Cimetidine in the management of synchronous crises of MEA I

Friesen,; As, Hermreck; Ce, Brackett; Rn, Schimke

doi:10.1007/bf02393111

Cited by 11 publications

(2 citation statements)

References 9 publications

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“…Literature BAO data are from 129 patients 11,14,35,[39][40][41]67,92,95,96,100,103,117,122,144,146,153,154,170,175,177,185,189,191,192,216,220,228,230,236,254,257,270,284,289,292,331,343,355,[369][370][371][372]378,401,439,443,446,474,491 ; and literature MAO data are from 55 patients 11,…”

Section: Discussionmentioning

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: Discussionmentioning

confidence: 99%

Multiple Endocrine Neoplasia Type 1 and Zollinger-Ellison Syndrome

et al. 2004

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“…If not, there are adequate medical therapies which should be instituted during the subsequent further investigation of the patient. The effects of hypergastrinemia can be controlled by the use of H2-receptor blockers [6] or proton pump blockers such as omeprazole [7]. Excess insulin production can be treated with diazoxide [8].…”

Section: Invited Commentarymentioning

confidence: 99%