Shepherd J J, Teh B T, Parameswaran V, David R
Department of Surgery, University of Tasmania, Hobart, Australia.
Henry Ford Hosp Med J. 1992;40(3-4):186-90.
In the largest reported family of patients with multiple endocrine neoplasia type 1 (MEN 1), hyperparathyroidism was expressed at first screening in 33 patients by elevation of ionized calcium (IC) (30 cases) or parathyroid hormone (three cases) without elevation of albumin-corrected total calcium (ACTC). Three of these 33 patients have shown a progressive rise in IC and later an elevation of ACTC. However, the age distribution suggests that in others the level of IC may remain stable at a minimally elevated level throughout life with ACTC remaining normal except for transient rises at the times of intercurrent illness or surgical operation. Even when ACTC is normal preoperatively, patients with an elevation of IC require radical subtotal parathyroidectomy or total parathyroidectomy and forearm implantation to restore IC to a normal level. Institutions that rely on ACTC as a screening test for hyperparathyroidism in MEN 1 will miss the diagnosis in nearly half of patients under the age of 30. The greatest deficiency in using ACTC occurs in the follow-up of patients who have undergone parathyroidectomy for MEN 1. Only three of 11 recurrences were evidenced by this measurement.
在已报道的最大的1型多发性内分泌腺瘤病（MEN 1）患者家系中，33例患者在首次筛查时因离子钙（IC）升高（30例）或甲状旁腺激素升高（3例）而出现甲状旁腺功能亢进，而白蛋白校正总钙（ACTC）未升高。这33例患者中有3例IC呈进行性升高，随后ACTC升高。然而，年龄分布表明，在其他患者中，IC水平可能在整个生命过程中保持在轻度升高的稳定水平，ACTC除了在并发疾病或手术时短暂升高外，其余时间均保持正常。即使术前ACTC正常，IC升高的患者也需要进行根治性甲状旁腺次全切除术或甲状旁腺全切除术及前臂植入术，以使IC恢复到正常水平。依赖ACTC作为MEN 1甲状旁腺功能亢进筛查试验的机构，将漏诊近一半30岁以下的患者。使用ACTC的最大不足发生在对因MEN 1接受甲状旁腺切除术的患者的随访中。11例复发患者中只有3例通过该测量得以证实。
