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{{short description|Condition in which tumours stimulate excessive gastric acid production}}
{{Refimprove|date=October 2007}}
{{Infobox medical condition (new) {{Infobox medical condition (new)
| name = Zollinger–Ellison syndrome | name = Zollinger–Ellison syndrome
| image = ZES endo.jpg | image = ZES endo.jpg
| caption = Endoscopy image of multiple small ] in the distal ] in a patient with Zollinger–Ellison syndrome | caption = Endoscopy image of multiple small ] in the distal ] in a patient with Zollinger–Ellison syndrome
| |
| pronounce = | pronounce =
| field = | field =
| synonyms = Gastrinoma, Pancreatic Ulcerogenic Tumor Syndrome, ZES, Z-E Syndrome<ref>{{cite web|title=Zollinger Ellison Syndrome|url=https://rarediseases.org/rare-diseases/zollinger-ellison-syndrome/|website=NORD|accessdate=16 July 2018|language=en|date=}}</ref> | synonyms = gastrinoma, pancreatic ulcerogenic tumor syndrome, ZES, Z-E syndrome<ref>{{cite web|title=Zollinger Ellison syndrome|url=https://rarediseases.org/rare-diseases/zollinger-ellison-syndrome/|website=NORD|access-date=16 July 2018}}</ref>
| symptoms = | symptoms =
| complications = | complications =
| onset = | onset =
| duration = | duration =
| types = | types =
| causes = | causes = ]
| risks = | risks =
| diagnosis = | diagnosis =
| differential = | differential =
| prevention = | prevention =
| treatment = | treatment =
| medication = | medication =
| prognosis = | prognosis =
| frequency = | frequency =
| deaths = | deaths =
}} |alt=}}
'''Zollinger–Ellison syndrome''' ('''ZES''') is a disease in which tumors cause the stomach to produce too much acid, resulting in ]. Symptoms include ] and ]. '''Zollinger–Ellison syndrome''' ('''Z-E syndrome''') is a rare disease in which tumors cause the stomach to produce too much acid, resulting in ]s. Symptoms include ] and ].


The syndrome is caused by a ], a ] that secretes a hormone called ].<ref name=":0">{{Cite news|url=http://www.mayoclinic.org/diseases-conditions/zollinger-ellison-syndrome/basics/definition/con-20024097|title=Zollinger-Ellison syndrome|last=|first=|date=|work=|access-date=2017-02-27|archive-url=|archive-date=|dead-url=|publisher=Mayo Clinic|language=en}}</ref> The tumor causes excessive production of ], which leads to the growth of ] and proliferation of ] and ]. The syndrome is caused by a ], a ] that secretes a hormone called ].<ref name=":0">{{Cite news|url=http://www.mayoclinic.org/diseases-conditions/zollinger-ellison-syndrome/basics/definition/con-20024097|title=Zollinger-Ellison syndrome|access-date=2017-02-27|publisher=Mayo Clinic}}</ref> Too much gastrin in the blood (hypergastrinemia) results in the overproduction of ] by parietal cells in the stomach. Gastrinomas most commonly arise in the duodenum, pancreas or stomach.{{cn|date=May 2022}}


In 75% of cases Zollinger–Ellison syndrome occurs sporadically, while in 25% of cases it occurs as part of an ] syndrome called ] (MEN 1).<ref>{{Cite journal|title=Gastrinoma (Duodenal and Pancreatic)|journal = Neuroendocrinology|volume = 84|issue = 3|pages = 173–82|last1=Rt|first1=Jensen|last2=B|first2=Niederle|date=2006|language=en|pmid=17312377|last3=E|first3=Mitry|last4=Jk|first4=Ramage|last5=T|first5=Steinmuller|last6=V|first6=Lewington|last7=A|first7=Scarpa|last8=A|first8=Sundin|last9=A|first9=Perren|doi = 10.1159/000098009|s2cid = 5096249| url=https://boris.unibe.ch/20316/ }}</ref>
ZES may occur on its own or as part of an ] syndrome called ] (MEN 1). The primary tumor is usually located in the ], ] or abdominal ]s, but ectopic locations (e.g., ], ], ], ], and ]) have also been described.<ref>{{EMedicine|article|183555|Zollinger-Ellison Syndrome}}</ref>


