Screening for the multiple endocrine neoplasia syndrome type I. A study of 11 kindreds in The Netherlands.
Screening for the multiple endocrine neoplasia syndrome type I. A study of 11 kindreds in The Netherlands.
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筛查 I 型多发性内分泌肿瘤综合征。荷兰 11 个家族的研究。
DOI：
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发表时间：
1989
期刊：
影响因子：
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通讯作者：
Lips Cj
中科院分区：
文献类型：
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作者：
H. F. Vasen;C. B. Lamers;Lips Cj
·自1974年以来，共有11个家族患有多发性内分泌瘤综合征I型（MEN-I），包括52名患者。这52例患者中有15例死于MEN-I相关并发症（平均年龄44岁），其中大多数发生在筛选开始前。在15例患者中，11例死于复杂性消化性溃疡，2例死于胰腺内分泌肿瘤转移，2例死于甲状旁腺功能亢进导致的肾功能衰竭。家庭筛查导致43个新的内分泌病变的诊断：甲状旁腺功能亢进症21例，胰腺内分泌肿瘤16例，垂体肿瘤6例。甲状旁腺功能亢进是MEN-I的第一个表现，或者在47例接受全面评估的患者中有44例（94%）与综合征的其他成分同时诊断。（Arch Intern Med）1989;149：2717-2722）
• Since 1974, a total of 11 families with the multiple endocrine neoplasia syndrome type I (MEN-I), including 52 patients, were identified. Fifteen of these 52 patients died of MEN-I—related complications (mean age, 44 years), most of them in the period before screening was started. In 11 of the 15 patients, death was caused by complicated peptic ulcer disease, in 2 by metastasis of an endocrine pancreatic tumor, and in 2 by renal failure due to hyperparathyroidism. Family screening led to the diagnosis of 43 new endocrine lesions: 21 cases of hyperparathyroidism, 16 endocrine pancreatic tumors, and 6 pituitary tumors. Hyperparathyroidism either was the first manifestation of MEN-I or was diagnosed simultaneously with the other components of the syndrome in 44 (94%) of the 47 patients who underwent full evaluation. ( Arch Intern Med . 1989;149:2717-2722)