Zollinger-Ellison syndrome: Difference between revisions

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'''For patient information click [[{{PAGENAME}} (patient information)|here]]'''
{{GS}}
{{Editor Join}}
{{Infobox_Disease |
{{Infobox_Disease |
   Name          = {{PAGENAME}} |
   Name          = {{PAGENAME}} |
   Image          = ZES endo.jpg |
   Image          = ZES endo.jpg |
   Caption        = Endoscopy image of multiple small [[ulcer]]s in the distal [[duodenum]] in a patient with Zollinger-Ellison syndrome |
   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 |
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{{Zollinger-Ellison}}
__NOTOC__
{{Zollinger-Ellison syndrome}}
'''For patient information click [[{{PAGENAME}} (patient information)|here]]'''
 
{{CMG}} {{AE}} {{ARK}} {{MJK}} {{S.M}}


==Overview==
{{SK}} ZE; Z-E syndrome; Pancreatic Ulcerogenic Tumor Syndrome; Excessive Gastrin Secretion; Gastrinoma; Pancreatic Ulcerogenic Tumor Syndrome; ZES.
'''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.
==[[Zollinger-Ellison syndrome overview|Overview]]==
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.
==[[Zollinger-Ellison syndrome historical perspective|Historical Perspective]]==


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.
==[[Zollinger-Ellison syndrome pathophysiology|Pathophysiology]]==


==Symptoms==
==[[Zollinger-Ellison syndrome causes|Causes]]==
*[[epigastric]] pain (stomachache)
*vomiting blood ([[hematemesis]]) (occasional)
*difficulty in eating
*[[diarrhea]]
*[[steatorrhea]]


==Diagnosis==
==[[Zollinger-Ellison syndrome differential diagnosis|Differentiating Zollinger-Ellison syndrome from other Diseases]]==
The diagnosis of Zollinger-Ellison syndrome is made by several laboratory tests and imaging studies.


Laboratory tests:
==[[Zollinger-Ellison syndrome epidemiology and demographics|Epidemiology and Demographics]]==
* [[secretin]] stimulation test, which measures [[gastrin]] levels in response to secretin
* fasting gastrin levels
* antral acidity


==Therapy==
==[[Zollinger-Ellison syndrome risk factors|Risk Factors]]==
[[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]].
==[[Zollinger-Ellison syndrome screening|Screening]]==
==[[Zollinger-Ellison syndrome natural history|Natural History, Complications and Prognosis]]==


[[Octreotide]] can be used to slow down acid secretion.
==Diagnosis==
[[Zollinger-Ellison syndrome history and symptoms|History and Symptoms]] | [[Zollinger-Ellison syndrome physical examination|Physical Examination]] | [[Zollinger-Ellison syndrome laboratory findings|Laboratory Findings]] | [[Zollinger-Ellison syndrome CT|CT]] | [[Zollinger-Ellison syndrome MRI|MRI]] | [[Zollinger-Ellison syndrome ultrasound|Ultrasound]] | [[Zollinger-Ellison syndrome other imaging findings|Other Imaging Findings]] | [[Zollinger-Ellison syndrome other diagnostic findings|Other Diagnostic Findings]]


==History==
==Treatment==
The disease entity was first described in 1955 by its namesakes: Zollinger RM, Ellison EH. ''Primary peptic ulcerations of the jejunum associated with islet cell tumors of the pancreas.'' Ann Surg 1955;142:709-23. PMID 13259432.
[[Zollinger-Ellison syndrome medical therapy|Medical Therapy]] | [[Zollinger-Ellison syndrome surgery|Surgery]] | [[Zollinger-Ellison syndrome cost-effectiveness of therapy|Cost-Effectiveness of Therapy]] | [[Zollinger-Ellison syndrome future or investigational therapies|Future or Investigational Therapies]]


==External links==
==Case Studies==
* [http://www.bidmc.org/YourHealth/ConditionsAZ.aspx?ChunkID=102706 Beth Israel Deaconess Medical Center: Zollinger-Ellison Syndrome]
[[Zollinger-Ellison syndrome case study one|Case #1]]


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[[Category:Endocrinology]]
[[Category:Endocrinology]]
[[Category:Syndromes]]
[[Category:Syndromes]]
 
[[Category:Oncology]]
[[de:Zollinger-Ellison-Syndrom]]
[[es:Síndrome de Zollinger-Ellison]]
[[fr:Syndrome de Zollinger-Ellison]]
[[pl:Zespół Zollingera-Ellisona]]
[[pt:Síndrome de Zollinger-Ellison]]
[[ru:Синдром Золлингера — Эллисона]]
[[sv:Zollinger-Ellisons syndrom]]
[[th:โรคเนื้องอกที่เกิดจากการหลั่งกรดผิดปกติ]]


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Latest revision as of 23:22, 26 September 2021

Zollinger-Ellison syndrome
Endoscopy image of multiple small ulcers in the distal duodenum in a patient with Zollinger-Ellison syndrome

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: Aravind Reddy Kothagadi M.B.B.S[2] Mohamad Alkateb, MBBCh [3] Shadan Mehraban, M.D.[4]

Synonyms and keywords: ZE; Z-E syndrome; Pancreatic Ulcerogenic Tumor Syndrome; Excessive Gastrin Secretion; Gastrinoma; Pancreatic Ulcerogenic Tumor Syndrome; ZES.

Overview

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