==Signs and symptoms== ==Signs and symptoms==
Patients with Zollinger–Ellison syndrome may experience abdominal pain and diarrhea.<ref name=":0" /> The diagnosis is also suspected in patients who have severe ulceration of the stomach and small bowel, especially if they fail to respond to treatment.{{Citation needed|date=July 2013}} Patients with Zollinger–Ellison syndrome may experience abdominal pain and diarrhea.<ref name=":0" /> If left untreated, the condition could result in severe gastroesophageal reflux disease (GERD) and refractory peptic ulcer disease.<ref>{{Cite journal |last1=Norton |first1=Jeffrey A. |last2=Foster |first2=Deshka S. |last3=Ito |first3=Tetsuhide |last4=Jensen |first4=Robert T. |date=September 2018 |title=Gastrinomas |journal=Endocrinology and Metabolism Clinics of North America |language=en |volume=47 |issue=3 |pages=577–601 |doi=10.1016/j.ecl.2018.04.009 |pmc=6092039 |pmid=30098717}}</ref> The diagnosis is also suspected in patients who have severe ulceration of the stomach and small bowel, especially if they fail to respond to treatment.<ref>{{cite web |url=https://www.niddk.nih.gov/health-information/digestive-diseases/zollinger-ellison-syndrome| title=Zollinger-Ellison Syndrome
|website= National Institute of Diabetes and Digestive and Kidney Diseases (NIDDK) |access-date= 22 July 2021}}</ref>
*Chronic diarrhea, including ] (fatty stools) * Chronic diarrhea, including ] (fatty stools)
*Pain in the esophagus, especially between and after meals at night * Pain in the esophagus, especially between and after meals at night
*Nausea * Nausea
*Wheezing * Wheezing
*Vomiting blood * Vomiting blood
*Malnourishment * Malnourishment
*Loss of appetite * Loss of appetite
* ]


]s may occur as single tumors or as multiple small tumors. About one-half to two-thirds of single gastrinomas are ] tumors that most commonly spread to the ] and to ]s near the pancreas and small bowel. ]s may occur as single tumors or as multiple small tumors. About one-half to two-thirds of single gastrinomas are ] tumors that most commonly spread to the ] and to ]s near the pancreas and small bowel.<ref>{{cite web |url=https://www.lecturio.com/concepts/gastrinoma/| title=Gastrinoma|website=The Lecturio Medical Concept Library |access-date= 22 July 2021}}</ref>
⚫ Nearly 25 percent of patients with gastrinomas have multiple tumors as part of a condition called ] (MEN 1). MEN I patients have tumors in their ] and ]s, in addition to tumors of the pancreas.<ref>{{Cite journal|last=Thakker|first=Rajesh V.|date=June 2010|title=Multiple endocrine neoplasia type 1 (MEN1)|journal=Best Practice & Research Clinical Endocrinology & Metabolism|volume=24|issue=3|pages=355–370|doi=10.1016/j.beem.2010.07.003|pmid=20833329|issn=1521-690X}}</ref>{{Citation needed|date=July 2013}}

⚫ Nearly 25 percent of patients with gastrinomas have multiple tumors as part of a condition called ] (MEN 1). MEN I patients have tumors in their ] and ]s, in addition to tumors of the pancreas.{{Citation needed|date=July 2013}}


