甲状旁腺全切除术治疗10例Sagliker综合征疗效评估

目的 评估甲状旁腺全切除术治疗重症继发性甲状旁腺功能亢进症(SHPT)致Sagliker综合征(SS)的疗效.方法 回顾性分析在我院因SHPT接受甲状旁腺切除术的212例患者中随访3年以上的SS病例.甲状旁腺全切除术疗效判定:术后全段甲状旁腺激素(iPTH)＜150 ng/L为治愈;150～300 ng/L为显效;301～500 ng/L为有效;＞500 ng/L为无效.术后iPTH＞150 ng/L定义为持续性SHPT.术后1周内iPTH＜100 ng/L,以后随访中逐渐上升＞150 ng/L定义为SHPT复发.结果 (1)入选的10例患者中,男4例,女6例,年龄30～54(39.3±10.4)岁.平均透析龄142个月,所有患者都有严重骨关节疼痛,伴进行性面部增大、鸡胸、驼背、髋部骨骼畸形,身高缩短.(2)术前检查:iPTH中位数2000(1800～2863)ng/L;血钙(2.45±0.21)mmol/L,血磷(2.19±0.51)mmol/L,碱性磷酸酶(1189.8±780.0)IU/L.10例患者颈部超声和99Tcm-甲氧基异丁基异腈(MIBI)扫描均证实有增大的甲状旁腺2～4枚.(3)局麻或伞麻下甲状旁腺全切除术.术后结合血钙水平补充钙剂和骨化三醇.(4)术后随访:术后骨痛、肌无力、皮肤瘙痒、失眠、燥热症状明显改善.全部患者术后有低血钙,2例发生一过性声音嘶哑.所有患者术后iPTH显著下降,术后1个月iPTH中位数55.5(10～967)ng/L,较术前显著下降(P＜0.001),其中疗效判定为治愈8例,持续性SHPT 2例(显效1例,无效1例),其中1例于术后第4年死于心力衰竭.长期随访骨骼畸形停止发展,营养不良得到改善,第3年iPTH中位数135(28～390)ng/L(P＜0.001),血钙、血磷和碱性磷酸酶也在达标范围.2例分别于第2年、第3年SHPT复发.结论 甲状旁腺全切除术可以有效治疗SS,改善患者预后,如骨痛消失、骨骼畸形发展停止、改善营养不良.长期随访部分患者iPTH有再升高可能,应该重视监测.

Abstract：

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.     

原发性甲状旁腺机能亢进症并发病态窦房结综合征一例

患者,女性,50岁。入院前一年体检发现血糖升高,拟诊为糖尿病．进行饮食治疗,尿糖控制基本正常。入院前一个月患者出现心悸症状伴腰背酸痛,检查骨密度显示骨质疏松,给予利维爱及钙剂治疗后症状无缓解。C肽释放试验检查提示为糖尿病。EKG示窦性心动过缓,频发室性早搏三联率,拟诊为糖尿病并病态窦房结综合征收治入院。体检：一般情况好,心率48次／min,余无阳性征。 实验室检查：(1)Holter全程窦性停搏710次,窦性停搏时间最长达4．85 s,大于4 s共2次。(2)阿托品试验阴性。(3)血液生化检查多次提示高钙低磷(均略微超过正常上下限)。(4)肾小管磷回吸收率(TRP)下降(0．638)。(5)磷廓清率(CP)增加(34．87 ml／min)。(6)氯磷比值(Cl／P)增加(40．5)。(7)颈部CT扫描提示双侧甲状腺未见异常,左侧甲状腺侧后方可见一类圆形肿块,直径1．5 cm×1．2 cm,边缘光滑,平扫CT值68 HU,增强后CT值105．8 HU。(8)彩超显示脂肪肝、右肾结石。临床诊断：(1)原发性甲状旁腺机能亢进症;(2)病态窦房结综合征;(3)糖尿病。     

