Fitzpatrick L A
Endocrine Research Unit, Mayo Clinic, Rochester, Minnesota.
Endocrinol Metab Clin North Am. 1989 Sep;18(3):741-52.
Multiple endocrine neoplasia includes disorders with hyperfunction of two or more endocrine tissues. In MEN type 1, hyperfunction of the parathyroid glands causing hypercalcemia is the most common clinical presentation. In vitro, suppression of parathyroid tissue by calcium is similar, but the set-point of hyperplastic tissue is shifted as compared with normal. The gene for MEN-1 has been localized to chromosome 11 and is linked to the basic fibroblast growth factor gene. Parathyroidectomy results in a high failure rate with recurrent hyperparathyroidism or autonomous graft function in autotransplanted tissue. Family screening is recommended once every 5 years in first-degree relatives. The approach to hyperparathyroidism in MEN-2 (2A) must be individualized during surgery for medullary thyroid carcinoma. Hyperparathyroidism in MEN-3 (2B) is often associated with normal serum calcium and may not require intervention.
多发性内分泌腺瘤病包括两个或更多内分泌组织功能亢进的疾病。在1型多发性内分泌腺瘤病中,甲状旁腺功能亢进导致高钙血症是最常见的临床表现。在体外,钙对甲状旁腺组织的抑制作用相似,但与正常组织相比,增生组织的设定点发生了偏移。1型多发性内分泌腺瘤病的基因已定位到11号染色体,并与碱性成纤维细胞生长因子基因相关。甲状旁腺切除术后复发率较高,会出现复发性甲状旁腺功能亢进或自体移植组织的自主移植功能。建议对一级亲属每5年进行一次家族筛查。在甲状腺髓样癌手术期间,2型多发性内分泌腺瘤病(2A)中甲状旁腺功能亢进的治疗方法必须个体化。3型多发性内分泌腺瘤病(2B)中的甲状旁腺功能亢进通常与血清钙正常有关,可能不需要干预。