==Pathophysiology== ==Pathophysiology==
] works on the ]s of the ], causing them to secrete more ]s into the stomach ]. In addition, gastrin acts as a ] for parietal cells, causing parietal cell ]. 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 ]s in the stomach and ] (first portion of the small bowel).{{Citation needed|date=July 2013}} ] works on the ]s of the ], causing them to secrete more ]s into the stomach ]. In addition, gastrin acts as a ] for parietal cells, causing parietal cell ]. Normally, hydrogen ion secretion is controlled by a ] loop by gastric cells to maintain a suitable pH, however, the neuroendocrine tumor that is present in individuals with Zollinger–Ellison Syndrome has no regulation, resulting in excessively large amounts of secretion.<ref>Cho MS, Kasi A. Zollinger Ellison Syndrome. . In: StatPearls . Treasure Island (FL): StatPearls Publishing; 2020 Jan-. Available from: https://www.ncbi.nlm.nih.gov/books/NBK537344/</ref><ref>{{cite journal |last1=Cho |first1=Min S. |last2=Kasi |first2=Anup |title=Zollinger Ellison Syndrome |url=https://www.ncbi.nlm.nih.gov/books/NBK537344/ |website=In: StatPearls |year=2022 |publisher=StatPearls Publishing |pmid=30726029 |access-date=1 December 2020 |ref=16}}</ref> 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 ]s in the stomach, ] (first portion of the small bowel) and occasionally the jejunum (second portion of the small bowel), the last of which is an 'atypical' ulcer.<ref>{{Cite journal|last1=Meko, M.D|first1=J. B.|last2=Norton, M.D|first2=J. A.|date=February 1995|journal=Annual Review of Medicine|volume=46|issue=1|pages=395–411|doi=10.1146/annurev.med.46.1.395|pmid=7598474|issn=0066-4219|title=Management of Patients with Zollinger-Ellison Syndrome}}</ref>


==Diagnosis== ==Diagnosis==
Zollinger–Ellison syndrome may be suspected when the above symptoms prove resistant to treatment, when the symptoms are especially suggestive of the syndrome, or when ] is suggestive. The diagnosis is made through several laboratory tests and imaging studies:<ref>{{Cite book|url=https://books.google.com/books?id=SaQKSRwPmsYC&pg=PA70&dq=Syndrome+de+Zollinger-Ellison&hl=en&sa=X&ved=0ahUKEwi0nOHWx7vYAhXBbRQKHZgSCggQ6AEIPzAF#v=onepage&q=Syndrome%20de%20Zollinger-Ellison&f=false|title=Endocrinologie|last=Hennen|first=Georges|date=2001-10-03|publisher=De Boeck Supérieur|year=|isbn=9782804138165|location=|pages=|language=fr}}</ref> Zollinger–Ellison syndrome may be suspected when the above symptoms prove resistant to treatment when the symptoms are especially suggestive of the syndrome, or when ] is suggestive. The diagnosis is made through several laboratory tests and imaging studies:<ref>{{Cite book|url=https://books.google.com/books?id=SaQKSRwPmsYC&q=Syndrome+de+Zollinger-Ellison&pg=PA70|title=Endocrinologie|last=Hennen|first=Georges|date=2001-10-03|publisher=De Boeck Supérieur|isbn=9782804138165|language=fr}}</ref>
⚫ * ] stimulation test, which measures evoked gastrin levels. Note that the mechanism underlying this test is in contrast to the normal physiologic mechanism whereby secretin inhibits ] release from G cells. Gastrinoma cells release gastrin in response to secretin stimulation,<ref name=pmid982259>{{cite journal |pmid=982259 |year=1976 |last1=Bradley |first1=E L |title=Diagnosis of gastrinoma by the secretin suppression test |journal=Surgery, Gynecology & Obstetrics |volume=143 |issue=5 |pages=784–8 |last2=Galambos |first2=J T }}</ref><ref name=pmid2565843>{{cite journal |pmid=2565843 |year=1989 |last1=Chiba |first1=T |title=Mechanism for increase of gastrin release by secretin in Zollinger-Ellison syndrome |journal=Gastroenterology |volume=96 |issue=6 |pages=1439–44 |last2=Yamatani |first2=T |last3=Yamaguchi |first3=A |last4=Morishita |first4=T |last5=Nakamura |first5=A |last6=Kadowaki |first6=S |last7=Fujita |first7=T |doi=10.1016/0016-5085(89)90510-6 }}</ref> thereby providing a sensitive means of differentiation.

