肿瘤源性骨软化症的临床特征

目的分析肿瘤源性骨软化症(tumor-induced osteomalacia,TIO)患者的临床特点、诊断、病理及手术后情况,以提高临床对TIO的认识。方法收集并整理2008年1月至12月于解放军总医院就诊的14例肿瘤源性骨软化症患者的病史、一般情况、临床表现、辅助检查、病理及术后情况。结果患者平均病程为(3. 0±3. 8)年,临床症状以骨痛(14/14,100%)和乏力(6/14,42. 9%)最常见,部分患者有病理性骨折,在确诊前均有误诊病史。生化特点以低血磷(0. 43±0. 12) mmol/L、高尿磷(13. 71±5. 32) mmol/L 24 h、血碱性磷酸酶升高(309. 30±146. 41) U/L和正常血钙(2. 19±0. 13 mmol/L)为主。定位诊断中,99Tcm-奥曲肽SPECT/CT扫描和68Ga-DOTA-TATE PET/CT阳性率均较高(100%),并经相应部位的CT、MRI或超声检查证实。肿瘤分布在全身不同部位的软组织(4/14,28. 6%)或骨组织(10/14,71. 4%)中。手术切除肿瘤后,血磷平均恢复时间为(6. 4±2. 3) d。1例因病灶难以完全切除,术后未缓解。2例患者术后出现复发。结论TIO起病隐匿,症状不具有特异性,容易被误诊。99Tcm-奥曲肽SPECT/CT扫描或68Ga-DOTATATE PET/CT检查对定位诊断效率较高。手术治疗可以治愈TIO,但远期仍可能复发,需要定期随访。     

肿瘤性骨软化症1例并文献复习

目的:通过典型病例了解肿瘤性骨软化症(TIO)的临床特点及诊疗方法,提高对TIO的认识水平。方法:对1例TIO患者的临床资料进行整理和分析,并复习相关文献。结果:此例患者表现为全身骨关节痛、活动障碍、血磷下降、肾小管磷重吸收率下降,经68Ga-TATE PET/CT及18-FDG PET/CT检查发现右肛周肿瘤,手术后第2天血磷恢复正常,病理报告为磷酸盐尿性间叶组织肿瘤。结论:对临床表现为全身骨痛、低血磷、肾小管磷重吸收率下降的患者,需高度怀疑TIO,建议积极行影像学检查寻找肿瘤病灶。     

低磷血症-骨软化-右股骨头占位

肿瘤相关性低血磷性骨软化症为罕见疾病。本文报道1例39岁男性,10余年前起逐渐出现乏力、多部位骨痛,进行性加重的活动困难,辅助检查提示低血磷,高尿磷,血钙正常,碱性磷酸酶(ALP)轻度增高,血pH正常,影像学有骨质疏松及骨软化表现,可见肋骨多发骨折,椎体双凹变。诊断为低血磷性骨软化症,因其成年起病,且无家族史,考虑肿瘤相关性低血磷性骨软化症可能性大,分别于2004年及2006年2次住院均未能明确定位诊断,对症服用中性磷制剂后症状明显改善。2010年第3次入院后通过PET-CT、奥曲肽显像及CT检查发现右股骨头内有占位性病变,行手术切除,术后血磷恢复正常,临床症状改善,病理学检查证实为磷酸盐尿性间叶组织肿瘤。     

