Kovaleva E V, Eremkina A K, Krupinova J A, Mirnaya S S, Kim I V, Kuznetzov N S, Andreeva E N, Karonova T L, Kryukova I V, Mudunov A M, Sleptcov I V, Melnichenko G A, Mokrysheva N G, Dedov I I
Endocrinology Research Center.
Network of Family Medical Centers No. 1.
Probl Endokrinol (Mosk). 2021 Aug 17;67(4):68-83. doi: 10.14341/probl12800.
Hypoparathyroidism is a rare disorder characterized by the absent or inappropriately decreased serum parathyroid hormone in the parathyroid glands, which is accompanied by impaired calcium-phosphorus metabolism.The main etiology of hypoparathyroidism remains damage or removal of the parathyroid glands during neck surgery. In view of the incidence of thyroid cancer, primary hyperparathyroidism and other pathologies of the neck organs, which radical treatment can lead to the parathyroid gland impairment, an increased number of patients with hypoparathyroidism is expected. Autoimmune hypoparathyroidism is the second most common form of the disease, usually occurring as part of type 1 autoimmune polyglandular syndrome. Autoimmune hypoparathyroidism usually occurs in childhood and is characterized by a severe course of the disease, especially in the case of concomitant malabsorption syndrome.Chronic hypoparathyroidism of any etiology requires lifelong multicomponent therapy, as well as careful monitoring and an individual approach to choose the optimal treatment strategy. In the absence of adequate follow-up, the risks of long-term complications significantly increase, particularly in the renal, cardiovascular systems; in the soft tissues and in the brain, it could lead to visual disturbances; pathology of the musculoskeletal system with a decreased bone remodeling and a potential risk of fractures, as well as to the neurocognitive disorders and an impaired health-related quality of life.Timely diagnosis, rational medical therapy and management strategy may reduce the risks of short-term and long-term complications, frequency of hospitalizations and disability of patients, as well as improve the prognosis.This review covers the main issues of Russian guidelines for the management of chronic hypoparathyroidism, approved in 2021, including laboratory and instrumental evaluation, treatment approaches and follow-up. This guidelines also include the recommendations for special groups of patients: with acute hypocalcemia, hypoparathyroidism during pregnancy.
甲状旁腺功能减退症是一种罕见的疾病，其特征是甲状旁腺中血清甲状旁腺激素缺乏或不适当降低，并伴有钙磷代谢受损。甲状旁腺功能减退症的主要病因仍然是颈部手术期间甲状旁腺的损伤或切除。鉴于甲状腺癌、原发性甲状旁腺功能亢进症和颈部器官的其他病变的发病率，根治性治疗可能导致甲状旁腺受损，预计甲状旁腺功能减退症患者数量会增加。自身免疫性甲状旁腺功能减退症是该疾病的第二常见形式，通常作为1型自身免疫性多腺体综合征的一部分出现。自身免疫性甲状旁腺功能减退症通常发生在儿童期，其疾病过程严重，尤其是在伴有吸收不良综合征的情况下。任何病因引起的慢性甲状旁腺功能减退症都需要终身多组分治疗，以及仔细的监测和个体化方法来选择最佳治疗策略。在缺乏充分随访的情况下，长期并发症的风险会显著增加，特别是在肾脏、心血管系统；在软组织和大脑中，可能导致视觉障碍；肌肉骨骼系统病变，骨重塑减少，有骨折的潜在风险，以及神经认知障碍和健康相关生活质量受损。及时诊断、合理的药物治疗和管理策略可以降低短期和长期并发症的风险、患者住院频率和残疾率，并改善预后。本综述涵盖了2021年批准的俄罗斯慢性甲状旁腺功能减退症管理指南的主要问题，包括实验室和仪器评估、治疗方法和随访。本指南还包括针对特殊患者群体的建议：急性低钙血症患者、妊娠期甲状旁腺功能减退症患者。
