Zollinger–Ellison syndrome
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| Zollinger–Ellison syndrome | |
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| Classification and external resources | |
 Endoscopy image of multiple small ulcers in the distal duodenum in a patient with Zollinger–Ellison syndrome |
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| ICD-10 | E16.4 |
| ICD-9 | 251.5 |
| MedlinePlus | 000325 |
| eMedicine | med/2437 ped/2472 |
| MeSH | D015043 |
Zollinger–Ellison syndrome is a triad of gastric acid hypersecretion, severe peptic ulceration, and non-beta cell islet tumor of pancreas (gastrinoma). In this syndrome 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.
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[edit] Pathophysiology
Gastrin works on stomach parietal cells 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. 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 multiple peptic ulcers in the stomach and duodenum (small bowel).
[edit] Presentation
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 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 I (MEN I). MEN I patients have tumors in their pituitary gland and parathyroid glands in addition to tumors of the pancreas.
[edit] Symptoms
- Chronic Diarrhea
- Pain in the Esophagus
especially between and after meals at night
- Nausea
- Wheezing
- Vomiting Blood (digested Blood)
- Malnourishment
- loss of weight due to loss of appettite
[edit] Diagnosis
Clinical suspicion of Zollinger–Ellison syndrome may be aroused when the above symptoms prove resistant to treatment, when the symptoms are especially suggestive of the syndrome, or endoscopy is suggestive. The diagnosis of Zollinger–Ellison syndrome is made by several laboratory tests and imaging studies.
- Secretin stimulation test, which measures evoked gastrin levels
- Fasting gastrin levels, on at least three separate occasions[1]
- Gastric acid secretion and pH. Normal basal gastric acid secretion is less than 10 mEq/hour, while in Zollinger–Ellison syndrome it is usually more than 15 mEq/hour.[2]
- Increased level of Chromogranin A is a common marker of endocrin tumors
In addition, the source of the increased gastrin production must be discovered. This is either done using MRI or somatostatin receptor scintigraphy, the investigation of choice.[3]
[edit] Therapy
Proton pump inhibitors (such as omeprazole and lansoprazole) and histamine H2-receptor antagonists (such as famotidine and ranitidine) are used to slow down acid secretion. Cure is only possible if the tumors are surgically removed, or treated with chemotherapy. Octreotide can be used to alleviate symptoms. for reduced concentration of acid hydrochloride
[edit] History
This syndrome was first described in 1955 by Robert Zollinger and Edwin Ellison, surgeons at The Ohio State University.[4]
[edit] References
- ^ http://www.patient.co.uk/showdoc/40001304/
- ^ Page 192 in: Elizabeth D Agabegi; Agabegi, Steven S. (2008). Step-Up to Medicine (Step-Up Series). Hagerstwon, MD: Lippincott Williams & Wilkins. ISBN 0-7817-7153-6.
- ^ Jensen RT (2004). "Gastrinomas: advances in diagnosis and management". Neuroendocrinology 80 Suppl 1: 23–7. doi:10.1159/000080736. PMID 15477712.
- ^ Zollinger RM, Ellison EH (1955). "Primary peptic ulcerations of the jejunum associated with islet cell tumors of the pancreas". Ann. Surg. 142 (4): 709–23; discussion, 724–8. doi:10.1097/00000658-195510000-00015. PMID 13259432.
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