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假性甲状旁腺功能减退症中甲状旁腺激素抵抗的机制。

Mechanisms of parathyroid hormone resistance in pseudohypoparathyroidism.

作者信息

Hosking D J, Kerr D

机构信息

Metabolism/Diabetes, City Hospital, Nottingham, U.K.

出版信息

Clin Sci (Lond). 1988 Jun;74(6):561-6. doi: 10.1042/cs0740561.

DOI:10.1042/cs0740561
PMID:2840234
Abstract

The problem with PHP is that it is almost certainly a heterogeneous condition encompassing a number of defects within the PTH receptor-adenylate cyclase complex. The clinical manifestations of PTH resistance are amplified and extended by a number of associated abnormalities. These include a low intracellular calcium concentration, secondary hyperparathyroidism, reduced production of 1,25-(OH)2D and perhaps the secretion of an abnormal PTH peptide. Almost every aspect of PTH function seems involved and this makes PHP a fascinating condition in which to test current views of the role of PTH in the integration of the calcium homoeostatic system.

摘要

假性甲状旁腺功能减退症的问题在于,它几乎可以肯定是一种异质性疾病,涵盖了甲状旁腺激素(PTH)受体 - 腺苷酸环化酶复合物中的多种缺陷。多种相关异常会加剧和扩展PTH抵抗的临床表现。这些异常包括细胞内钙浓度降低、继发性甲状旁腺功能亢进、1,25 - 二羟维生素D[1,25-(OH)2D]生成减少,以及可能分泌异常的PTH肽。PTH功能的几乎每个方面似乎都涉及其中,这使得假性甲状旁腺功能减退症成为一个极具吸引力的疾病,可用于检验当前关于PTH在钙稳态系统整合中作用的观点。

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