12例肿瘤性骨软化症的临床诊治及术后随访

目的 通过对肿瘤性骨软化症(tumor-induced osteomalacia,TIO)患者临床特点、诊断及术后疗效随访的分析,提高对TIO的认识及临床诊治水平.方法 本院自2004年至2010年4月间收治确诊的TIO患者12例.分析患者的临床特点、诊断、治疗及手术前后临床及生化指标变化.结果 12例中男性7例,女性5例,平均年龄(41.8±9.6)岁(20～56岁),病程2～14年,中位病程4.0年.12例患者均有骨痛、肌无力及活动障碍.12例患者血磷均呈明显低下,范围0.30～0.56 mmol/L,血钙正常或轻度降低,碱性磷酸酶除1例外均有不同程度的升高,甲状旁腺素(PTH)5例正常,7例轻度升高.本组患者有9例行99mTc-奥曲肽扫描(99mTc-Oct),发现头部(1例),躯干(1例)及四肢不同部位(7例)生长抑素受体高表达病变,另3例患者经查体发现右腰背、左大腿内侧、足底软组织肿物.12例患者均行手术治疗,经病理证实有6例为血管内皮细胞瘤或纤维血管瘤,4例为腱鞘巨细胞瘤或纤维瘤,1例为脂肪肉瘤,1例为尿磷性间叶性肿瘤.手术切除肿瘤后10例血磷恢复正常,患者术后随访2～64个月,血磷正常,骨痛及肌无力均显著改善,可正常行走及生活.结论 加强在低磷软骨病患者中TIO的筛查,采用99mTc-Oct及其他影像检查可有效定位肿瘤,手术治疗去除病灶可使血磷恢复正常,临床症状改善、消失,取得良好的治疗效果.

Abstract：

Objective To better understand the clinical management of tumor-induced osteomalacia (TIO) by analyzing the clinical features, diagnosis, treatment, postoperative biochemical changes, and clinical status in 12 cases of TIO. Methods Twelve cases of TIO hospitalized from 2004 to April 2010 were reviewed retrospectively. All cases were diagnosed based on their clinical manifestation, hypophosphatemia, and image study including technetium-99m octreotide scintigraphy (99mTc-Oct). Resuits There were 7 males and 5 females with mean age of (41.8±9.6) years (20 to 56 years). The course of disease was from 2 to 14 years ( median course 4.0 years). They all presented with bone pain, gait disturbance, muscle pain, and muscle weakness. Serum phosphate( Pi)levels were low in 12 cases with a range from 0.30 to 0.56 mmol/L. 99mTc-Oct was performed in 9 cases and it showed that the lesions were located in head of femur, fibula, retrocalcaneal area, foot, humerus,metacarpal, posterior chest wall or near nasal bone (apex partis petrosae ossis temporalis). Subcutaneous soft tissue mass was found in another 3 cases at loin, thigh, and foot by physical examination. The tumors were confirmed by CT, MRI or ultrasonography. Twelve patients underwent operation to remove the tumors and histopathology showed hemangioendothelioma or fibrous angioma (6 cases), giant cell tumor or fibroma of tendon sheath(4 cases), liposarcoma(1case), and phosphaturic mesenchymal tumor(1case). Serum Pi levels returned to normal in 10 patients after resection of tumor. During 2 to 64 months follow up, symptoms of bone pain and muscle weakness were improved obviously. Conclusions Patients with hypophosphatemic osteomalacia should be thoroughly investigated for TIO. 99mTc-Oct and other imaging examinations can effectively locate the tumors. Once the hidden tumor is found and excised, the patient will recover and enjoy normal life with normalized Pi concentrations and marked improvement of symptoms.     

阿德福韦酯导致低血磷性骨软化症一例并文献复习

目的 探讨阿德福韦酯导致的低血磷性骨软化症的临床特征、治疗方案及转归.方法 对1例慢性乙型肝炎患者服用阿德福韦酯后发生的低血磷性骨软化症进行分析,并就其在全世界慢性乙型肝炎患者中的发生情况做相关文献复习.结果 本例患者的临床症状出现时间与服用阿德福韦酯时间平行,在停用阿德福韦酯并补充中性磷后,临床症状减轻,血磷恢复正常(症状较重时0.77mmol/L,停用阿德福韦酯10周时升至0.98 mmol/L),诊断为阿德福韦酯相关低血磷性骨软化症.复习文献发现阿德福韦酯导致的尿磷排泄增多存在剂量依赖性、时间依赖性、可逆性,而小剂量阿德福韦酯(10 mg/d)治疗慢性乙型肝炎后出现低血磷性骨软化症的报道均来自亚裔人群.结论 阿德福韦酯导致的低血磷性骨软化症为罕见疾病.凡因乙型肝炎服用阿德福韦酯的患者,无论剂量大小,均应定期进行相关检查,如血钙、血磷,以监测是否发生低血磷性骨软化,一旦发生,可换用其他抗病毒药物.