⚫ * Fasting gastrin levels on at least three occasions<ref>{{cite web|url=http://patient.info/doctor/zollinger-ellison-syndrome|title=Zollinger–Ellison Syndrome. Information about ZES Syndrome|website=Patient.info|access-date=14 January 2018}}</ref>
⚫ * ] stimulation test, which measures evoked gastrin levels. Note that the mechanism underlying this test is in contrast to the normal physiologic mechanism whereby secretin inhibits ] release from G cells. Gastrinoma cells release gastrin in response to secretin stimulation,<ref name=pmid982259>{{cite journal |pmid=982259 |year=1976 |author1=Bradley |first1=E L |title=Diagnosis of gastrinoma by the secretin suppression test |journal=Surgery, Gynecology & Obstetrics |volume=143 |issue=5 |pages=784–8 |last2=Galambos |first2=J T }}</ref><ref name=pmid2565843>{{cite journal |pmid=2565843 |year=1989 |author1=Chiba |first1=T |title=Mechanism for increase of gastrin release by secretin in Zollinger-Ellison syndrome |journal=Gastroenterology |volume=96 |issue=6 |pages=1439–44 |last2=Yamatani |first2=T |last3=Yamaguchi |first3=A |last4=Morishita |first4=T |last5=Nakamura |first5=A |last6=Kadowaki |first6=S |last7=Fujita |first7=T }}</ref> thereby providing a sensitive means of differentiation.
⚫ * Gastric acid secretion and pH (normal basal gastric acid secretion is less than 10 mEq/hour; in Zollinger–Ellison patients, it is usually more than 15 mEq/hour)<ref name=agabegi2nd192>{{cite book |first1=Elizabeth|last1=D Agabegi |author2=Agabegi, Steven S|editor1-last=Duncan|editor1-first=Mark D|editor2-last=Chuang|editor2-first=Kelley|title=Step-Up to Medicine |url=https://pre-med.jumedicine.com/wp-content/uploads/sites/7/2020/10/step-up-to-medicine-5th.pdf|edition=5th|publisher=Wolters Kluwer
⚫ * Fasting gastrin levels on at least three separate occasions<ref>{{cite web|url=http://patient.info/doctor/zollinger-ellison-syndrome|title=Zollinger-Ellison Syndrome. Information about ZES Syndrome|website=Patient.info|accessdate=14 January 2018}}</ref>
|year=2020|page=415 (of PDF)|isbn=978-1-9751-0362-0}}</ref>
⚫ * Gastric acid secretion and pH (normal basal gastric acid secretion is less than 10 mEq/hour; in Zollinger–Ellison patients, it is usually more than 15 mEq/hour)<ref name=agabegi2nd192>{{cite book |author=Elizabeth D Agabegi |author2=Agabegi, Steven S |title=Step-Up to Medicine |series=Step-Up |publisher=Lippincott Williams & Wilkins |location=Hagerstwon, MD |year=2008 |page=192 |isbn=0-7817-7153-6}}</ref>
* An increased level of ] is a common marker of neuroendocrine tumors. * An increased level of ] is a common marker of neuroendocrine tumors.


In addition, the source of the increased gastrin production must be determined using ] or ].<ref>{{cite journal |author=Jensen RT |title=Gastrinomas: advances in diagnosis and management |journal=Neuroendocrinology |volume=80 Suppl 1 |issue= |pages=23–7 |year=2004 |pmid=15477712 |doi=10.1159/000080736}}</ref> In addition, the source of the increased gastrin production must be determined using ] or ].<ref>{{cite journal |author=Jensen RT |title=Gastrinomas: advances in diagnosis and management |journal=Neuroendocrinology |volume=80 |pages=23–7 |year=2004 |issue=Suppl 1 |pmid=15477712 |doi=10.1159/000080736|s2cid=44311651 }}</ref>


