Calcium infusion suggests a "set-point" abnormality of parathyroid gland function in familial benign hypercalcemia and more complex disturbances in primary hyperparathyroidism.
Calcium infusion suggests a "set-point" abnormality of parathyroid gland function in familial benign hypercalcemia and more complex disturbances in primary hyperparathyroidism.
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DOI：
10.1210/jcem.76.3.8445032
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发表时间：
1993-03
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影响因子：
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通讯作者：
S. Khosla;P. Ebeling;A. Firek;M. F. Burritt;P. Kao;H. Heath
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文献类型：
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作者：
S. Khosla;P. Ebeling;A. Firek;M. F. Burritt;P. Kao;H. Heath
PTH 显然在维持家族性良性高钙血症（FBH 或家族性低钙尿性低钙血症）的高钙血症中发挥着作用。为了更好地定义 FBH 和原发性甲状旁腺功能亢进症（1 度 HPT）中的甲状旁腺功能异常，我们使用了完整 PTH 的双位点免疫化学发光测定法来检查正常个体和 FBH 或 1 度 HPT 患者的 PTH 抑制情况。 12 名正常受试者、11 名 FBH 受试者和 7 名 1 度 HPT 受试者静脉注射钙 (Ca)，并频繁采样离子化 Ca 和完整 PTH。在正常和 FBH 受试者中，血浆 PTH 水平对静脉注射 Ca 的反应基本上相同地下降。在 1 度 HPT 组中，PTH 通常无法被抑制。然而，1 度 HPT 患者存在一系列反应性，肿瘤质量与 PTH 非抑制程度之间存在显着相关性 (r = 0.87，P = 0.01)。对三组血浆 PTH 和离子化 Ca 值之间关系的分析表明，FBH 曲线向右移动，但斜率没有差异，这与 FBH 中简单“设定点”误差的概念一致。相比之下，1度HPT的曲线不仅向右移动，而且斜率也与正常值不同（正常，-8.92；1度HPT，-3.92，P = 0.04）。因此，我们认为 FBH 中的甲状旁腺功能异常代表一个简单的设定点误差，而 1 度 HPT 中的缺陷由设定点误差与不同程度的 Ca 不可抑制的 PTH 分泌（可能与肿瘤质量有关）组成。
PTH clearly plays a role in maintaining the hypercalcemia of familial benign hypercalcemia (FBH or familial hypocalciuric hypocalcemia). To better define the abnormalities of parathyroid function in FBH and primary hyperparathyroidism (1 degree HPT), we used a two-site immunochemiluminometric assay for intact PTH to examine PTH suppressibility in normal individuals and patients having FBH or 1 degree HPT. Twelve normal, 11 FBH, and 7 1 degree HPT subjects were given calcium (Ca) iv with frequent sampling for ionized Ca and intact PTH. In normal and FBH subjects, plasma PTH levels decreased essentially identically in response to iv Ca. In the 1 degree HPT group, PTH was not normally suppressible. However, there was a spectrum of responsiveness in 1 degree HPT patients, with a significant correlation between tumor mass and degree of PTH nonsuppressibility (r = 0.87, P = 0.01). Analysis of the relationship between plasma PTH and ionized Ca values in the three groups demonstrated a shift to the right in the FBH curve, with no difference of slope, consistent with the notion of a simple "set-point" error in FBH. In contrast, the curve in 1 degree HPT was not only shifted to the right but also differed from normal in slope (normal, -8.92; 1 degree HPT, -3.92, P = 0.04). Thus, we propose that the parathyroid functional abnormality in FBH represents a simple set-point error, whereas the defect in 1 degree HPT consists of a set-point error combined with varying degrees of Ca nonsuppressible PTH secretion that may be related to tumor mass.