连续性血液净化对危重病人血磷水平的影响

目的：研究连续性血液净化对病人血磷水平的影响。方法：390例行连续性血液净化治疗的患者,分为正常值以上血磷及轻、中、重度低磷血症。取治疗前日、治疗次日、（未加干预）治疗结束次日晨7时的空腹静脉血磷值,比较差异。结果：接受连续性血液净化治疗后血磷水平较治疗前显著下降（P〈0.01）,且82.3%患者存在低磷血症。如不加干预,治疗结束次日晨血磷不能恢复至治疗前水平（P〈0.01）。结论：连续性血液净化治疗可以降低患者的血磷水平,造成低磷血症,停止血液净化治疗后不能自行恢复正常,需给予适当磷制剂补充治疗。     

Effective detection of the tumors causing osteomalacia using [Tc-99m]-HYNIC-octreotide (99mTc-HYNIC-TOC) whole body scan

Purpose

Tumor-induced osteomalacia (TIO) is an endocrine disorder caused by tumors producing excessive fibroblast growth factor-23 (FGF-23). The causative tumors are generally small, slow-growing benign mesenchymal tumors. The only cure of the disease depends on resection of the tumors, which are extremely difficult to localize due to their small sizes and rare locations. Since these tumors are known to express somatostatin receptors, this research was undertaken to evaluate efficacy of [Tc-99m]-HYNIC-octreotide (99mTc-HYNIC-TOC) whole body imaging in this clinical setting

Methods

Images of 99mTc-HYNIC-TOC scans and clinical chart from 183 patients with hypophosphatemia and clinically suspected TIO were retrospectively reviewed. The scan findings were compared to the results of histopathological examinations and clinical follow-ups.

Results

Among 183 patients, 72 were confirmed to have TIO while 103 patients were found to have other causes of hypophosphatemia. The possibility of TIO could not be either diagnosed or excluded in the remaining 8 patients. For analytical purposes, these 8 patients who could neither be diagnosed nor excluded as having TIO were regarded as having the disease, bringing the total of TIO patients to 80. The 99mTc-HYNIC-TOC scan identified 69 tumors in 80 patients with TIO, which rendered a sensitivity of 86.3% (69/80). 99mTc-HYNIC-TOC scintigraphy excluded 102 patients without TIO with a specificity of 99.1% (102/103). The overall accuracy of 99mTc-HYNIC-TOC whole body scan in the localization of tumors responsible for osteomalacia is 93.4% (171/183).

Conclusions

Whole body 99mTc-HYNIC-TOC imaging is effective in the localization of occult tumors causing TIO.     

阿德福韦酯致范可尼综合征并发低血磷性骨软化症的肾毒性研究

目的探讨标准剂量阿德福韦酯(Adefovir dipivoxil,ADV)致范可尼综合征(Fanconi syndrome,FS)并发低血磷性骨软化症(hypophosphatemic osteomalacia,HPOM)对肾毒性的相关性。方法回顾性分析2014年9月至2020年4月南通大学附属海安医院收治的ADV致FS并发HPOM 7例患者的临床资料、肝功能、肾功能、尿常规、电解质和骨密度等指标,分析肾毒性早期敏感指标。结果7例患者均存在不同程度的血钾、血钙、血磷和血尿酸水平降低,ALP和SOP升高,PRO和GLU阳性等异常情况。经过相关性分析,血尿酸与血磷呈负相关(OR=3.26,95%CI:1.73~4.35,P=0.019),血尿酸与ALP呈负相关(OR=2.89,95%CI:1.59~2.89,P=0.041)。7例患者均存在不同程度的骨质疏松表现,6例患者存在不同程度的骨痛情况。6例患者停用ADV后,病情均逐渐得到明显改善;其中1例66岁的男性患者服药史14年,入院前已有恶液质表现,最终全身衰竭死亡。结论血尿酸与血磷水平降低可能是ADV相关肾毒性的灵敏性和特异性指标,临床上应加强监测和早期干预,以改善疾病预后。     

Axial osteomalacia: Report of a new case with selective increase in axial bone mineral density

