假性甲状旁腺功能减退症1型骨代谢特点

Characteristics of bone metabolism in pseudohypoparathyroidism

假性甲状旁腺功能减退症(pseudohypoparathyroidism,PHP)是由于甲状旁腺激素(parathyroid hormone,PTH)抵抗导致,以低血钙、高血磷、高PTH血症为主要生化特征,其骨骼可能呈现纤维囊性骨炎表现,也可出现骨密度升高甚至骨硬化,这可能是由于骨组织仍保留对PTH的反应性。因此,应用维生素D制剂和钙剂治疗时,除了纠正低钙血症,还需要控制合理的PTH水平以避免长期高PTH对骨组织的不利影响。不同分子亚型的PHP1型患者骨骼表现、治疗方案及目标是否有差异,目前研究数量有限,仍需进一步探讨。

Pseudohypoparathyroidism(PHP)is caused by parathyroid hormone(PTH)resistance,with hypocalcemia,hyperphosphatemia and elevated serum PTH levels as main characteristics.The skeletal manifestations of PHP varied from fibrocystic osteitis,to increased bone density and even bone sclerosis.Some studies have found that the bone tissue of PHP still retains the responsiveness to PTH.The treatment of PHP with calcium and vitamin D should target to both normalized serum calcium and optimal PTH levels to avoid the negative impact of long-term elevated PTH on bone tissue.Due to the limited studies,further studies are needed to explore the differences in skeletal changes,treatment options,and goals in different subtypes of PHP patients.