老年原发性甲状旁腺机能亢进症七例临床分析

原发性甲状旁腺机能亢进症 (甲旁亢 )是甲状旁腺分泌过多甲状旁腺激素引起的临床症状群 ,发病年龄通常在 2 0～ 5 0岁 ,有骨痛、泌尿系结石、纤维囊性骨炎等典型临床表现。无症状或症状不典型者亦不少见 ,尤其是老年患者。临床资料1.一般资料 :老年不典型甲旁亢患者 7例 ,年龄 60～ 82岁 ,男性 2例 ,女性 5例。无任何临床表现 2例 ,仅在查体时发现血钙增高。其余 5例的主要症状为乏力 2例 ,腰痛 1例 ,双下肢肌肉疼痛 1例 ,关节痛 1例 ,烦渴、多饮、多尿 1例。合并结节性甲状腺肿 4例。2 .实验室检查 :血钙 (3 .0 4± 0 .16)ｍｍｏｌ/Ｌ ,血…     

完全腹腔镜治疗巨大甲状旁腺腺瘤伴甲状旁腺功能亢进症一例

正邓欣,黄华平,张迪,赖建勤,龚瑾     

丹麦人甲状旁腺机能亢进和随后的癌症风险

甲状旁腺机能亢进(HPT)是一种由于甲状旁腺激素生成过多导致高钙血症的疾病。研究显示此病有上升趋势,并与内分泌和其它恶性肿瘤有关。本研究旨在研究HPT与随后发生癌症的风险的可能因果关系。     

甲状旁腺全切除术并改良自体移植术治疗尿毒症继发甲状旁腺功能亢进的疗效观察

目的探讨甲状旁腺全切除术并自体前臂移植5颗甲状旁腺组织颗粒(1 mm×1 mm×1 mm)(TPTX+AT)治疗尿毒症继发甲状旁腺功能亢进症(SHPT)的疗效和可行性。方法选择该院2016-01~2017-12的尿毒症SHPT患者,行甲状旁腺全切除术并自体前臂移植5颗甲状旁腺组织颗粒,观察患者术后甲状旁腺激素(i PTH)和血钙等生化指标变化情况、症状缓解情况、平均住院时间、并发症及复发情况。结果共纳入13例患者进行了TPTX+AT手术治疗。全部患者无术中喉返神经损伤,术后第1天i PTH均降至正常水平,术后1周、3个月、6个月、12个月的i PTH水平较术前均显著降低(P0.05),且均在正常值范围内,无复发病例。骨痛、皮肤瘙痒等症状在术后1周内均缓解或消失,术后骨骼变形、身高缩短等症状均改善或未继续进展。结论 TPTX+AT安全有效可行,保留了甲状旁腺功能的同时控制了术后复发情况,可能是治疗SHPT的更优术式。     

假性甲状旁腺功能减退伴亢进症一例报告

患者男,14岁,1990年2月19日入院。该患者2年前起无明显诱因出现阵发性胸闷、心悸伴恶心、呕吐。多次心电图示不完全性右束支传导阻滞及Q-T间期延长。近半年来常有发作性晕厥,发作多在运动或情绪激动时,历时约数秒钟至数分钟不等,伴脉弱、心动过速(>250次/分)。脑电图示癫痫样改变。     

甲状旁腺功能亢进症合并重症急性胰腺炎1例报告

急性胰腺炎(acute pancreatitis)是常见的急腹症之一,是多种病因导致胰酶在胰腺内被激活后引起胰腺组织自身消化、水肿、出血甚至坏死的炎症反应。临床以急性上腹痛、恶心、呕吐、发热和血胰酶增高等为特点。其不仅是胰     

甲状旁腺腺瘤切除术对甲状旁腺机能亢进性骨病疗效的随访研究

目的评价甲状旁腺腺瘤切除术（PTX）对原发性甲状旁腺机能亢进症（PHPT）所致骨病的疗效。方法回顾分析32例PHPT患者PTX术后0.5、1 a的临床表现、骨结构和骨密度（BMD）恢复情况。其中12例患者完成6 a随访,观察骨骼修复情况。结果所有患者半年随访时临床症状均消失,各项生化指标正常,BMD显示骨量增加,X线片骨皮质增厚、骨痂形成。骨折手术治疗的患者1 a内达到骨性愈合,未再出现骨折。12例患者PTX术后6 aBMD显示骨量增加,但未达到正常水平,仍存在骨量减少。结论 PTX虽能明显改善患者骨病,但骨病恢复可能需要很长时间,PHPT患者PTX术后应延长随访时间,监测BMD变化,采取必要的干预措施,促进患者及早康复。     

