INTERNAL MEDICINE


GISELE DE AZEVEDO PRAZERES, MD

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Zollinger-Ellison Syndrome


Zollinger-Ellison (ZE) is a syndrome caused by a non-beta islet cell tumor biologically malignant in about 60% of cases.The tumor produces gastrin and is associated with gastric acid hypersecretion and peptic ulcer disease. The most common organ involved is the pancreas, but it can also be found in other sites like the stomach, spleen, duodenum, and lymph nodes. The size of the tumor varies from 0,2 to 20 cm and in only 10% of the cases its resectable.

A.CLINICAL MANIFESTATIONS

1)Abdominal Pain resulting from an ulcer is the most common find. In about 70% of the cases the ulcer is located in the duodenal bulb, but also may developt in stomach, postbulbar duodenum, jejunum or esophagus.

2)Endocrine abnormalites commonly occur associated with(ZE). About 20 to 30 percent of patient with (ZE) have Multiple endocrine neoplasia(MEN I)syndrome which consist of pancreatic, pituitary and parathyroid tumors and shows an autossomal dominant pattern of inheritance.Twenty percent of patients have hyperparathyroidism.

3)Diarrhea occurs in about 50% of the patients and results from the large volumes of fluid secreted by the stomach,the right gastrin levels that cause incomplet Na+ and water absortion and the damage caused by the high acid levels in the intestinal mucosa.

B.DIAGNOSIS

1)Patients with (ZE)often have basal gastric acid output rates of more than 10 mEq/hr and basal-to-peak output ratios of greater than 0.6.

These patients demonstrate elevated basal-state gastrin levels that do not increase 1 hour after a meal.Gastrin levels rise by 200 units(rather than fall) after intravenous administration of secretin and increase markedly(rather than modestly) after intravenous calcium administration.

C)THERAPY

1)For many years total gastrectomy was the treatment of choice, but it is not clear if this the best treatment since in some centers the mortality ratio is greater in total gastrectomy than in other surgical procedures for ulcer disease.

2)H2-receptor antagonists combined with anticholinergic agentshas been used in conjuction with vagotomy with drainage.

3) Omeprazole(proton pump inhbitor)is probably the medical treatment of choice.

4)A newly described technique of tumor localization involves sampling gastrin levels through cannulation of multiple pancreatic and abdominal veins.This technique offers the hope of surgical cure of multiple primary tumors.


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