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挪威甲状腺髓样癌。43例患者的300名亲属的血清降钙素。

Medullary carcinoma of the thyroid gland in Norway. Serum calcitonin in 300 relatives of 43 patients.

作者信息

Normann T, Gautvik K M

出版信息

Ann Chir Gynaecol. 1977;66(4):187-94.

PMID:907311
Abstract

Measurements of serum calcitonin (iCT) were performed in 300 relatives of 43 Norwegian patients with medullary thyroid carcinoma (MCT). Prior to the screening, familial occurrence of the disease was known in only one of the families. Persistant hypercalcitoninemia (serum levels greater than 0.50 ng/ml) was demonstrated in 2 healthy persons from this family and in 3 first degree relatives of 2 patients with apparently sporadic disease. Additional 7 relatives of 5 other "sporadic" cases showed elevation of serum iCT on one occasion, while later controls revealed normal values. Twenty persons had high normal serum levels (0.35 ng/ml less than or equal to iCT less than 0.50 ng/ml). The significance of transitory hypercalcitoninemia and high normal values are at the present unknown, but these persons will have further tests. Stimulation tests for iCT secretion were found to be of limited value in distinguishing between normal and pathological serum iCT. All individuals found to have chronic hypercalcitoninemia belonged to families in which the probands showed one or several of the following "high risk" factors: A positive history of thyroid disease, early age of onset, bilateral presence of the thyroid tumour, association with other endocrine or peripheral neurogenic tumours, and marphanoid habitus. The iCT screening was however negative in the relatives of the two probands revealing 4 and 5 of these factors. Since neither the family history nor the presence or absence of "high risk" factors distinguished between sporadic and familial cases, it is concluded that serum iCT measurements should be carried out in primary relatives of all patients with MCT.

摘要

对43名挪威甲状腺髓样癌(MCT)患者的300名亲属进行了血清降钙素(iCT)检测。在筛查之前,仅在其中一个家族中已知该疾病的家族性发病情况。在这个家族的2名健康人和2名明显散发疾病患者的3名一级亲属中发现了持续性高降钙素血症(血清水平大于0.50 ng/ml)。另外5例“散发”病例的7名亲属在某一时刻血清iCT升高,但后来的检测显示值正常。20人血清水平处于高正常范围(0.35 ng/ml≤iCT<0.50 ng/ml)。目前尚不清楚短暂性高降钙素血症和高正常水平的意义,但这些人将接受进一步检查。发现iCT分泌刺激试验在区分正常和病理性血清iCT方面价值有限。所有被发现患有慢性高降钙素血症的个体都属于先证者表现出以下一种或几种“高风险”因素的家族:甲状腺疾病阳性病史、发病年龄早、甲状腺肿瘤双侧存在、与其他内分泌或周围神经源性肿瘤相关以及类马方体型。然而,在显示出4种和5种这些因素的两名先证者的亲属中,iCT筛查结果为阴性。由于家族史以及“高风险”因素的有无均无法区分散发性和家族性病例,因此得出结论,应对所有MCT患者的一级亲属进行血清iCT检测。

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