Zhang Ling, Yao Li, Hua Zhan, Bian Wei-jing, Li Wen-ge
Department of Nephrology, China-Japan Friendship Hospital, Beijing 100029, China.
Zhonghua Nei Ke Za Zhi. 2011 Jul;50(7):562-7.
To evaluate the efficacy of the parathyroidectomy (PTX) in the treatment of severe secondary hyperparathyroidism (SHPT) with Sagliker syndrome (SS).
A retrospective review was undertaken among 212 SS patients underwent PTX in our hospital and with more than 3 years' follow up. The definitions of the efficacy were based on the postoperative intact parathyroid hormone level (iPTH). "Cure" showed that the iPTH was < 150 ng/L; "marked effectiveness" was 150 - 300 ng/L; "effectiveness" was 301 - 500 ng/L; "ineffectiveness" was > 500 ng/L. The status was defined as persistent SHPT if iPTH was > 150 ng/L after surgery. The status was considered as SHPT recurrence if iPTH was < 100 ng/L in the first week after surgery, and gradually increased and > 150 ng/L with the follow-up.
(1) Ten patients were involved and the average dialysis time was 142 months [male/female: 4/6; age 30 - 54 (39.3 ± 10.4) years]. All patients had severe bone and joint pain, accompanied with progressive facial increases, chicken breast, kyphosis, hip bone deformities, and body height shortening. (2) Preoperative tests: the median of iPTH 2000 (1800 - 2863) ng/L; serum calcium (2.45 ± 0.21) mmol/L, phosphorus (2.19 ± 0.51) mmol/L, alkaline phosphatase (ALP) (1189.8 ± 780.0) IU/L. Two to four enlarged parathyroid glands were confirmed by ultrasound and (99)Tc(m)-MIBI parathyroid scintigraphy. (3) Surgical procedures: local or general anesthesia for PTX. Supplement with calcium and calcitriol implemented low serum calcium after PTX. (4) FOLLOW-UP: symptoms, including bone pain, muscle weakness, skin itching, and insomnia, were significantly improved after surgery. Transient hoarseness occurred in 2 cases. The iPTHs of all patients were decreased significantly after surgery. The median of iPTH was 55.5 (10 - 967) ng/L at 1 month post PTX, and was significantly less than prior to PTX (P < 0.001). Eight patients were "cure", 1 "marked effectiveness", and 1 "ineffectiveness". Two patients were persistent SHPT, and 1 died of heart failure in the 4(th) year after PTX. The development of bone deformities was stopped and malnutrition was improved in long-time follow up. The level of iPTH 135 (28 - 390) ng/L (P < 0.001), serum calcium, phosphorus, and ALP showed normal in the third year. The SHPT recurrence was appeared in the 2(nd) and 3(rd) year in 2 out of 8 patients, respectively.
Total PTX can effectively treat SS by SHPT. It can improve prognosis for patients, such as bone pain disappearing, bone deformities stopping and malnutrition improving, etc. The level of iPTH may rise again in some patients in the future. Therefore, more attentions should be paid to monitoring.
评估甲状旁腺切除术（PTX）治疗伴有萨利克综合征（SS）的重度继发性甲状旁腺功能亢进症（SHPT）的疗效。
对我院212例行PTX且随访超过3年的SS患者进行回顾性研究。疗效定义基于术后完整甲状旁腺激素水平（iPTH）。“治愈”表明iPTH＜150 ng/L；“显效”为150 - 300 ng/L；“有效”为301 - 500 ng/L；“无效”为＞500 ng/L。若术后iPTH＞150 ng/L，则定义为持续性SHPT。若术后第一周iPTH＜100 ng/L，随后逐渐升高且随访时＞150 ng/L，则视为SHPT复发。
（1）纳入10例患者，平均透析时间为142个月[男/女：4/6；年龄30 - 54（39.3±10.4）岁]。所有患者均有严重的骨和关节疼痛，伴有进行性面部增大、鸡胸、脊柱后凸、髋骨畸形及身高缩短。（2）术前检查：iPTH中位数为2000（1800 - 2863）ng/L；血清钙（2.45±0.21）mmol/L，磷（2.19±0.51）mmol/L，碱性磷酸酶（ALP）（1189.8±780.0）IU/L。超声及（99）Tc（m）-甲氧基异丁基异腈甲状旁腺显像证实有2 - 4个甲状旁腺增大。（3）手术操作：PTX采用局部或全身麻醉。PTX后补充钙剂和骨化三醇以纠正低血钙。（4）随访：术后骨痛、肌肉无力、皮肤瘙痒及失眠等症状明显改善。2例出现短暂性声音嘶哑。所有患者术后iPTH均显著下降。PTX后1个月iPTH中位数为55.5（10 - 967）ng/L，显著低于术前（P＜0.001）。8例“治愈”，1例“显效”，1例“无效”。2例为持续性SHPT，1例在PTX后第4年死于心力衰竭。长期随访中骨畸形发展停止，营养不良改善。第3年iPTH水平为135（28 - 390）ng/L（P＜0.001），血清钙、磷及ALP均正常。8例患者中有2例分别在第2年和第3年出现SHPT复发。
全甲状旁腺切除术可有效治疗由SHPT引起的SS。可改善患者预后，如骨痛消失、骨畸形发展停止及营养不良改善等。部分患者未来iPTH水平可能再次升高。因此，应加强监测。