==Treatment== ==Treatment==
]s (such as ] and ]) and histamine ]s (such as ] and ]) are used to slow acid secretion. Once gastric acid is suppressed, symptoms normally improve. Surgery to remove peptic ulcers or tumors might also be considered.<ref>{{Cite web|url=https://rarediseases.info.nih.gov/diseases/7918/zollinger-ellison-syndrome|title=Zollinger-Ellison syndrome {{!}} Genetic and Rare Diseases Information Center (GARD) – an NCATS Program|website=rarediseases.info.nih.gov|language=en|access-date=2018-04-17}}</ref> ]s (such as ] and ]) and histamine ]s (such as ] and ]) are used to slow acid secretion. Once gastric acid is suppressed, symptoms normally improve. Surgery to remove peptic ulcers or tumors might also be considered.<ref>{{Cite web|url=https://rarediseases.info.nih.gov/diseases/7918/zollinger-ellison-syndrome|title=Zollinger-Ellison syndrome {{!}} Genetic and Rare Diseases Information Center (GARD) – an NCATS Program|website=rarediseases.info.nih.gov|access-date=2018-04-17}}</ref>


== Epidemiology == == Epidemiology ==
The condition most commonly affects people between the ages of 30 and 60.<ref>{{cite web|url=https://rarediseases.org/rare-diseases/zollinger-ellison-syndrome/|title=Zollinger Ellison Syndrome - NORD (National Organization for Rare Disorders)|website=Rarediseases.org|accessdate=14 January 2018}}</ref> The prevalence in unknown, but estimated to be about 1 per 100,000 people.<ref>{{cite web|url=http://www.orpha.net/consor/cgi-bin/Disease_Search.php?lng=EN&data_id=3595&Disease_Disease_Search_diseaseGroup=Zollinger-Ellison-syndrome&Disease_Disease_Search_diseaseType=Pat&Disease(s)/group+of+diseases=Zollinger-Ellison-syndrome&title=Zollinger-Ellison-syndrome&search=Disease_Search_Simple|title=Orphanet: Zollinger Ellison syndrome|website=Orpha.net|accessdate=14 January 2018}}</ref> The condition most commonly affects people between the ages of 30 and 60.<ref>{{cite web|url=https://rarediseases.org/rare-diseases/zollinger-ellison-syndrome/|title=Zollinger Ellison Syndrome - NORD (National Organization for Rare Disorders)|website=Rarediseases.org|access-date=14 January 2018}}</ref> The prevalence is unknown, but estimated to be about 1 in 100,000 people.<ref>{{cite web|url=http://www.orpha.net/consor/cgi-bin/Disease_Search.php?lng=EN&data_id=3595&Disease_Disease_Search_diseaseGroup=Zollinger-Ellison-syndrome&Disease_Disease_Search_diseaseType=Pat&Disease(s)/group+of+diseases=Zollinger-Ellison-syndrome&title=Zollinger-Ellison-syndrome&search=Disease_Search_Simple|title=Orphanet: Zollinger Ellison syndrome|website=Orpha.net|access-date=14 January 2018}}</ref>


==History== ==History==
Sporadic reports of unusual cases of peptic ulceration in the presence of pancreatic tumors occurred prior to 1955, but R. M. Zollinger and E. H. Ellison, surgeons at ], were the first to postulate a causal relationship between these findings. The ] meeting in Philadelphia in April 1955 heard the first public description of the syndrome, and Zollinger and Ellison subsequently published their findings in '']''.<ref name="pmid13259432"> Sporadic reports of unusual cases of peptic ulceration in the presence of pancreatic tumors occurred prior to 1955, but ] and Edwin H. Ellison, surgeons at ]<!--Wikipedians do not use "The" as part of Ohio State's name; it is considered a marketing gimmick, and routinely deleted.-->, were the first to postulate a causal relationship between these findings. The ] meeting in Philadelphia in April 1955 heard the first public description of the syndrome, and Zollinger and Ellison subsequently published their findings in '']''.<ref name="pmid13259432">
{{cite journal |vauthors=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 |pmc=1465210}} {{cite journal |vauthors=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 |pmc=1465210}}
</ref> </ref>
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==References== ==References==
{{Reflist}} {{Reflist}}

