Name
ZOLLINGER-ELLISON SYNDROME ( GASTRINOMA )
DESCRIPTION
DETAIL
D.D. : ( CONDITIONS WITH HYPERGASTRINEMIA ) - GASTRINOMAS - RETAINED ANTRUM SYNDROME - H. PYLORI INFECTION - ANTRAL G CELL HYPERPLASIA / HYPERFUNCTION - GASTRIC OUTLET OBSTRUCTION - HYPERCALCEMIA - ACHLORHYDRIA - RENAL FAILURE ( RARELY ) - OTHERS LESS COMMON LIKE * DIABETES * RHEUMATOID ARTHRITIS * VITILIGO * PHEOCHROMOCYTOMA * USE OF H2 BLOCKERS & PROTON PUMP INHIBITORSOTHER TESTS : * GASTRIC ACID ANALYSIS- HIGH GASTRIC ACIDITY ( PH < 3.0 ) * FASTING GASTRIN LEVELS , NORMAL < 150 PG/ML , > 150 TO 200 PG / ML ( OR >1000 NG/ L ; 10 TIMES NORMAL ) IS DIAGNOSTIC * BASAL GASTRIC ACID OUTPUT( HYPERCHLORHYDRIA ) - IN > 80% BAO IS ELEVATED ( 15 MEQ / H ) . *SECRETIN ( 2 IU/KG OF BODY WT I.V.) STIMULATION TEST - POSITIVE IN > 80% ( >200 NG/L INCREASE IN SERUM GASTRIN LEVEL WITHIN 15 MTS OF IV SECRETIN) * ULTRA SOUND ABDOMEN, CT ABDOMEN, CELIAC ANGIOGRAPHY, AORTOGRAPHY ARE FREQUENTLY INSENSITIVE & MAY LOCALIZE ABOUT 60% OF GASTRINOMAS. * ENDOSCOPIC ULTRA SOUND OR INTRAOPERATIVE ULTRA SOUND * TRANSHEPATIC PORTAL VENOUS SAMPLE MAY LOCALIZE OCCULT TUMORS. * LOCALIZATION MAY BE DONE BY COMPARISION OF GASTRIN LEVELS IN THE RT. HEPATIC VEIN FOLLOWING SELECTIVE INFUSION OF THE INTRAPANCREATIC ARTRIES WITH SECRETIN.
TYPENOTES
MAY OCCUR AS PART OF AUTOSOMAL DOMINANT FAMILIAL SYNDROME CALLED MEN TYPE 1Medical Care: " The goals of treatment are medical control of gastric acid hypersecretion and surgical resection of the tumor. o If the patient is acutely ill, immediate control of gastric acid hypersecretion can be achieved with intravenous proton pump inhibitors. Previously, this was accomplished with histamine 2 (H2) receptor blockers. Intravenous pantoprazole was approved recently by the US Food and Drug Administration. Proton pump inhibitors are superior to H2 blockers for the control of gastric acid hypersecretion. o Patients who are candidates for surgical resection should be referred for resection of the tumor. o For patients with metastatic disease, chemotherapy, interferon, and octreotide may be helpful. The response to these agents in most studies has been low. Liver transplantation for hepatic metastasis also has been reported. For patients with a single confined liver metastatic lesion, surgical resection may be attempted. Surgical Care: " All patients with sporadic ZES without hepatic metastases or medical contraindications to surgery are advised to undergo surgical resection of the tumor because this decreases the risk of developing liver metastases, which can decrease the survival of these patients. " The role and timing of surgical resection in patients with MEN 1 is less clear. An attempt at surgical resection has been recommended if the tumor is larger than 2.5 cm. Cure is rarely achieved by surgical resection in patients with MEN 1; however, it may reduce the risk of subsequent metastatic disease. " Because this is a rare tumor, surgical resection should be attempted only at centers with personnel experienced in treating patients with ZES.
RELATED DISEASE
Not Available Disease
DISEASE
INVESTIGATION
COMPLETE BLOOD COUNT, ENDOSCOPY UPPER G.I.