成年起病的原发性甲状旁腺功能减退症200例临床分析  

Clinical characteristics of adult-onset primary hypoparathyroidism： a retrospective analysis of 200 cases

作　　者：全婷婷[1] 李悦芃[1] 王鸥[1] 姜艳[1] 夏维波[1] 李梅[1] 程歆琦[1] 孟迅吾[1] 邢小平[1] 

机构地区：[1]中国医学科学院北京协和医学院北京协和医院内分泌科国家卫生和计划生育委员会内分泌重点实验室,100730

出　　处：《中华内科杂志》2017年第1期19-23,共5页Chinese Journal of Internal Medicine

基　　金：国家自然科学基金（81270873,81100559）;国家临床重点专科建设项目（WBYZ2011-873）

摘　　要：目的探讨成年起病的原发性甲状旁腺功能减退症（甲旁减）临床特点。方法回顾性分析1987年12月-2015年12月于北京协和医院内分泌科就诊的成年起病的200例原发性甲旁减患者临床特点，在规律随诊的128例患者中分析泌尿系统并发症比例及其影响因素。结果患者初诊时以手足搐搦（81．5％，163／200）、手足麻木（62．0％，124／200）症状多见，62例（32．0％）有癫痫发作史。颅内钙化及白内障发生比例分别为60．9％（98／161）和74．4％（96／129）。从发病到确诊时间长于1年者占77．5％（155／200）。所有患者均给予口服钙剂、维生素D及其衍生物治疗，部分低镁血症患者同时给予补镁治疗。随诊期间67．2％（86／128）患者出现高钙尿症，除尿钙外，其初诊及随诊时的临床特点、生化检查与正常尿钙者相比差异无统计学意义。6．5％（5／77）发生肾脏钙化／泌尿系统结石，6．6％（6／91）发生肾功能下降，回归分析提示肾功能下降与年龄、病程相关。结论成年起病的原发性甲旁减以手足麻木、手足搐搦及癫痫发作多见，易被漏诊或误诊，钙剂和维生素D及其衍生物治疗可有效缓解临床症状及体征，但需密切监测血钙、尿钙、泌尿系B超，警惕泌尿系统并发症发生。Objective To study the clinical characteristics of primary hypoparathyroidism in adults. Methods 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. Results 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. Hyperealciuria 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 hypercaleiuria subjects and the non-hypercalciuria subjects. Renal nephroealcinosis 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. Conclusions 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.

关 键 词：甲状旁腺功能减退症 原发性 成年起病 泌尿系统并发症 

分 类 号：R582.2[医药卫生—内分泌]