== External links ==
{{Medical resources {{Medical resources
| DiseasesDB = | DiseasesDB =
| ICD10 = {{ICD10|E|16|4|e|15}} | ICD10 = {{ICD10|E|16|4|e|15}}
| ICD9 = {{ICD9|251.5}} | ICD9 = {{ICD9|251.5}}
| ICDO = | ICDO =
| OMIM = | OMIM =
| MedlinePlus = 000325 | MedlinePlus = 000325
| eMedicineSubj = med | eMedicineSubj = med
| eMedicineTopic = 2437 | eMedicineTopic = 2437
| eMedicine_mult = {{eMedicine2|ped|2472}} | eMedicine_mult = {{eMedicine2|ped|2472}}
| MeshID = D015043 | MeshID = D015043
}} }}
{{Digestive system diseases}}
{{Endocrine pathology}}
{{Paraneoplastic syndromes}} {{Paraneoplastic syndromes}}


{{DEFAULTSORT:Zollinger-Ellison syndrome}} {{DEFAULTSORT:Zollinger-Ellison syndrome}}
] ]
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Latest revision as of 07:06, 22 November 2024

Condition in which tumours stimulate excessive gastric acid production Medical condition
Zollinger–Ellison syndrome
Other namesgastrinoma, pancreatic ulcerogenic tumor syndrome, ZES, Z-E syndrome
Endoscopy image of multiple small ulcers in the distal duodenum in a patient with Zollinger–Ellison syndrome
SpecialtyEndocrinology Edit this on Wikidata
CausesGastrinoma

Zollinger–Ellison syndrome (Z-E syndrome) is a rare disease in which tumors cause the stomach to produce too much acid, resulting in peptic ulcers. Symptoms include abdominal pain and diarrhea.

The syndrome is caused by a gastrinoma, a neuroendocrine tumor that secretes a hormone called gastrin. Too much gastrin in the blood (hypergastrinemia) results in the overproduction of gastric acid by parietal cells in the stomach. Gastrinomas most commonly arise in the duodenum, pancreas or stomach.

In 75% of cases Zollinger–Ellison syndrome occurs sporadically, while in 25% of cases it occurs as part of an autosomal dominant syndrome called multiple endocrine neoplasia type 1 (MEN 1).

Signs and symptoms

Patients with Zollinger–Ellison syndrome may experience abdominal pain and diarrhea. If left untreated, the condition could result in severe gastroesophageal reflux disease (GERD) and refractory peptic ulcer disease. The diagnosis is also suspected in patients who have severe ulceration of the stomach and small bowel, especially if they fail to respond to treatment.

  • Chronic diarrhea, including steatorrhea (fatty stools)
  • Pain in the esophagus, especially between and after meals at night
  • Nausea
  • Wheezing
  • Vomiting blood
  • Malnourishment
  • Loss of appetite
  • Malabsorption

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 to lymph nodes 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 1 (MEN 1). MEN I patients have tumors in their pituitary gland and parathyroid glands, in addition to tumors of the pancreas.

Pathophysiology

Gastrin works on the parietal cells of the gastric glands, causing them to secrete more hydrogen ions into the stomach lumen. In addition, gastrin acts as a trophic factor for parietal cells, causing parietal cell hyperplasia. Normally, hydrogen ion secretion is controlled by a negative feedback loop by gastric cells to maintain a suitable pH, however, the neuroendocrine tumor that is present in individuals with Zollinger–Ellison Syndrome has no regulation, resulting in excessively large amounts of secretion. 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 ulcers in the stomach, duodenum (first portion of the small bowel) and occasionally the jejunum (second portion of the small bowel), the last of which is an 'atypical' ulcer.

Diagnosis

Zollinger–Ellison syndrome may be suspected when the above symptoms prove resistant to treatment when the symptoms are especially suggestive of the syndrome, or when endoscopy is suggestive. The diagnosis is made through several laboratory tests and imaging studies:

  • Secretin stimulation test, which measures evoked gastrin levels. Note that the mechanism underlying this test is in contrast to the normal physiologic mechanism whereby secretin inhibits gastrin release from G cells. Gastrinoma cells release gastrin in response to secretin stimulation, thereby providing a sensitive means of differentiation.
  • Fasting gastrin levels on at least three occasions
  • Gastric acid secretion and pH (normal basal gastric acid secretion is less than 10 mEq/hour; in Zollinger–Ellison patients, it is usually more than 15 mEq/hour)
  • An increased level of chromogranin A is a common marker of neuroendocrine tumors.

In addition, the source of the increased gastrin production must be determined using MRI or somatostatin receptor scintigraphy.

