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Familial Endocrine Adenomatosis: A Family With Hyperinsulinism as the Predominant Manifestation

Familial Endocrine Adenomatosis: A Family With Hyperinsulinism as the Predominant Manifestation Abstract Introduction In 1903, Erdheim 1 first described the occurrence of multiple endocrine tumors. Since then, the recognition of a syndrome has emerged. This syndrome, referred to as familial endocrine adenomatosis, or familial polyglandular adenomatosis, is characterized by the occurrence of a tumor or tumors, not necessarily simultaneously, of more than one endocrine gland, generally the parathyroid, pituitary, or islet cells of the pancreas, in more than one member of a family. This is the report of a family of 12 in whom three members—a father, son, and daughter—had one or more endocrine tumors. The presenting complaint in all three patients was related to hyperinsulinism. Report of Cases Case 1.—This patient was an 18-year-old white male who entered another hospital on Feb 3, 1961, with the chief complaint of "passing-out spells" for six to seven years. These episodes of unconsciousness, which were transient, were frequently preceded by nausea and perspiring and References 1. Erdheim, J.: Zur normalen und pathologischen Histologie der Glandula thyreoidea, Parathyreoidea und Hypophysis , Beitr Path Anat 33:158, 1903. 2. Yalow, R. S., and Berson, S. A.: Immunoassay of Endogenous Plasma Insulin in Man , J Clin Invest 39:1157, 1960.Crossref 3. Fajans, S. S., and Floyd, J. C., Jr.: Personal communication to the authors. 4. Wermer, P.: Genetic Aspects of Adenomatosis of Endocrine Glands , Amer J Med 16:363, 1954.Crossref 5. Moldawer, M.; Nardi, G. L.; and Raker, J. W.: Concomitance of Multiple Adenomas of the Parathyroids and Pancreatic Islets With Tumor of the Pituitary: A Syndrome With Familial Incidence , Amer J Med Sci 228:190, 1954.Crossref 6. Moldawer, M.: Multiple Endocrine Tumors and the Zollinger-Ellison Syndrome in Families: One or Two Syndromes; A Report of Two New Families , Metabolism 11:153, 1962. 7. Pender, B.: Islet Cell Tumor of the Pancreas Associated With Peptic Ulceration , Lancet 1:123, 1959.Crossref 8. Underdahl, L. O.; Woolner, L. B.; and Black, B. M.: Multiple Endocrine Adenomas: Report of Eight Cases in Which Parathyroid, Pituitary and Pancreatic Islets Were Involved , J. Clin Endocr 13:20, 1953.Crossref 9. Makaresz, D.: Familial Insulinoma , Zbl Chir 86:2116, 1961. 10. Baker, R. L.: Familial Adenomatosis of Endocrine Glands: Presentation of a New Family , Northwest Med 60:905, 1961. 11. Mieher, W. C.; Hartrock, R. J.; Goekas, M. C.; Ballard, H. S.; and Frame, B.: Peptic Ulcer as a Manifestation of Familial Polyendocrine Disease , JAMA 179:854, 1962.Crossref 12. Schmid, J. R.; Labhart, A.; and Rossier, P. H.: Relationship of Multiple Endocrine Adenomas to the Syndrome of Ulcerogenic Islet Cell Adenomas (Zollinger-Ellison) , Amer J Med 31:343, 1961.Crossref 13. Zollinger, R. M., and Elliot D. W.: Pancreatic Endocrine Function and Peptic Ulceration , Gastroenterology 37:401, 1959. 14. Williams, E. D., and Celestin, L. R.: The Association of Bronchial Carcinoid and Pleuriglandular Adenomatosis , Thorax 17:120, 1962.Crossref 15. Hershfield, B. M., and Janis, R.: A Case of Acute Myocardial Infarction With Multiple Endocrine Adenomatosis and Two Primary Carcinomas , Jew Mem Hosp Bull (NY) 6-7:167, 1962. 16. Whipple, A. O.: The Surgical Therapy of Hyperinsulinism , J Int Chir 3:237, 1938. 17. Crain, E. L., Jr., and Thorn, G. W.: Functioning Pancreatic Islet Cell Adenomas , Medicine 28: 427, 1949.Crossref 18. Fajans, S. S.; Schneider, J. M.; Schteingart, D. E.; and Conn, J. W.: Diagnostic Value of Sodium Tolbutamide in Hypoglycemic States , J Clin Endocr 21:371, 1961.Crossref http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png American Journal of Diseases of Children American Medical Association

Familial Endocrine Adenomatosis: A Family With Hyperinsulinism as the Predominant Manifestation

