Bürgi H, Burckhardt P, Gardiol D
Schweiz Med Wochenschr. 1982 Dec 11;112(50):1820-5.
In a 22-year old man plasma calcitonin was normal in the basal state but elevated to 2.6 ng/ml after stimulation with calcium and pentagastrin. Two other family members had had bilateral pheochromocytoma accompanied by medullary carcinoma of the thyroid in one case and by adenomas of the parathyroid glands in the other. The literature of Sipple's syndrome, the value of calcitonin assay and the grounds for screening in affected families are reviewed. Surgery (total thyroidectomy) confirmed the clinical suspicion of C-cell hyperplasia and medullary microcarcinoma of the thyroid gland. Hyperplasia of the parathyroid glands was also found.
一名22岁男性,基础状态下血浆降钙素正常,但经钙和五肽胃泌素刺激后升高至2.6 ng/ml。另外两名家族成员,其中一人患有双侧嗜铬细胞瘤并伴有甲状腺髓样癌,另一人患有双侧嗜铬细胞瘤并伴有甲状旁腺腺瘤。本文回顾了西普尔综合征的文献、降钙素测定的价值以及在受累家族中进行筛查的依据。手术(甲状腺全切除术)证实了临床怀疑的甲状腺C细胞增生和甲状腺髓样微小癌。还发现了甲状旁腺增生。