Successful treatment of SAPHO syndrome with adalimumab: a case report

SAPHO syndrome is a disorder involving the skin, bone and joints. The underlying causes of SAPHO are poorly understood, and treatment is, therefore, directed towards the individual symptoms. However, many patients are refractory to treatment, and new treatment options are needed. Herein, we describe a 28-year-old patient with SAPHO syndrome and palmoplantar pustulosis seen at our hospital. Treatment was initiated with non-steroidal anti-inflammatory drugs, but clinical improvement was poor. The addition of sulfasalazine and oral alendronate also failed to alleviate symptoms. We subsequently commenced treatment with adalimumab 40 mg every 15 days and suspended bisphosphonates. Following 4 weeks’ treatment with adalimumab, there was clear articular improvement and disappearance of palmoplantar pustulous lesions. Nocturnal inflammatory lumbar pain and global disease assessment were also improved. To our knowledge, this is the first report on the use of adalimumab for SAPHO. More studies are required to confirm our findings.     

骨髓增生异常综合征转纯红系白血病一例

纯红系白血病是一种少见类型的急性白血病,老年发病多见,临床进展较快,中位生存期短,预后差。本研究中,我们分析1例骨髓增生异常综合征(MDS)转化纯红系白血病临床表现、治疗经过及临床诊断,并结合2016年世界卫生组织对造血和淋巴组织肿瘤分类关于红白血病的修订进行文献复习。     

血浆置换成功治疗毒蕈中毒性肝病1例

患者男性，47岁，无慢性肝炎病史，无长期大量饮酒史，近期无服用药物史。2005年11月13日与其他4人进食野蘑菇，10h后均出现呕吐、腹泻等胃肠道中毒症状。其中2人次日出现尿黄，并逐渐出现皮肤、巩膜黄染。第4天收治入当地医院治疗，诊断为急性胃肠炎、黄疸性肝炎。肝功能检查：总胆红素（TBil）289μmol／L，直接胆红素（DBil）211．9μmol／L，丙氨酸氨基转移酶（ALT）449U／L，天冬氨酸氨基转移酶（AST）200U／L。电解质和肾功能均正常。经输液治疗，胃肠道症状好转出院。第6日出现头晕、嗜睡，并逐渐出现意识障碍（肝性脑病）。2005年11月20日收治入我科。     

Successful treatment of rubral tremor by high-dose trihexyphenidyl: a case report

A 24-year-old male suffered from acute-onset right-sided hemiparesis, dysarthria, and ophthalmoplegia in February 2001. Brain magnetic resonance imaging revealed a cavernous angioma with hemorrhage over the left thalamus. Moreover, some rhythmic, coarse, low-frequency (2-3 Hz) oscillation over the right wrist and elbow was noted 1 month later. Action tremor was more predominant than resting tremor. Rubral tremor was diagnosed on the basis of the clinical presentation and tremography analysis. Rubral tremor is not unusual, and pharmacotherapy is nearly always ineffective in clinical practice. Deep brain stimulation, thalamotomy, and pallidotomy are all considered effective according to recent research. However, they are either very expensive or invasive, and involve surgical risks. In our patient, we tried valproate, clonazepam, and verapamil one after another, but all in vain. Finally, titration of trihexyphenidyl provided significant benefit. The tremor was successfully controlled by a single high daily dose of trihexyphenidyl (38 mg) without severe or uncomfortable side effects. Here, we report a case of successful monotherapy of rubral tremor with high-dose trihexyphenidyl.     

