Endocrine Unit, Massachusetts General Hospital and Harvard Medical School, 50 Blossom St., Boston, Massachusetts 02114, USA.
Division of Endocrinology, College of Physicians and Surgeons, Columbia University Medical Center, New York, New York, USA.
Nat Rev Dis Primers. 2017 Aug 31;3:17055. doi: 10.1038/nrdp.2017.55.
Hypoparathyroidism is a disease characterized by inadequately low circulating concentrations of parathyroid hormone (PTH) resulting in low calcium levels and increased phosphate levels in the blood. Symptoms of the disease result from increased neuromuscular irritability caused by hypocalcaemia and include tingling, muscle cramps and seizures. The most common cause of the disease is inadvertent removal of, or injury to, the parathyroid glands during neck surgery, followed by genetic, idiopathic and autoimmune aetiologies. Conventional treatment includes activated vitamin D and/or calcium supplements, but this treatment does not fully replace the functions of PTH and can lead to short-term problems (such as hypocalcaemia, hypercalcaemia and increased urinary calcium excretion) and long-term complications (which include nephrocalcinosis, kidney stones and brain calcifications). PTH replacement has emerged as a new treatment option. Clinical trials using human PTH(1-34) and PTH(1-84) showed that this treatment was safe and effective in studies lasting up to 6 years. Recombinant human PTH(1-84) has been approved in the United States and Europe for the management of hypoparathyroidism; however, its effect on long-term complications is still being evaluated. Clinical practice guidelines, which describe the consensus of experts in the field, have been published and recognize the need for more research to optimize care. In this Primer, we summarize current knowledge of the prevalence, pathophysiology, clinical presentation and management of hypoparathyroidism.
甲状旁腺功能减退症是一种疾病，其特征为甲状旁腺激素（PTH）循环浓度不足，导致血钙水平降低和血磷酸盐水平升高。该疾病的症状源于低钙血症引起的神经肌肉兴奋性增加，包括刺痛、肌肉痉挛和癫痫发作。该疾病最常见的病因是颈部手术中甲状旁腺的意外切除或损伤，其次是遗传、特发性和自身免疫病因。常规治疗包括活性维生素 D 和/或钙补充剂，但这种治疗不能完全替代 PTH 的功能，并且可能导致短期问题（如低钙血症、高钙血症和尿钙排泄增加）和长期并发症（包括肾钙质沉着症、肾结石和脑钙化）。PTH 替代已成为一种新的治疗选择。使用人 PTH(1-34)和 PTH(1-84)的临床试验表明，这种治疗在长达 6 年的研究中是安全有效的。重组人 PTH(1-84)已在美国和欧洲获得批准用于治疗甲状旁腺功能减退症；然而，其对长期并发症的影响仍在评估中。描述该领域专家共识的临床实践指南已经发布，并认识到需要更多的研究来优化治疗。在这篇综述中，我们总结了甲状旁腺功能减退症的患病率、病理生理学、临床表现和管理的现有知识。
