Gastric Hypersecretion, Peptic Ulceration and Islet-Cell Tumor of the Pancreas (the Zollinger–Ellison Syndrome)

Donaldson, Robert M.; Eigen, Paul R. vom; Dwight, Richard W.

doi:10.1056/nejm195711142572003

Cited by 83 publications

(13 citation statements)

References 16 publications

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“…Five of the 107 NIH patients with MEN1/ZES did not have hyperparathyroidism. The 240 patients in the literature were from 146 reports 1,12,16,22,31,40,47,50,63,65,70,[72][73][74]79,87,93,94,96,[101][102][103][104][107][108][109]116,120,121,123,126,130,132,135,148,149,152,157,161,170,[173][174][175]177,179,183,186,192,195,210,211,…”

Section: Methodsmentioning

confidence: 99%

Multiple Endocrine Neoplasia Type 1 and Zollinger-Ellison Syndrome

et al. 2004

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In patients with multiple endocrine neoplasia type 1 (MEN1), the most common functional pancreatic endocrine tumor (PET) syndrome is Zollinger-Ellison syndrome (ZES). ZES has been well studied in its sporadic form (that is, without MEN1); however, there are limited data on patients with MEN1 and ZES (MEN1/ZES), and the long-term natural history is largely unknown. To address this issue we report the results of a prospective long-term National Institutes of Health (NIH) study of 107 MEN1/ZES patients and compare our results with those of 1009 MEN1/ZES patients in 278 case reports and small series in the literature. Patients were clinically, radiologically, and biochemically evaluated yearly for all MEN1 manifestations (mean follow-up, 10 yr; range, 0.1-31 yr). Compared with patients from the literature, the NIH MEN1/ZES patients more frequently had pituitary (60%) and adrenal (45%) disease and carcinoid tumors (30%), but had equal frequency of hyperparathyroidism (94%), thyroid disease (6%), or lipomas (5%). Twenty-five percent of both the NIH and the literature patients lacked a family history of MEN1; ZES was the initial clinical manifestation of MEN1 in 40%. ZES onset preceded the diagnosis of hyperparathyroidism in 45%. However, ZES was rarely (8%) the only initial manifestation of MEN1 if careful testing was done. ZES occurred before age 40 years in 50%-60% of the current patients, in contrast to older studies. The diagnosis of ZES is delayed 3-5 years from its onset and is delayed as long as in sporadic ZES cases. Pituitary disease and carcinoid tumors (gastric > bronchial, thymic) are more frequent than generally reported, whereas a second functional PET is uncommon. In patients with MEN1/ZES without a family history of MEN1, the MEN1 manifestations are not as severe. This study shows that MEN1/ZES patients differ in many aspects from those commonly reported in older studies involving few MEN1/ZES patients. In this study we have identified a number of important clinical and laboratory features of MEN1/ZES that were not previously appreciated, which should contribute to earlier diagnosis and improve both short- and long-term management.

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Section: Methodsmentioning

confidence: 99%

Multiple Endocrine Neoplasia Type 1 and Zollinger-Ellison Syndrome

et al. 2004

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“…On the other hand, it has been shown by repeated observations in ZES that increased fecal water and electrolyte losses cease as soon as no, or only normal, quantities of acid gastric juice are allowed to enter the intestine. This has been noted during constant gastric aspiration [7] and after total gastrectomy [19], despite persistently elevated plasma and urinary gastrin levels [24]. Moreover, the fact that the diarrhea in ZES can be cured by total gastrectomy indicates that gastric acid is an essential factor in producing diarrhea.…”

Section: Discussionmentioning

confidence: 94%

Synthetic human gastrin I and gastrin-like pentapeptide in studies of intestinal absorption in man

et al. 1982

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The effects of synthetic human gastric I (SHG I) and gastrin-like pentapeptide (PG) on jejunal water, electrolyte, and glucose absorption were studied in 11 normal subjects. The i.v. administration of graded doses of SHG I increased plasma gastrin levels similar to those after food intake and in the Zollinger-Ellison syndrome. SHG I and PG caused no significant changes in the net movement of water and solute. The findings indicate that gastrin has no direct effect on intestinal absorption in normal man, and does not account for the mechanism of diarrhea in the Zollinger-Ellison syndrome.

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“…DISCUSSION Verner and Morrison (1958) noticed particularly the refractory watery diarrhoea in their two patients, Islet tumours of the pancreas with intractable diarrnoea and were able to collect from the literature seven further patients. In five of these the diarrhoea preceded the symptoms of peptic ulcer by many years, a point noted originally by Zollinger and Ellison (1955), later by Donaldson, vom Eigen, and Dwight (1957), Maynard and Point (1958), Summerskill (1959), and Rawson et al (1960). The association of steatorrhoea with the Zollinger-Ellison syndrome has been recorded by Maynard and Point (1958), Summerskill (1959), and Rawson et al (1960).…”

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confidence: 79%

“…The absence of peptic ulceration in seven, and its late appearance in four, of the patients in Table I, certainly suggest that excessive acid secretion was not the prime cause in these 11 patients. It is true that in only a few case reports has the degree of gastric hypersecretion been measured (Zollinger and Ellison, 1955;Donaldson et al, 1957;Maynard and Point, 1958;Summerskill, 1959;Rawson et al, 1960). A further difficulty is the absence of precise analysis of the faecal discharge; we have been able to discover no report of the electrolytic composition of the faeces, and only in one patient (Rawson et al, 1960) has the faecal volume been measured.…”

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confidence: 89%