Bone Metabolism Program, Division of Endocrinology, Department of Medicine, Lenox Hill Hospital and Zucker School of Medicine at Hofstra/Northwell.
International Education and Research, Emeritus, Division of Endocrinology, Department of Medicine, Columbia University College of Physicians & Surgeons, New York, New York, USA.
Curr Opin Rheumatol. 2019 Jul;31(4):381-387. doi: 10.1097/BOR.0000000000000618.
Hypoparathyroidism is a rare endocrine disorder characterized by low or insufficient parathyroid hormone (PTH) concentrations leading to hypocalcemia, hyperphosphatemia, and markedly reduced bone turnover. Despite being a rare disease, hypoparathyroidism has a profound impact on affected patients.
Recent epidemiologic surveys demonstrate a prevalence of between 5.3 and 40/100 000, confirming the orphan status of this disease. There is a female predominance, and anterior neck surgery is the most common etiology. Recent studies have better elucidated the chronic manifestations of the disease, impacting quality of life and multiple organ systems including the renal, cardiovascular, and skeletal systems. There are now data on longer term use of parathyroid hormone (PTH) and PTH analogs.
This review focuses on recent contributions to the literature on the prevalence and epidemiology of the disease, risk of chronic manifestations, and treatment with PTH(1-34) and rhPTH(1-84). Further research is needed to determine the pathophysiology of complications in hypoparathyroidism and whether interventions can decrease future risk of these complications. In addition, further data are needed with regards to more physiologic dosing regimens and long-term treatment with PTH and PTH analogs.
甲状旁腺功能减退症是一种罕见的内分泌疾病,其特征为甲状旁腺激素(PTH)浓度降低或不足,导致低钙血症、高磷血症和明显的骨转换减少。尽管甲状旁腺功能减退症是一种罕见疾病,但它对受影响的患者有深远的影响。
最近的流行病学调查显示,患病率在 5.3 至 40/100000 之间,证实了这种疾病的孤儿地位。女性患病率较高,颈部前侧手术是最常见的病因。最近的研究更清楚地阐明了疾病的慢性表现,影响生活质量和多个器官系统,包括肾脏、心血管和骨骼系统。现在有关于甲状旁腺激素(PTH)和 PTH 类似物长期使用的数据。
本综述重点介绍了甲状旁腺功能减退症的患病率和流行病学、慢性表现的风险以及 PTH(1-34)和 rhPTH(1-84)治疗的最新文献贡献。需要进一步研究以确定甲状旁腺功能减退症并发症的发病机制,以及干预是否可以降低这些并发症的未来风险。此外,还需要更多关于更生理性给药方案和 PTH 及 PTH 类似物长期治疗的数据。