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Harrison's Principles of Internal Medicine, 18e
| Part 16. Endocrinology and Metabolism > Section 1. Endocrinology > |
Chapter 351. Disorders Affecting Multiple Endocrine Systems
Sections: Neoplastic Disorders Affecting Multiple Endocrine Organs, Immunologic Syndromes Affecting Multiple Endocrine Organs, Further Readings.
Topics Discussed: endocrinologic disorders; multiple endocrine neoplasia.
Excerpt:"Multiple endocrine neoplasia syndrome is defined as a disorder with neoplasms in two or more different hormonal tissues in several members of a family. Several distinct genetic disorders predispose to endocrine gland neoplasia and cause hormone excess syndromes (Table 351-1). DNA-based genetic testing is available for these disorders, but effective management requires an understanding of endocrine neoplasia and the range of clinical features that may be manifested in an individual patient.MEN 1, or Wermer's syndrome, is inherited as an autosomal dominant trait. This syndrome is characterized by neoplasia of the parathyroid glands, enteropancreatic tumors, anterior pituitary adenomas, and other neuroendocrine tumors with variable penetrance (Table 351-1). Although rare, MEN 1 is the most common multiple endocrine neoplasia syndrome, with an estimated prevalence of 2–20 per 100,000 in the general population. It is caused by inactivating mutations of the tumor-suppressor gene MEN1 located at chromosome 11q13. The MEN1 gene codes for a nuclear protein called Menin. Menin interacts with JunD, suppressing JunD-dependent transcriptional activation. It is unclear how this accounts for Menin growth regulatory activity, since JunD is associated..."
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