Wakasugi T, Aoyama T, Hirai J, Saga T
Department of Internal Medicine, Fukui Prefectural Hospital, Japan.
Jpn J Med. 1991 Mar-Apr;30(2):146-53. doi: 10.2169/internalmedicine1962.30.146.
The proband, a 42-year-old woman without any symptoms or hypertension, was admitted for examination of accidentally discovered bilateral adrenal masses. Physical examination disclosed bilateral nodular goiter and mild sinus tachycardia. Pheochromocytomas and medullary thyroid carcinomas were revealed by biochemical and histopathological examinations. Hypercholesterolemia and abnormal glucose metabolism returned to normal after bilateral total adrenalectomy and thyroidectomy. Screening examination showed four affected family members. Three of them were also asymptomatic and normotensive. In pheochromocytoma patients, normotension, hypercholesterolemia, and impaired glucose metabolism might be signs of excess secretion of epinephrine rather than norepinephrine.
先证者为一名42岁女性，无任何症状或高血压，因偶然发现双侧肾上腺肿块入院检查。体格检查发现双侧结节性甲状腺肿和轻度窦性心动过速。生化和组织病理学检查显示患有嗜铬细胞瘤和甲状腺髓样癌。双侧肾上腺全切术和甲状腺切除术后，高胆固醇血症和糖代谢异常恢复正常。筛查检查发现4名家族成员患病。其中3人也无症状且血压正常。在嗜铬细胞瘤患者中，血压正常、高胆固醇血症和糖代谢受损可能是肾上腺素而非去甲肾上腺素分泌过多的体征。
