Get 20M+ Full-Text Papers For Less Than $1.50/day. Start a 14-Day Trial for You or Your Team.

Learn More →

Nesidioblastosis in Familial Endocrine Adenomatosis

Nesidioblastosis in Familial Endocrine Adenomatosis Symptomatic and asymptomatic members of a family with multiple endocrine adenomatosis (MEA) (pancreatic islet, parathyroid, and adrenal cortex) were studied. Of the six subjects studied representing three generations, all had excessive secretion of insulin, three showed excessive plasma glucagon responses to oral administration of dextrose, and four had elevated plasma gastrin levels. This evidence of islet hyperactivity suggests the following: (1) A genetic defect resulted in proliferation of the primordial cell of the islands of Langerhans, a condition termed nesidioblastosis. (2) Neoplastic islet cells have the capacity to secrete insulin, glucagon, and/or gastrinlike peptides. (3) The variable involvement of other endocrine glands in the familial MEA syndrome may be secondary to chronic hypersecretion of one or more of the islet hormones. http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png JAMA American Medical Association

Nesidioblastosis in Familial Endocrine Adenomatosis

Download PDF
4 pages

Loading next page...
 
/lp/american-medical-association/nesidioblastosis-in-familial-endocrine-adenomatosis-oCvqpBv3bH

References (15)

Publisher
American Medical Association
Copyright
Copyright © 1969 American Medical Association. All Rights Reserved. Applicable FARS/DFARS Restrictions Apply to Government Use.
ISSN
0098-7484
eISSN
1538-3598
DOI
10.1001/jama.1969.03150220095010
Publisher site
See Article on Publisher Site

Abstract

Symptomatic and asymptomatic members of a family with multiple endocrine adenomatosis (MEA) (pancreatic islet, parathyroid, and adrenal cortex) were studied. Of the six subjects studied representing three generations, all had excessive secretion of insulin, three showed excessive plasma glucagon responses to oral administration of dextrose, and four had elevated plasma gastrin levels. This evidence of islet hyperactivity suggests the following: (1) A genetic defect resulted in proliferation of the primordial cell of the islands of Langerhans, a condition termed nesidioblastosis. (2) Neoplastic islet cells have the capacity to secrete insulin, glucagon, and/or gastrinlike peptides. (3) The variable involvement of other endocrine glands in the familial MEA syndrome may be secondary to chronic hypersecretion of one or more of the islet hormones.

Journal

JAMA – American Medical Association

Published: Mar 3, 1969

There are no references for this article.