Quan T T, Li Y P, Wang O, Jiang Y, Xia W B, Li M, Cheng X Q, Meng X W, Xing X P
Key Laboratory of Endocrinology of National Health And Family Planning Commission, Department of Endocrinology, Peking Union Medical College Hospital, Peking Union Medical College, Chinese Academy of Medical Sciences, Beijing 100730, China.
Zhonghua Nei Ke Za Zhi. 2017 Jan 1;56(1):19-23. doi: 10.3760/cma.j.issn.0578-1426.2017.01.006.
To study the clinical characteristics of primary hypoparathyroidism in adults. The clinical data of 200 cases with adult-onset primary hypoparathyroidism in Peking Union Medical College Hospital during December 1987 to December 2015 were collected and analyzed retrospectively. Among them, 128 cases were followed up for a median period of 3 years. The major manifestations at their first visits were tetany and numbness in the distal extremities(81.5%, 163/200 and 62.0%, 124/200). Thirty-two percent of the cases (62 cases) had history of seizures, and 60.9%(98/161) and 74.4%(96/129) of them were with intracerebral calcifications and cataracts, respectively.Most of subjects(155/200)had more than one year delay in diagnosis. Hypercalciuria occurred in 67.2%(86/128) of the cases during the follow-up. No significant differences in the clinical characteristics and biochemical markers between the hypercalciuria subjects and the non-hypercalciuria subjects. Renal nephrocalcinosis or stones were found in 6.5%(5/77) of the cases, and kidney function decreased in 6.6%(6/91) of the patients. Kidney function was negatively associated with age and duration of disease. The predominant manifestations of primary hypoparathyroidism in adults included tetany and numbness in the distal extremities and seizures. It is often misdiagnosed. Calcium supplement combined with vitamin D or its metabolites effectively relieve clinical symptoms and signs. The serum and urinary calcium levels should be monitored frequently to reduce renal complications.
研究成人原发性甲状旁腺功能减退症的临床特征。回顾性收集并分析1987年12月至2015年12月在北京协和医院就诊的200例成人起病的原发性甲状旁腺功能减退症患者的临床资料。其中128例患者进行了随访，中位随访时间为3年。患者初诊时的主要表现为手足搐搦和肢体远端麻木（分别为81.5%，163/200；62.0%，124/200）。32%的患者（62例）有癫痫发作史，其中60.9%（98/161）有脑内钙化，74.4%（96/129）有白内障。大多数患者（155/200）诊断延迟超过1年。随访期间67.2%（86/128）的患者出现高钙尿症。高钙尿症患者与非高钙尿症患者在临床特征和生化指标上无显著差异。6.5%（5/77）的患者发现有肾钙质沉着症或肾结石，6.6%（6/91）的患者肾功能下降。肾功能与年龄和病程呈负相关。成人原发性甲状旁腺功能减退症的主要表现包括手足搐搦、肢体远端麻木和癫痫发作。该病常被误诊。补充钙剂联合维生素D或其代谢产物可有效缓解临床症状和体征。应定期监测血清和尿钙水平以减少肾脏并发症。