Axial osteomalacia was diagnosed in a 60-year-old white Caucasian male, on the basis of X-ray examination showing typical coarsened and sponge-like appearance of trabecular bone strictly located in the axial skeleton. Dual energy X-ray absorptiometry demonstrated a marked increase in axial bone density at lumbar spine levels in both anteroposterior and lateral views (T score: +7.9 and +5.5, respectively), contrasting with normal values at femoral neck, trochanter and midfemoral diaphysis (T score: +0.6, +0.3, and +0.8, respectively). Histomorphometry of iliac crest showed marked thickening of cortices (1730 and 2763 μm, N = 967 ± 57), increased trabecular bone volume (28.9%, N = 17.4 ± 5.6), normal eroded surface (3.5%, N = 3.6 ± 1.1), and increased osteoid surface and volume (31.6%, N =15.3 ± 9.4 and 5.8%, N = 2.8 ± 1.8). Osteoid thickness was increased (39.6 μm, N = 10.5 ± 1.8, up to 120 pm in one site) and mineral apposition rate was decreased in the trabecular sites (0.43 μm/day, N = 0.72 ± 0.12, down to <0.2 pm/day, focally). Bone fluoride content was not increased (0.08%, N < 0.10%). All biochemical parameters of bone metabolism, including serum osteocalcin, PTH, calcidiol, calcitriol, and renal tubular reabsorption of calcium or phosphate were normal.     

Tumor-induced osteomalacia: long-term follow-up of two patients cured by removal of their tumors

Two patients with mesenchymal tumors had osteomalacia associated with marked hypophosphatemia and renal phosphate wasting. Their serum calcium and parathyroid hormone levels were normal. The first patient also had aminoaciduria and glucosuria in addition to phosphaturia. Both patients were treated with very large doses of vitamin D2 and phosphate without improvement in the osteomalacia or normalization of the serum phosphate. Complete removal of a low-grade fibrosarcoma in the second patient and removal of an ossifying fibroma in the first patient resulted in a cure in both patients despite no further therapy with vitamin D or phosphate. The excessive aminoaciduria and glucosuria of the first patient also remitted. During a 14-year follow-up period, there has been no evidence of tumor recurrence, hypophosphatemia, or osteomalacia in either patient. The levels of 1,25-dihydroxycholecalciferol remain within the normal range in both patients.     

肿瘤相关性低磷抗D骨软化症的外科干预治疗

目的观察分析采用外科手术方法治疗肿瘤相关性低磷抗D骨软化症的早期疗效及此类肿瘤的外科学特点.方法从2004年2月至2005年3月,对7例诊断为肿瘤相关性低磷抗D骨软化症的患者进行手术治疗,完整切除软组织肿瘤5例,骨肿瘤2例.术后平均随访4个月（2周～12月）.结果6例患者术后临床症状明显改善,血磷恢复正常,1例未见明显改善.结论肿瘤低磷抗D骨软化症是一种罕见病例,5例软组织肿瘤临床症状少,常规体检不易发现;2例骨肿瘤在X线片上仅表现为骨密度增高区,无明显溶骨及破坏,易被误诊.一经确诊,手术治疗效果良好.     

肿瘤性骨软化症特征及诊治

 目的 通过对肿瘤性骨软化症(tumor-induced osteomalacia ,TIO)患者临床特点、诊断过程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 回顾性分析15例TIO确诊患者的临床特点及手术前后临床及生化指标变化。结果 15例年龄(42.8±10.7)岁,病程为(3.5±2.8)年;其中2例复发(13.3%),1例复发后恶变(6.7%)。血磷(0.41±0.10)mmol/L、肾磷酸根阈值( RTPC)(0.33±0.10)mmol/L,均低于正常值。15例共住院手术19人次,肿瘤位于骨组织10人次,软组织9人次,13例手术一次性治愈(86.7%)。19次术后病理结果均为磷酸盐尿性间叶细胞瘤混合结缔组织型(PMTMCT)。分析显示:肿瘤位于软组织患者较肿瘤位于骨组织患者血磷及RTPC均更低(t=2.264,P=0.041);血磷主要受RTPC影响(标准系数0.836,t=3.862,P=0.005);RTPC受肿瘤体积、肿瘤组织来源相关影响(标准系数0.405、0.546,t=-2.635、3.925,P=0.030、0.004)。结论 对于TIO的患者应警惕恶性及复发的可能;RTPC能较24 h尿磷更好地反映肾小管重吸收磷的受损情况;肿瘤位于软组织患者较肿瘤位于骨组织病情更为严重。     

Osteoporosis and Kyphoplasty

ABSTRACT

Questions from patients about analgesic pharmacotherapy and responses from authors are presented to help educate patients and make them more effective self-advocates. The topics addressed in this issue are osteoporosis and osteomalacia, with a discussion of conservative management, including kyphoplasty, vertebral augmentation, pain control, and quality of life.