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References (22)

Publisher
American Medical Association
Copyright
Copyright © 1963 American Medical Association. All Rights Reserved.
ISSN
0002-922X
DOI
10.1001/archpedi.1963.02080050220018
Publisher site
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Abstract

Abstract Introduction In 1903, Erdheim 1 first described the occurrence of multiple endocrine tumors. Since then, the recognition of a syndrome has emerged. This syndrome, referred to as familial endocrine adenomatosis, or familial polyglandular adenomatosis, is characterized by the occurrence of a tumor or tumors, not necessarily simultaneously, of more than one endocrine gland, generally the parathyroid, pituitary, or islet cells of the pancreas, in more than one member of a family. This is the report of a family of 12 in whom three members—a father, son, and daughter—had one or more endocrine tumors. The presenting complaint in all three patients was related to hyperinsulinism. Report of Cases Case 1.—This patient was an 18-year-old white male who entered another hospital on Feb 3, 1961, with the chief complaint of "passing-out spells" for six to seven years. These episodes of unconsciousness, which were transient, were frequently preceded by nausea and perspiring and References 1. Erdheim, J.: Zur normalen und pathologischen Histologie der Glandula thyreoidea, Parathyreoidea und Hypophysis , Beitr Path Anat 33:158, 1903. 2. Yalow, R. S., and Berson, S. A.: Immunoassay of Endogenous Plasma Insulin in Man , J Clin Invest 39:1157, 1960.Crossref 3. Fajans, S. S., and Floyd, J. C., Jr.: Personal communication to the authors. 4. Wermer, P.: Genetic Aspects of Adenomatosis of Endocrine Glands , Amer J Med 16:363, 1954.Crossref 5. Moldawer, M.; Nardi, G. L.; and Raker, J. W.: Concomitance of Multiple Adenomas of the Parathyroids and Pancreatic Islets With Tumor of the Pituitary: A Syndrome With Familial Incidence , Amer J Med Sci 228:190, 1954.Crossref 6. Moldawer, M.: Multiple Endocrine Tumors and the Zollinger-Ellison Syndrome in Families: One or Two Syndromes; A Report of Two New Families , Metabolism 11:153, 1962. 7. Pender, B.: Islet Cell Tumor of the Pancreas Associated With Peptic Ulceration , Lancet 1:123, 1959.Crossref 8. Underdahl, L. O.; Woolner, L. B.; and Black, B. M.: Multiple Endocrine Adenomas: Report of Eight Cases in Which Parathyroid, Pituitary and Pancreatic Islets Were Involved , J. Clin Endocr 13:20, 1953.Crossref 9. Makaresz, D.: Familial Insulinoma , Zbl Chir 86:2116, 1961. 10. Baker, R. L.: Familial Adenomatosis of Endocrine Glands: Presentation of a New Family , Northwest Med 60:905, 1961. 11. Mieher, W. C.; Hartrock, R. J.; Goekas, M. C.; Ballard, H. S.; and Frame, B.: Peptic Ulcer as a Manifestation of Familial Polyendocrine Disease , JAMA 179:854, 1962.Crossref 12. Schmid, J. R.; Labhart, A.; and Rossier, P. H.: Relationship of Multiple Endocrine Adenomas to the Syndrome of Ulcerogenic Islet Cell Adenomas (Zollinger-Ellison) , Amer J Med 31:343, 1961.Crossref 13. Zollinger, R. M., and Elliot D. W.: Pancreatic Endocrine Function and Peptic Ulceration , Gastroenterology 37:401, 1959. 14. Williams, E. D., and Celestin, L. R.: The Association of Bronchial Carcinoid and Pleuriglandular Adenomatosis , Thorax 17:120, 1962.Crossref 15. Hershfield, B. M., and Janis, R.: A Case of Acute Myocardial Infarction With Multiple Endocrine Adenomatosis and Two Primary Carcinomas , Jew Mem Hosp Bull (NY) 6-7:167, 1962. 16. Whipple, A. O.: The Surgical Therapy of Hyperinsulinism , J Int Chir 3:237, 1938. 17. Crain, E. L., Jr., and Thorn, G. W.: Functioning Pancreatic Islet Cell Adenomas , Medicine 28: 427, 1949.Crossref 18. Fajans, S. S.; Schneider, J. M.; Schteingart, D. E.; and Conn, J. W.: Diagnostic Value of Sodium Tolbutamide in Hypoglycemic States , J Clin Endocr 21:371, 1961.Crossref

Journal

American Journal of Diseases of Children – American Medical Association

Published: Aug 1, 1963

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