Multiple Endocrine Neoplasia, Type I (MEN I)

Éberlé, F.; R, Grün

doi:10.1007/978-3-642-67954-4_3

Cited by 29 publications

(81 citation statements)

References 202 publications

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“…36 This false-negative result is particularly noteworthy because hypercalcemia is typically the presenting manifestation of MEN1 and is often used as a screen for asymptomatic adults in affected families. 37 In conclusion, abundant evidence establishes the importance of ionized calcium in several pathologic conditions. Although its direct measurement remains costly and technically challenging, the algorithms to predict ionized calcium from total calcium have not proved accurate, especially in patients with complex illness.…”

mentioning

confidence: 90%

When Is It Appropriate to Order an Ionized Calcium?

Calvi

Bushinsky

2008

Journal of the American Society of Nephrology

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mentioning

confidence: 90%

When Is It Appropriate to Order an Ionized Calcium?

Calvi

Bushinsky

2008

Journal of the American Society of Nephrology

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“…This is an important consideration in all young Liver metastases were all histologically documented either during the initial evaluation or if possible liver metastases on imaging developed. 2 All patients underwent yearly follow-up imaging studies (CT scan, MRI, ultrasound). 3 Neuroendocrine tumour refers to the presence of a pancreatic endocrine tumour of any type in the indicated location.…”

Section: The Effect Of Hypercalcaemia On Expression Of the Zes Acid mentioning

confidence: 99%

Management of the Zollinger–Ellison syndrome in patients with multiple endocrine neoplasia type 1

Jensen¹

1998

Journal of Internal Medicine

175

169

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Zollinger-Ellison syndrome (ZES) is the most common symptomatic pancreatic endocrine tumour in patients with MEN-1. Besides the treatment of the usual endocrinopathies seen in patients with MEN-1, the treatment of the ZES requires attention be paid to controlling the gastric acid hypersecretion, to dealing with the gastrinomas per se which are malignant in 18-60% of cases, and to the diagnosis and treatment of gastric carcinoid tumours, that are increasingly seen in these patients. In this article the current management of each of the areas is reviewed and what is known or uncertain discussed, based on our studies at the NIH and data from others. Data from 231 patients including 45 with MEN-1 and 186 without MEN-1 is contrasted in this report. Gastric acid hypersecretion has been controlled in all patients medically with MEN-1 and ZES at the NIH for up to 22 years. The current drugs of choice are H ϩ -K ϩ ATPase inhibitors and twice a day dosing is recommended. Periods of parenteral drug therapy (surgery, etc.) and pregnancy require important modifications. The appropriate surgical therapy of the gastrinoma is controversial. Eighty per cent of patients have a duodenal gastrinoma and 20-30% have a pancreatic tumour. Recent studies suggest gastrinoma enucleation combined with duodenotomy rarely results in cure. Aggressive surgery (Whipple resection) can result in cure of gastrinoma but effect on survival is unclear.There are important differences in gastrinoma location, extent, and percentage with aggressive disease in patients with or without MEN-1, which are discussed. Confusion has occurred because of lack of information on the natural history of the gastrinoma compared to the other pancreatic endocrine tumours that occur in MEN-1 and survival data from patients with and without MEN-1 is contrasted. The occurrence of gastric carcinoids in patients with and without MEN-1 with ZES is contrasted and the areas of certainty and disagreement reviewed.

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“…Pancreatic islet cell involvement was documented in at least 80% of cases with MEN 1 reported [4]. Incomplete expression of MEN type 1 syndrome is unlikely in our patients due to the lack of other endocrine disease in subjects older than 50 years of age, suggesting the absence of the autosomal MEN type 1 gene which has a high penetrance by the age of 20 years [10].…”

Section: Discussionmentioning

confidence: 94%

“…The principal features of this condition are hyperparathyroidism, tumors of the pancreatic islet cells and adenomas of the anterior pituitary. Eberle and Grim [4] found evidence of pituitary involvement in 54% of 122 cases reviewed as MEN type 1, and 13 of these patients were acromegalic. Nevertheless, familial cases of isolated acromegaly are extremely rare, and there have been only three reliable reports [5][6][7] on such isolated familial acromegaly since a reliable biochemical means of assessing GH was established [8].…”

Section: Discussionmentioning

confidence: 99%

“…The absence of hypercalcaemia or pancreatic islet dysfunction in our acromegalic subjects may constitute strong evidence against a diagnosis of MEN 1. Hyperparathyroidism is the most common feature of MEN 1, and clinical or biochemical evidence of hyperparathyroidism was present in 97% of 122 cases with MEN 1 [4]. Benson et al [9] identified 55 patients with MEN 1 in 16 families, all of whom demonstrated hyperparathyroidism at the time of diagnosis.…”

Section: Discussionmentioning

confidence: 99%

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