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Multiple Endocrine Neoplasia, Type I: Association With Marfanoid Habitus, Optic Atrophy, and Other Abnormalities

Multiple Endocrine Neoplasia, Type I: Association With Marfanoid Habitus, Optic Atrophy, and... Abstract • A patient had a parathyroid adenoma and prolactin-secreting pituitary tumor, suggestive of the multiple endocrine neoplasia (MEN) I syndrome. The presence of a marfanoid habitus—found more typically in MEN III syndrome—as well as mitral valve prolapse, mental retardation, and bilateral optic atrophy suggests a new variant of the MEN syndrome, possibly representing widespread dysplasia of endocrine and other tissues. (Arch Intern Med 1983;143:2315-2316) References 1. Ballard HS, Frame B, Hartsock RJ: Familial multiple endocrine edenoma—peptic ulcer complex. Medicine 1964;43:481-516.Crossref 2. Steiner AL, Goodman AD, Powers SR: Study of a kindred with pheochromocytoma, medullary thyroid carcinoma, hyperparathyroidism and Cushing's disease: Multiple endocrine neoplasia, type 2. Medicine 1968;47:371-409.Crossref 3. Khairi MRA, Dexter RN, Burzynski NJ, et al: Mucosal neuroma, pheochromocytoma and medullary thyroid carcinoma: Multiple endocrine neoplasia type 3. Medicine 1975;54:89-112.Crossref 4. Levine JH, Sagel J, Rosebrock G, et al: Prolactin-secreting adenoma as part of the multiple endocrine neoplasia—type I (MEN-I) syndrome. Cancer 1979;43:2492-2496.Crossref 5. Prosser PR, Karam JH, Townsend JJ, et al: Prolactin-secreting pituitary adenomas in multiple endocrine adenomatosis, type I. Ann Intern Med 1979;91:41-44.Crossref 6. Farid NR, Buehler S, Russell NA, et al: Prolactinomas in familial multiple endocrine neoplasia syndrome type I. Am J Med 1980;69:874-880.Crossref 7. Dyck PJ, Carney JA, Sizemore GW, et al: Multiple endocrine neoplasia, type 2b: Phenotype recognition; neurological features and their pathological basis. Ann Neurol 1979;6:302-314.Crossref 8. DeSchryver-Kecskemeti K, Clouse RE, Goldstein MN, et al: Intestinal ganglioneuromatosis: A manifestation of overproduction of nerve growth factor? N Engl J Med 1983;308:635-639.Crossref 9. Newell FW: Ophthalmology, Principles and Concepts . St Louis, CV Mosby Co, 1978, p 71. http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png Archives of Internal Medicine American Medical Association

Multiple Endocrine Neoplasia, Type I: Association With Marfanoid Habitus, Optic Atrophy, and Other Abnormalities

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References (10)

Publisher
American Medical Association
Copyright
Copyright © 1983 American Medical Association. All Rights Reserved.
ISSN
0003-9926
eISSN
1538-3679
DOI
10.1001/archinte.1983.00350120109023
Publisher site
See Article on Publisher Site

Abstract

Abstract • A patient had a parathyroid adenoma and prolactin-secreting pituitary tumor, suggestive of the multiple endocrine neoplasia (MEN) I syndrome. The presence of a marfanoid habitus—found more typically in MEN III syndrome—as well as mitral valve prolapse, mental retardation, and bilateral optic atrophy suggests a new variant of the MEN syndrome, possibly representing widespread dysplasia of endocrine and other tissues. (Arch Intern Med 1983;143:2315-2316) References 1. Ballard HS, Frame B, Hartsock RJ: Familial multiple endocrine edenoma—peptic ulcer complex. Medicine 1964;43:481-516.Crossref 2. Steiner AL, Goodman AD, Powers SR: Study of a kindred with pheochromocytoma, medullary thyroid carcinoma, hyperparathyroidism and Cushing's disease: Multiple endocrine neoplasia, type 2. Medicine 1968;47:371-409.Crossref 3. Khairi MRA, Dexter RN, Burzynski NJ, et al: Mucosal neuroma, pheochromocytoma and medullary thyroid carcinoma: Multiple endocrine neoplasia type 3. Medicine 1975;54:89-112.Crossref 4. Levine JH, Sagel J, Rosebrock G, et al: Prolactin-secreting adenoma as part of the multiple endocrine neoplasia—type I (MEN-I) syndrome. Cancer 1979;43:2492-2496.Crossref 5. Prosser PR, Karam JH, Townsend JJ, et al: Prolactin-secreting pituitary adenomas in multiple endocrine adenomatosis, type I. Ann Intern Med 1979;91:41-44.Crossref 6. Farid NR, Buehler S, Russell NA, et al: Prolactinomas in familial multiple endocrine neoplasia syndrome type I. Am J Med 1980;69:874-880.Crossref 7. Dyck PJ, Carney JA, Sizemore GW, et al: Multiple endocrine neoplasia, type 2b: Phenotype recognition; neurological features and their pathological basis. Ann Neurol 1979;6:302-314.Crossref 8. DeSchryver-Kecskemeti K, Clouse RE, Goldstein MN, et al: Intestinal ganglioneuromatosis: A manifestation of overproduction of nerve growth factor? N Engl J Med 1983;308:635-639.Crossref 9. Newell FW: Ophthalmology, Principles and Concepts . St Louis, CV Mosby Co, 1978, p 71.

Journal

Archives of Internal MedicineAmerican Medical Association

Published: Dec 1, 1983

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