Abstract：

Objective To investigate the clinical features and treatment protocol and prognosis for the hypophosphataemic osteomalacia related to adefovir dipivoxil.Methods Analysis was made upon a case of patient with chronic hepatitis B developed hypophosphataemic osteomalacia after administration of adefovir dipivoxil.Literature review was carried out to survey the global prevalence of hypophosphataemic osteomalacia after administration of adefovir dipivoxil among patients with chronic hepatitis B.Results The clinical symptoms started paralleling to the time taking adefovir dipivoxil,and alleviated after the patient withdrawn adefovir dipivoxil 10 weeks and was given phosphorus.Meanwhile,serum inorganic phosphorus recovered to normal ( 0.98 mmol/L),which lowest level was 0.77 mmol/L.Systematic review of the literature showed that hyperphosphaturia related to adefovir dipivoxil was dose-dependent,time-dependent and reversible.All reported cases of hypophosphataemic osteomalacia secondary to adefovir dipivoxil ( 10mg/d) were from Asian population.ConclusionsAdefovir dipivoxil induced hypophosphataemic osteomalacia is rarely seen in clinical practice.Those patients with chronic hepatitis B who take adefovir dipivoxil,no matter dosages,should take periodical examinations including blood calcium and serium inorganic phosphorus to monitor whether hypophosphataemic osteomalacia occurs.Other anti-virus drugs could be used when it happens.     

特利加压素治疗肝硬化食管胃底静脉曲张出血患者发生低钠血症的临床特征和危险因素

目的探讨特利加压素治疗肝硬化静脉曲张出血过程中,发生低钠血症的患者临床特征及危险因素。方法收集2013年1月1日至2016年12月31日因肝硬化急性静脉曲张出血在上海中医药大学附属普陀医院感染科住院接受特利加压素治疗的患者(特利加压素组),同时入选同期因肝硬化静脉曲张出血住院接受奥曲肽治疗的患者作为对照(奥曲肽组),对其临床表现、实验室检查等资料进行回顾性分析。结果纳入研究患者共107例,特利加压素组患者41例,血钠水平从(138.6±6.4)mmol/L降低至(127±7.8)mmol/L,血钠下降值为(11.9±9.4)mmol/L。其中39例(95.1%)出现血钠水平下降,20例(48.8%)出现显著血钠下降(≥10mmol/L),包括5例(5/20,25%)患者出现神经系统症状,2例患者因血钠迅速上升而出现渗透性脱髓鞘综合征。奥曲肽组患者66例,其中26例(39.4%)出现血钠水平下降,3例(4.5%)出现显著血钠下降,血钠下降值为(0.7±4.0)mmol/L。多因素分析发现,较高基线的血钠水平和白蛋白水平是发生显著低钠血症的独立危险因素。死亡率与低钠血症发生无关。结论特利加压素治疗肝硬化静脉曲张出血过程中发生低钠血症比较普遍,尤其是基线血钠和白蛋白水平较高的患者,需要加强监测。     

踝部肿瘤诱发低磷性骨软化症1例

肿瘤源性骨软化症(tumor-induced osteomalacia,TIO)是成人低磷性骨软化症(hypophosphatemic osteomalacia,HO)的重要病因。本文报告1例双侧颈部、胸部肋骨、腰背部、髋膝关节疼痛1年,伴明显低磷血症的患者,通过PET-CT检查发现右踝致病肿瘤,手术切除后病理证实为磷酸盐尿性间叶肿瘤,患者术后血磷逐渐升高,术后1周血磷恢复正常,术后2周骨痛、肌无力缓解,术后2个月症状完全消失。提示PET-CT检查在TIO的诊断中有一定的价值。     