Treatment

Proton pump inhibitors (such as omeprazole and lansoprazole) and histamine H2-receptor antagonists (such as famotidine and ranitidine) are used to slow acid secretion. Once gastric acid is suppressed, symptoms normally improve. Surgery to remove peptic ulcers or tumors might also be considered.

Epidemiology

The condition most commonly affects people between the ages of 30 and 60. The prevalence is unknown, but estimated to be about 1 in 100,000 people.

History

Sporadic reports of unusual cases of peptic ulceration in the presence of pancreatic tumors occurred prior to 1955, but Robert M. Zollinger and Edwin H. Ellison, surgeons at Ohio State University, were the first to postulate a causal relationship between these findings. The American Surgical Association meeting in Philadelphia in April 1955 heard the first public description of the syndrome, and Zollinger and Ellison subsequently published their findings in Annals of Surgery.

References

  1. "Zollinger Ellison syndrome". NORD. Retrieved 16 July 2018.
  2. ^ "Zollinger-Ellison syndrome". Mayo Clinic. Retrieved 2017-02-27.
  3. Rt, Jensen; B, Niederle; E, Mitry; Jk, Ramage; T, Steinmuller; V, Lewington; A, Scarpa; A, Sundin; A, Perren (2006). "Gastrinoma (Duodenal and Pancreatic)". Neuroendocrinology. 84 (3): 173–82. doi:10.1159/000098009. PMID 17312377. S2CID 5096249.
  4. Norton, Jeffrey A.; Foster, Deshka S.; Ito, Tetsuhide; Jensen, Robert T. (September 2018). "Gastrinomas". Endocrinology and Metabolism Clinics of North America. 47 (3): 577–601. doi:10.1016/j.ecl.2018.04.009. PMC 6092039. PMID 30098717.
  5. "Zollinger-Ellison Syndrome". National Institute of Diabetes and Digestive and Kidney Diseases (NIDDK). Retrieved 22 July 2021.
  6. "Gastrinoma". The Lecturio Medical Concept Library. Retrieved 22 July 2021.
  7. Thakker, Rajesh V. (June 2010). "Multiple endocrine neoplasia type 1 (MEN1)". Best Practice & Research Clinical Endocrinology & Metabolism. 24 (3): 355–370. doi:10.1016/j.beem.2010.07.003. ISSN 1521-690X. PMID 20833329.
  8. Cho MS, Kasi A. Zollinger Ellison Syndrome. . In: StatPearls . Treasure Island (FL): StatPearls Publishing; 2020 Jan-. Available from: https://www.ncbi.nlm.nih.gov/books/NBK537344/
  9. Cho, Min S.; Kasi, Anup (2022). "Zollinger Ellison Syndrome". In: StatPearls . StatPearls Publishing. PMID 30726029. Retrieved 1 December 2020.
  10. Meko, M.D, J. B.; Norton, M.D, J. A. (February 1995). "Management of Patients with Zollinger-Ellison Syndrome". Annual Review of Medicine. 46 (1): 395–411. doi:10.1146/annurev.med.46.1.395. ISSN 0066-4219. PMID 7598474.
  11. Hennen, Georges (2001-10-03). Endocrinologie (in French). De Boeck Supérieur. ISBN 9782804138165.
  12. Bradley, E L; Galambos, J T (1976). "Diagnosis of gastrinoma by the secretin suppression test". Surgery, Gynecology & Obstetrics. 143 (5): 784–8. PMID 982259.
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ClassificationD
External resources
Diseases of the human digestive system
Upper GI tract
Esophagus
Stomach
Lower GI tract
Enteropathy
Small intestine
(Duodenum/Jejunum/Ileum)
Large intestine
(Appendix/Colon)
Large and/or small
Rectum
Anal canal
GI bleeding
Accessory
Liver
Gallbladder
Bile duct/
Other biliary tree
Pancreatic
Other
Hernia
Peritoneal
Paraneoplastic syndromes
Endocrine
Hematological
Neurological
Musculoskeletal
Mucocutaneous
Reactive erythema
Papulosquamous
Other
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