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[Ⅰ型假性甲状旁腺功能减退症中骨骼对甲状旁腺激素的敏感性。6例报告]

[Sensitivity of bone to parathyroid hormone in type I pseudohypoparathyroidism. 6 cases].

作者信息

Wemeau J L, Duquesnoy B, Grimbert I, Hubaud P, Hardouin P, Lemaitre G, Fossati P, Decoulx M

出版信息

Ann Med Interne (Paris). 1986;137(3):220-5.

PMID:3767189
Abstract

The sensitivity of bone to parathormone in pseudoparathyroidism is not well known. Six patients with Type I pseudohypoparathyroidism (4 with Albright's osteodystrophy) had increased alkaline phosphatase levels (5 patients) and radiological signs of periosteal resorption in the hand in one case. All patients had histological signs of increased surfaces of resorption and periosteocytic lacunae, increased osteoid surfaces and relative osteoid volume with no change of the index of osteoid thickness. These changes are identical to those observed in hyperparathyroidism which leads on to the discussion of the role of the increased parathormone secretion induced by the lack of calcium on the remodeling of bone. Our six cases show that there is no bone resistance to parathormone. The diversity of bone changes in hyperparathyroidism, similar to that of primary hyperparathyroidism, is without doubt dependent on the degree of renal insensitivity to PTH through the inactivation of vitamin D.

摘要

假性甲状旁腺功能减退症中骨骼对甲状旁腺激素的敏感性尚不清楚。6例I型假性甲状旁腺功能减退症患者(4例患有奥尔布赖特骨营养不良),碱性磷酸酶水平升高(5例),1例手部有骨膜吸收的放射学征象。所有患者均有吸收表面和骨膜细胞陷窝增加、类骨质表面增加和相对类骨质体积增加的组织学征象,而类骨质厚度指数无变化。这些变化与甲状旁腺功能亢进症中观察到的变化相同,这引发了关于钙缺乏诱导的甲状旁腺激素分泌增加在骨重塑中的作用的讨论。我们的6例病例表明,骨骼对甲状旁腺激素不存在抵抗。甲状旁腺功能亢进症中骨骼变化的多样性,与原发性甲状旁腺功能亢进症相似,无疑取决于肾脏通过维生素D失活对甲状旁腺激素不敏感的程度。

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