FGF23、MEPE和sFRP4在肿瘤性骨软化症发病机制中的作用

肿瘤性骨软化症(TIO)是由于肿瘤引起肾脏排磷增加,所造成的获得性低血磷骨软化-症.近来研究显示,肿瘤分泌的体液因子(如调磷因子)可以影响体内磷的平衡,而成纤维细胞牛长因子-23(FGF-23)、细胞外基质磷酸糖蛋白(MEPE)和分泌型卷曲相关蛋白(sFRP)4可能就是调磷因子.它们通过抑制肾小管上皮细胞磷的回吸收,抑制肾脏1,α羟化酶的活性,从而调节血磷,使患者血磷降低,尿磷增多,血1,25(OH)2D3水平降低或正常,从而参与TIO的发生.     

肿瘤诱发低磷性骨软化症1例并文献复习

低血磷性骨软化症（hypophosphatemic osteomalacia,HO）是由于低磷血症和活性维生素D生产不足造成的、以骨骼矿化不良、骨软化或佝偻病为主要特征的一组疾病。分为以下3种类型：X连锁显性低磷性骨软化症（XLH）、常染色体显性遗传低磷性骨软化症（ADHR）和肿瘤诱发低磷性骨软化症（TIO）。现将在黑龙江省大庆油田总医院就诊的1例肿瘤诱发低磷性骨软化症报告如下。     

奥曲肽对晚期消化道肿瘤患者血清胰岛素样生长因子-Ⅰ水平的影响

背景:奥曲肽是一种体外合成的生长抑素类似物,其对肿瘤细胞的抑制作用越来越受到人们的关注.新生血管形成对肿瘤的生长和转移起重要作用.许多抗新生血管形成的治疗已经引入临床试验,生长抑素类似物可用作抗新生血管形成的治疗.目的:评价奥曲肽治疗晚期消化道肿瘤的临床受益反应(CBR)及其对患者血清胰岛素样生长因子(ICF)-Ⅰ水平的影响,探讨奥曲肽的抗肿瘤作用机制.方法:予28例晚期消化道肿瘤患者奥曲肽0.2 mg,每12 h皮下注射1次,直至病情进展或不能耐受治疗为止,综合评估CBR;采用双抗体夹心酶联免疫吸附测定(ELISA)检测治疗前和治疗1个月后的血清IGF-Ⅰ水平.结果:奥曲肽治疗的总体CBR率为60.7%(17/28);治疗1个月后,患者血清IGF-Ⅰ水平(54.8 ng/L±27.4 ng/L)较治疗前(183.1 ng/L±56.6 ng/L)显著下降(P<0.01),其中19例(67.9%)患者为明显下降.结论:奥曲肽能显著降低晚期消化道肿瘤患者血清IGF-Ⅰ水平,抑制肿瘤生长,使患者获得较高的CBR率.     

Tumor-induced osteomalacia caused by a massive phosphaturic mesenchymal tumor of the acetabulum: A case report

We report a case of tumor-induced osteomalacia (TIO) caused by a massive phosphaturic mesenchymal tumor (PMT) of the acetabulum. A 68-year-old woman presented with progressive bone pain of the rib cage, and polyarthralgia and back pain for 3 years. She was diagnosed with hypophosphatemic osteomalacia because laboratory testing was remarkable for low serum phosphorus and a low level of 1,25(OH)2 vitamin D. Three years later, her hip radiograph revealed an osteolytic lesion of the acetabulum. Magnetic resonance imaging of the acetabulum showed a massive lesion. Laboratory data showed hypophosphatemia and an elevated serum level of fibroblast growth factor 23 (FGF-23). Samples obtained with open biopsy showed a low-grade spindle cell neoplasm with FGF-23 positivity, identified by using immunohistochemical staining, confirming the diagnosis of a PMT mixed connective tissue variant. Curettage of the tumor was performed, and the defects were filled with bone allografts. The hip joint was reconstructed with total hip arthroplasty using a Muller support ring. To our knowledge, this report represents the first documented case of massive PMT of the acetabulum causing TIO.     

