Screening for the Multiple Endocrine Neoplasia Syndrome Type I: A Study of 11 Kindreds in the Netherlands

doi:10.1001/archinte.1989.00390120069014

You are seeing this message because your Web browser does not support basic Web standards. Find out more about why this message is appearing and what you can do to make your experience on this site better.

Vol. 149 No. 12, December 1989

Online Only
	•	Online First Table of Contents

ORIGINAL INVESTIGATIONS

•	Online Features

This Article
•	References
•	Full text PDF
•	Reply to article
•	Send to a friend
•	Save in My Folder
•	Save to citation manager
•	Permissions

Citing Articles
•	Citation map
•	Citing articles on HighWire
•	Citing articles on Web of Science (85)
•	Contact me when this article is cited

Related Content
•	Similar articles in this journal

Social Bookmarking
	What's this?

Screening for the Multiple Endocrine Neoplasia Syndrome Type I

A Study of 11 Kindreds in the Netherlands

Hans F. A. Vasen, MD; Cornelis B. H. W. Lamers, MD; Cees J. M. Lips, MD

Arch Intern Med. 1989;149(12):2717-2722.

Abstract

• Since 1974, a total of 11 families with the multipleendocrine neoplasia syndrome type I (MEN-I), including 52 patients,were identified. Fifteen of these 52 patients died of MEN-I—relatedcomplications (mean age, 44 years), most of them in the periodbefore screening was started. In 11 of the 15 patients, deathwas caused by complicated peptic ulcer disease, in 2 by metastasisof an endocrine pancreatic tumor, and in 2 by renal failuredue to hyperparathyroidism. Family screening led to the diagnosisof 43 new endocrine lesions: 21 cases of hyperparathyroidism,16 endocrine pancreatic tumors, and 6 pituitary tumors. Hyperparathyroidismeither was the first manifestation of MEN-I or was diagnosedsimultaneously with the other components of the syndrome in44 (94%) of the 47 patients who underwent full evaluation.

(Arch Intern Med. 1989;149:2717-2722)

Author Affiliations

From the Foundation for the Detection of Hereditary Tumors, Utrecht, the Netherlands (Dr Vasen); Department of Gastroenterology, University Hospital, Leiden, the Netherlands (Dr Lamers); and Department of Internal Medicine, University Hospital, Utrecht (Dr Lips).

Footnotes

Accepted for publication July 17, 1989.

Reprint requests to Foundation for the Detection of HereditaryTumors, PO Box 12.009, 3501 AA Utrecht, the Netherlands (DrVasen).

Add to CiteULike CiteULike Add to Connotea Connotea Add to Delicious Delicious Add to Digg Digg Facebook Add to Reddit Reddit Add to Technorati Technorati Twitter What's this?

THIS ARTICLE HAS BEEN CITED BY OTHER ARTICLES

Multiple endocrine neoplasia type 1 in Brazil: MEN1 founding mutation, clinical features, and bone mineral density profile
Lourenco et al.
Eur J Endocrinol 2008;159:259-274.
ABSTRACT | FULL TEXT

Pancreatic endocrine neoplasms: epidemiology and prognosis of pancreatic endocrine tumors
Halfdanarson et al.
Endocr Relat Cancer 2008;15:409-427.
ABSTRACT | FULL TEXT

Multiple endocrine neoplasia type 1 in Northern Finland; clinical features and genotype-phenotype correlation
Vierimaa et al.
Eur J Endocrinol 2007;157:285-294.
ABSTRACT | FULL TEXT

Hereditary Cancer Predisposition Syndromes
Garber and Offit
JCO 2005;23:276-292.
ABSTRACT | FULL TEXT

Multiple Endocrine Neoplasia Type 1 Variant with Frequent Prolactinoma and Rare Gastrinoma
Hao et al.
J. Clin. Endocrinol. Metab. 2004;89:3776-3784.
ABSTRACT | FULL TEXT

Familial Isolated Hyperparathyroidism Is Rarely Caused by Germline Mutation in HRPT2, the Gene for the Hyperparathyroidism-Jaw Tumor Syndrome
Simonds et al.
J. Clin. Endocrinol. Metab. 2004;89:96-102.
ABSTRACT | FULL TEXT

Mutational and gross deletion study of the MEN1 gene and correlation with clinical features in Spanish patients
Cebrian et al.
J. Med. Genet. 2003;40:e72-72.
FULL TEXT

Prospective Study of the Natural History of Gastrinoma in Patients with MEN1: Definition of an Aggressive and a Nonaggressive Form
Gibril et al.
J. Clin. Endocrinol. Metab. 2001;86:5282-5293.
ABSTRACT | FULL TEXT

Pituitary Macroadenoma in a 5-Year-Old: An Early Expression of Multiple Endocrine Neoplasia Type 1
Stratakis et al.
J. Clin. Endocrinol. Metab. 2000;85:4776-4780.
ABSTRACT | FULL TEXT

Four Cases of Mucosal Neuroma Syndrome: Multiple Endocrine Neoplasm 2B or Not 2B?
Gordon et al.
J. Clin. Endocrinol. Metab. 1998;83:17-20.
FULL TEXT

Multiple Endocrine Neoplasm, Type 1: Gastrinomas, Pancreatic Neoplasms, Microcarcinoids, the Zollinger-Ellison Syndrome, Lymph Nodes, and Hepatic Metastases
Shepherd et al.
Arch Surg 1993;128:1133-1142.
ABSTRACT

Cause of Death in Multiple Endocrine Neoplasia Type 1
Wilkinson et al.
Arch Surg 1993;128:683-690.
ABSTRACT

The Natural History of Multiple Endocrine Neoplasia Type 1: Highly Uncommon or Highly Unrecognized?
Shepherd
Arch Surg 1991;126:935-952.
ABSTRACT

| | |