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Objective To evaluate the efficacy of the parathyroidectomy (PTX) in the treatment of severe secondary hyperparathyroidism (SHPT) with Sagliker syndrome (SS). Methods 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. Results ( 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 99Tcm-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 4th 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 2nd and 3rd year in 2 out of 8 patients, respectively. Conclusions 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.