肿瘤性骨软化症3例并文献复习

目的分析肿瘤性骨软化症的临床特点,以提高对该病的认识。方法回顾性分析近1年确诊的3例肿瘤性骨软化症患者的临床资料,并复习相关文献。结果 3例患者均表现为全身骨痛、身高缩短和活动困难,实验室检查提示低血磷和高骨转换,影像学检查提示骨质疏松、骨软化和假性骨折,2例经PET-CT等影像学检查、1例经查体发现肿瘤,均予手术切除后病理证实,3例患者术后血磷均显著上升。结论肿瘤性骨软化症有典型临床特点,切除肿瘤后可治愈。及早明确肿瘤位置是关键,仔细查体和PET-CT等影像学检查对肿瘤的发现和定位具有重要意义。     

Oncogenic osteomalacia and inappropriate antidiuretic hormone secretion due to oat-cell carcinoma

The syndrome of tumor-induced osteomalacia has been previously thought to occur only in association with mesenchymal tumors, although one report has linked prostatic carcinoma with the syndrome. We report the case of a patient who presented first with the clinical and biochemical features of the syndrome of inappropriate antidiuretic hormone secretion, and then oncogenic osteomalacia. The first syndrome was characterized by headaches, nausea, and vomiting; serum sodium determinations ranged between 107 and 118 meq/L with simultaneous urine spot sodium concentrations of 100 to 116 meq/L. The circulating antidiuretic hormone level was markedly elevated to 261.5 microU/mL. The osteomalacia was discovered incidentally when depressed serum phosphorus levels of 1.2 to 1.7 mg/dL were noted in association with 24-hour urine phosphorus excretion exceeding 1000 mg/24 h. Undecalcified tetracycline-labeled bone biopsy samples confirmed oncogenic osteomalacia. Only afterward was a small-cell carcinoma of the lung identified as the likely source of both of these syndromes.     

体检人群非酒精性脂肪性肝病检出率及其危险因素分析

目的 分析体检人群非酒精性脂肪性肝病(NAFLD)流行率及其危险因素。方法 2018年1月～2020年1月我院体检中心接受体检人群1742例,排除干扰因素后,纳入485例,经超声检查诊断NAFLD者78例,非NAFLD者407例。使用CT扫描仪测量腹腔内脏脂肪面积(VAT)和腹壁皮下脂肪面积(SAT)。检测空腹血糖(FPG)等生化指标。应用单因素和多因素Logistic回归分析NAFLD发病的影响因素。结果 NAFLD组高血压、糖尿病和血脂血症患病率分别为29.5%、35.9%和51.3%,显著高于非NAFLD组(分别为18.7%、23.8%和23.6%,P<0.05),BMI、腰围和臀围分别为(25.1±1.)kg/m²、(90.4±6.3)cm和(101.3±8.4)cm,显著高于非NAFLD组【分别为(22.4±1.1)kg/m²、(81.6±5.7)cm和(95.7±6.2)cm,P<0.05】;血清TG、LDL-C和VAT分别为(2.9±0.3)mmol/L、(3.7±0.4)mmol/L和(146.3±12.1)cm<...     

