Zollinger-Ellison syndrome 357810 220595308 2008-06-20T16:52:18Z DOI bot 6652755 Citation maintenance. You can [[WP:DOI|use this bot]] yourself! Please [[User:DOI_bot/bugs|report any bugs]]. {{Refimprove|date=October 2007}} {{Infobox_Disease | Name = {{PAGENAME}} | Image = ZES endo.jpg | Caption = Endoscopy image of multiple small [[ulcer]]s in the distal [[duodenum]] in a patient with Zollinger-Ellison syndrome | DiseasesDB = | ICD10 = {{ICD10|E|16|4|e|15}} | ICD9 = {{ICD9|251.5}} | ICDO = | OMIM = | MedlinePlus = 000325 | eMedicineSubj = med | eMedicineTopic = 2437 | eMedicine_mult = {{eMedicine2|ped|2472}} | MeshID = D015043 | }} '''Zollinger-Ellison syndrome''' is a disorder where increased levels of the hormone [[gastrin]] are produced, causing the [[stomach]] to produce excess [[hydrochloric acid]]. Often the cause is a tumor ([[gastrinoma]]) of the [[duodenum]] or [[pancreas]] producing the hormone [[gastrin]]. Gastrin then causes an excessive production of acid which can lead to peptic ulcers (in almost 95% of patients) Gastrin works on stomach [[parietal cell]]s causing them to [[Hydrogen potassium ATPase|secrete]] more [[hydrogen ion]]s into the stomach lumen. In addition, gastrin acts as a trophic factor for parietal cells, causing parietal cell hyperplasia. Thus there is an increase in the number of acid-secreting cells, and each of these cells produces acid at a higher rate. The increase in acidity contributes to the development of [[peptic ulcer]]s in the stomach and duodenum. High acid levels lead to multiple [[ulcer]]s in the stomach and small bowel. Patients with Zollinger-Ellison syndrome may experience abdominal pain and [[diarrhea]]. The diagnosis is also suspected in patients without symptoms who have severe ulceration of the stomach and small bowel, especially if they fail to respond to treatment. Gastrinomas may occur as single tumors or as multiple, small tumors. About one-half to two-thirds of single gastrinomas are [[malignant]] tumors that most commonly spread to the [[liver]] and [[lymph node]]s near the pancreas and small bowel. Nearly 25 percent of patients with gastrinomas have multiple tumors as part of a condition called [[multiple endocrine neoplasia]] type I (MEN I). MEN I patients have tumors in their [[pituitary gland]] and [[parathyroid gland]]s in addition to tumors of the pancreas. ==Symptoms== *[[epigastric]] pain (stomachache) *vomiting blood ([[hematemesis]]) (occasional) *difficulty in eating *[[diarrhea]] *[[steatorrhea]] ==Diagnosis== The diagnosis of Zollinger-Ellison syndrome is made by several laboratory tests and imaging studies. Laboratory tests: * [[secretin]] stimulation test, which measures [[gastrin]] levels in response to secretin * fasting gastrin levels * antral acidity ==Therapy== [[Proton pump inhibitor]]s (such as [[omeprazole]] and [[lansoprazole]]) and histamine [[H2-receptor antagonist]]s (such as [[famotidine]] and [[ranitidine]]) are used to slow down acid secretion. If possible the tumours should be surgically removed, or treated with [[chemotherapy]]. [[Octreotide]] can be used to slow down acid secretion. ==History== This syndrome was first described in [[1955]] by R Zollinger and E Ellison.<ref name="pmid13259432">{{cite journal |author=Zollinger RM, Ellison EH |title=Primary peptic ulcerations of the jejunum associated with islet cell tumors of the pancreas |journal=Ann. Surg. |volume=142 |issue=4 |pages=709–23; discussion, 724–8 |year=1955 |pmid=13259432|doi=10.1097/00000658-195510000-00015}}</ref> ==Footnotes== <references/> {{Endocrine pathology}} [[Category:Digestive diseases]] [[Category:Endocrinology]] [[Category:Syndromes]] [[Category:Eponyms]] [[de:Zollinger-Ellison-Syndrom]] [[es:Síndrome de Zollinger-Ellison]] [[fr:Syndrome de Zollinger-Ellison]] [[it:Sindrome di Zollinger-Ellison]] [[nl:Syndroom van Zollinger-Ellison]] [[pl:Zespół Zollingera-Ellisona]] [[pt:Síndrome de Zollinger-Ellison]] [[ru:Синдром Золлингера — Эллисона]] [[sv:Zollinger-Ellisons syndrom]] [[th:โรคเนื้องอกที่เกิดจากการหลั่งกรดผิดปกติ]]