Hyperparathyroidism-jaw tumor syndrome: A case report

We report the clinical and genetic findings in a 23-year-old woman with hyperparathyroidism-jaw tumor syndrome (HPT-JT). The patient had a family history of primary hyperparathyroidism (PHPT) and uterine fibroma in her mother. The patient presented muscle weakness. The diagnosis of PHPT was confirmed by an elevated parathyroid hormone level above 1450 pg/ml with hypercalcemia and hypercalciuria. X-ray radiographies showed a radiolucent lesion in the right body of the mandible. Bilateral neck exploration was performed. An inferior right parathyroidectomy, a left thyroid lobectomy with isthmectomy and thymectomy were carried out. Histopathological examination of the specimen showed a diffuse hyperplasia of the parathyroid principal cells. The association of PHPT with a right jaw tumor and uterine fibroma suggested the diagnosis of HPT-JT syndrome. Mutation screening of HRPT2 gene was carried out and identified a germline mutation, consisting in a base deletion in exon 1, 85delG, inducing a frameshift. The diagnosis of HPT-JT syndrome is clinically important because of its hereditary component and its high risk of parathyroid malignancy, making a genetic inquiry necessary.     

高龄功能正常甲状腺病综合征1例

笔者成功诊治1例85岁男性高龄突发昏迷的功能正常甲状腺病综合征（ESS）患者。探讨ESS在高龄患者突发昏迷中的作用以及ESS的发病机制、甲状腺素对脑功能改善的意义。排除甲状腺功能异常及导致昏迷的其他相关因素，在进行基础治疗的同时，应用甲状腺素进行干预治疗。患者意识恢复正常，跟踪观察2年，没有发现服用甲状腺素导致的不良反应。我们认为，高龄患者因脑供血减少、脑功能容易受到各种基础病的影响，更易合并ESS，有选择地采取适度的甲状腺素干预治疗对疾病的转归有益。     

成人Still’s病合并急性重症肝损伤和噬血综合征1例报告

正1病例资料患者女性,36岁,因反复皮疹、发热、淋巴结肿大4年,黄疸4 d于2011年10月29日入院。2007年9月开始出现发热,体温38~40℃,伴全身散在红色皮疹、干咳、呕吐、乏力、食欲不振,颈部、腋下和腹股沟多处淋巴结肿大,持续10余天后自行好转,每月发作1次,多次化验血常规白细胞、中性粒细胞增高,颈部淋巴结活组织检查示:淋巴滤泡反应性增生,非甾体     

Successful treatment of pancreatic schwannoma by enucleation: A case report

Rationale:Pancreatic schwannomas are extremely rare and are difficult to diagnose preoperatively. Over the past 50 years, only 96 cases of pancreatic schwannoma have been reported in English literature. Herein, we report a case of pancreatic schwannoma treated with enucleation.Patient concerns:A 66-year-old woman visited a local hospital due to ventosities. Ultrasonography and computed tomography revealed a pancreatic mass. She visited our hospital for further diagnosis and treatment.Diagnosis and interventions:Magnetic resonance imaging revealed a tumor in the pancreatic body, and a solid pseudopapillary tumor was considered preoperatively. During the surgery, a pancreatic mass was found growing in the pancreatic body and tail. A successful tumor enucleation was performed. The mass was 7 × 6 × 3 cm in size with a thin capsule. Pathological examination revealed that the tumor was mainly composed of spindle-shaped cells with a palisading arrangement and no atypia. Both hypercellular and hypocellular areas were visible. Immunohistochemical staining showed that protein S-100 was strongly positive. The tumor was diagnosed as a benign schwannoma originating from the pancreatic body and tail.Outcomes:Postoperatively, the patient showed good recovery. During the 24-month follow-up period, the patient remained well and free of complications.Lessons:Pancreatic schwannomas are extremely rare and difficult to diagnose using imaging examinations. Enucleation is a safe and efficacious treatment for exophytic pancreatic schwannomas.     

老年甲状旁腺功能异常诊治特点

老年甲状旁腺功能异常主要见于原发和继发性甲状旁腺功能亢进症(甲旁亢)。老年人中原发甲旁亢以女性患病为多,乏力和骨质疏松为常见症状。注意血钙的筛查,>26 mmol/L者检测甲状旁腺激素(PTH)有助于及早发现患者。无手术禁忌、能定位的选择手术治疗能更多获益。老年人继发性甲旁亢主要见于慢性终末期肾脏功能不全需血液透析或腹膜透析的患者,注意检测PTH水平、早期给予适量钙制剂和较大剂量骨化三醇有助于维持血钙和控制PTH水平。