CHAPTER 36 – Multiple Endocrine Neoplasia Type 2

作者: ROBERT F. GAGEL

DOI: 10.1016/B978-012098651-4/50038-9

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摘要: It has been difficult to study the natural history of hyperparathyroidism in Multiple Endocrine Neoplasia type 2 (MEN-2) because its low incidence (5–20%) and frequent late onset clinical features hyperparathyroidism. Nonetheless, several interesting points have emerged: (1) In some kindreds occurs with greater frequency than others and, (2) observation made families, is lack gene carriers who were thyroidectomized for early C cell abnormalities, despite follow-up periods 15–25 years. Hypercalcemia nephrolithiasis are most common presenting manifestations found MEN-2 patients. Pheochromocytoma may rarely cause hypercalcemia, possibly related production parathyroid hormone-related protein, should be excluded prior surgical exploration. Hyperplasia multiple glands abnormality associated MEN-2, although many reports emphasize occurrence adenomas individuals present fully developed syndrome, generally after age 35

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