Highly metabolic thrombus of the portal vein： ^18F fluorodeoxyglucose positron emission tomography/computer tomography demonstration and clinical significance in hepatocellular carcinoma

AIM： To assess the ability of ^18F-fluorodeoxyglucose positron emission tomography/computer tomography （^18F-FDG PET/CT） to differentiate between benign and malignant portal vein thrombosis in hepatocellular carcinoma （HCC） patients.
METHODS： Five consecutive patients who had HBV cirrhosis, biopsy-proven HCC, and thrombosis of the main portal vein and/or left/right portal vein on ultrasound （US）, computer tomography （CT） or magnetic resonance imaging （MRI） were studied with ^18F-FDG PET/CT. The presence or absence of a highly metabolic thrombus on ^18F-FDG PET/CT was considered diagnostic for malignant or benign portal vein thrombosis, respectively. All patients were followed-up monthly with US, CT or MRI. Shrinkage of the thrombus or recanalization of the vessels on US, CT or MRI during follow-up was considered to be definitive evidence of the benign nature of the thrombosis, whereas enlargement of the thrombus, disruption of the vessel wall, and parenchymal infiltration over follow-up were considered to be consistent with malignancy. ^18SF-FDG PET/CT, and US, CT or MRI results were compared.
RESULTS： Follow-up （1 to 10 mo） showed signs of malignant thrombosis in 4 of the 5 patients. US, CT or MRI produced a true-positive result for malignancy in 4 of the patients, and a false-positive result in 1. ^18F-FDG PET/CT showed a highly metabolic thrombus in 4 of the 5 patients. ^18F-FDG PET/CT achieved a true-positive result in all 4 of these patients, and a true-negative result in the other patient. No false-positive result was observed using ^18F-FDG PET/CT.
CONCLUSION： ^18F-FDG PET/CT may be helpful in discriminating between benign and malignant portal vein thrombi. Patients may benefit from ^18F-FDG PET/CT when portal vein thrombi can not be diagnosed exactly by US, CT or MRI.     

F-FDG PET/CT诊断胆管细胞癌分期价值研究

目的 探讨采用¹⁸F-氟代脱氧葡糖糖(¹⁸F-FDG)正电子发射计算机断层扫描(PET/CT)诊断肝内胆管细胞癌(ICC)分期的效能。方法 2017年1月～2020年1月我院收治的107例ICC患者和同期107名体检且身体健康的志愿者,接受¹⁸F-FDG PET/CT检查,行TNM分期评估,勾画感兴趣区域,计算最大标准摄取值(SUV_max)。所有患者接受手术治疗,术后行组织病理学检查。结果 Ⅰ期ICC组SUV_max值为(4.1±1.1)、Ⅱ期为(7.2±1.5)、Ⅲ期为(9.3±1.8),均显著高于健康组的【(2.0±0.9),P＜0.05】;¹⁸F-FDG PET/CT检查显示在107例ICC患者中,TNM分期Ⅰ期为35例,Ⅱ期为47例,Ⅲ期为25例,术后组织病理学检查显示Ⅰ期、Ⅱ期和Ⅲ期分别为34例、48例和24例;经一致性分析,¹⁸F-FDG PET/CT诊断ICC患者TNM Ⅰ期的灵敏度为0.968,特异度为0.500,准确率为0.914,阳性预测值为0.938,阴性预测值为0.667,诊断Ⅱ期的灵敏度为0.976,特异度为0.667,准确率为0.956,阳性预测值为0.976,阴性预测值为0.667,诊断Ⅲ期的灵敏度为0.955,特异度为0.667,准确率为0.920,阳性预测值为0.955,阴性预测值为0.667。结论 ¹⁸F-FDG PET/CT检查能够帮助诊断ICC,并协助TNM分期的评估,具有较高的临床应用价值。     

应用MRI多回波水脂分离技术评估非酒精性脂肪性肝病患者肝脏脂肪含量效能研究

目的 探讨使用磁共振成像(MRI)多回波水脂分离技术(IDEAL-IQ)诊断非酒精性脂肪性肝病(NAFLD)的效能.方法 2018年2月～2020年2月我院诊治的120例NAFLD患者和65例体检健康人,接受MRI检查,分别采用IDEAL-IQ定量检测肝脏相对脂肪含量(RLC)和采用氢质子磁共振波谱(MRS)检测脂肪分...