet Cell,Islet, Pancreatic,Islets, Pancreatic,Langerhans Islands,Langerhans Islets,Nesidioblast,Pancreatic Islet
D008297 Male Males
D008875 Middle Aged An adult aged 45 - 64 years. Middle Age
D010180 Pancreatectomy Surgical removal of the pancreas. (Dorland, 28th ed) Pancreatectomies
D002118 Calcium A basic element found in nearly all tissues. It is a member of the alkaline earth family of metals with the atomic symbol Ca, atomic number 20, and atomic weight 40. Calcium is the most abundant mineral in the body and combines with phosphorus to form calcium phosphate in the bones and teeth. It is essential for the normal functioning of nerves and muscles and plays a role in blood coagulation (as factor IV) and in many enzymatic processes. Coagulation Factor IV,Factor IV,Blood Coagulation Factor IV,Calcium-40,Calcium 40,Factor IV, Coagulation
D005743 Gastrectomy Excision of the whole (total gastrectomy) or part (subtotal gastrectomy, partial gastrectomy, gastric resection) of the stomach. (Dorland, 28th ed) Gastrectomies
D005755 Gastrins A family of gastrointestinal peptide hormones that excite the secretion of GASTRIC JUICE. They may also occur in the central nervous system where they are presumed to be neurotransmitters. Gastrin
D005934 Glucagon A 29-amino acid pancreatic peptide derived from proglucagon which is also the precursor of intestinal GLUCAGON-LIKE PEPTIDES. Glucagon is secreted by PANCREATIC ALPHA CELLS and plays an important role in regulation of BLOOD GLUCOSE concentration, ketone metabolism, and several other biochemical and physiological processes. (From Gilman et al., Goodman and Gilman's The Pharmacological Basis of Therapeutics, 9th ed, p1511) Glucagon (1-29),Glukagon,HG-Factor,Hyperglycemic-Glycogenolytic Factor,Proglucagon (33-61),HG Factor,Hyperglycemic Glycogenolytic Factor

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