妊娠合并原发性甲状旁腺功能亢进症五例临床分析

Pregnancy with primary hyperparathyroidism： analysis of five cases

目的探讨妊娠合并原发性甲状旁腺功能亢进症的临床表现、诊断及治疗策略。方法2005年1月至2014年12月，北京协和医院共收治妊娠合并原发性甲状旁腺功能亢进症患者5例，回顾性分析其临床表现、治疗策略、母体并发症及母儿转归。结果5例患者中位年龄32（29～41）岁，妊娠前诊断1例，妊娠期诊断3例，产后诊断1例。临床表现以恶心、呕吐、食欲减退等非特异性表现为主，此外尿频和夜尿增多1例，意识不清、急性胰腺炎和子痫相关表现1例。母体并发症最多见为肾结石、肾积水（3例），骨质疏松、贫血、低钾血症（2例）；严重并发症包括高血钙危象2例，合并急性胰腺炎、子痫、溶血肝酶升高和血小板减少综合征、弥散性血管内凝血、脑梗死、双胎之一胎死宫内1例。中位血清钙水平3．70（2．78～4．50）mmol／L；中位血清甲状旁腺激素水平294（151～634）pg／ml。甲状旁腺超声检查均为阳性，4例行甲状旁腺核素显像检查均阳性。1例未特殊治疗，妊娠结局良好，但新生儿出现甲状旁腺功能减退；2例于妊娠中期行手术治疗，1例妊娠结局良好，1例因放弃行中期引产术；2例于产后进行手术治疗，其中1例妊娠结局良好，另1例双胎分别胎死宫内和新生儿死亡。接受手术的4例患者，术后病理均为单发甲状旁腺腺瘤，术后血清钙及甲状旁腺激素水平下降满意；其中1例患者术后继发甲状旁腺功能减退，对症治疗后好转。结论妊娠合并原发性甲状旁腺功能亢进症临床表现不特异，但可以引起严重的母儿并发症，尽早诊断、合理治疗可以得到较好的妊娠结局，手术是安全且有效的治疗手段。

Objective To study the clinical presentations, diagnosis and managements of primary hyperparathyroidism （pHPT） in pregnancy. Methods A total of five cases of pHPT in pregnancy were enrolled from January 2005 to December 2014 in Peking Union Medical College Hospital. Their clinical presentations, managements, maternal-fetal complications and pregnancy outcomes were retrospectively analyzed. Results The median age was 32 （29, 41） years. Of the five cases, three were diagnosed in the second trimester, one was before pregnancy and one was after delivery. Most of the clinical symptoms were nonspecific to pHPT, such as nausea, vomiting and loss of appetite. Frequent urination and nocturia occurred in one; unconsciousness and manifestations of acute pancreatitis and eclampsia relevant symptoms were complained of by one. The common maternal complications were nephrolithiasis and hydronephrosis （3/5）, osteoporosis （2/5）, anemia （2/5） and kaliopenia （2/5）, while the severe complications were hypercalcemic crisis （2/5）, acute pancreatitis （1/5）, eclampsia （1/5）, HELLP （hemolysis, elevated liver enzymes and low platelets） syndrome （1/5）, disseminated intravascular coagulation （DIC） （1/5）, cerebral infarction （1/5） and intrauterine fetal death of one twin （1/5）. The median level of calcium in serum samples was 3.70 （2.78, 4.50） mmol/L; the median level of parathyroid hormone （PTH） in serum samples was 294 （151,634） pg/ml. All of the five cases were positive for parathyroid ultrasonography. Four cases received parathyroid radionuclide imaging and had positive results. One asymptomatic patient received no specific treatment, whereas the neonate presented with hypocalcemia after birth. Two cases received surgical resections in the second trimester; one of them had a live birth without fetal complication, while the other had induced abortion. Two cases received postpartum surgery; one asymptomatic patient had a live birth without fetal complication, while the other with twin pregnancy suffered stillbirths （one intrauterine fetal death and one neonatal death）. Pathologic diagnosis were solitary parathyroid adenomas in four cases who received surgery. Their operations were effective except that one case, which was improved after treatment, was complicated with secondary hypoparathyroidism. Coaclusions The symptoms of pHPT in pregnancy are often nonspecific, but it can cause matemal and fetal morbidity and mortality. Early diagnosis of pHPT, followed by appropriate managements, has been shown to significantly reduce the complications. Surgical management should be a safe and effective choice.