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甲状腺功能减退症伴多发性肌炎样综合征三例

【摘要】 目的 提高对甲状腺功能减退致多肌炎综合征的认识。方法 报道3例甲状腺功能减退症致多肌炎综合征的临床特点。男2例,年龄分别为33、38岁；女1例,年龄64岁,均表现为四肢近端肌肉肌无力,肌酶显著升高。结果 2例男性诊断为原发性甲状腺功能减退症,女性为亚临床型甲状腺功能减退症,甲状腺激素替代治疗后效果好。结论 临床上对有肌病表现、肌酶显著升高的患者须注意有无甲状腺功能减退症,多发性肌炎样综合征,应注意筛查甲状腺功能。

Hemispherebrain Multiple Sclerosis Misdiagnosed as the "Spinal Cord Lesions":3 Cases Clinical and MRI Analysis

多发性硬化临床表现多样,其中大脑半球型多表现为精神症状、癫痫、偏瘫或感觉异常等,而以截瘫及排尿障碍为表现者少见。本文对表现为“脑性截瘫”的3 例MS患者的临床和MRI特点进行回顾分析,以此提高对于MS的认识水平。

KL-6联合肺功能在多发性肌炎/皮肌炎合并间质性肺疾病中的应用价值

目的 探讨血清KL-6和肺功能检测在多发性肌炎（polymyositis，PM）/皮肌炎（dermatomyositis，DM）合并间质性肺疾病（interstitial lung disease，ILD）中的临床应用价值。方法 采用回顾性分析，纳入初发PM/DM患者（n=64）临床资料，分为间质肺组（n=40）和无间质肺组（n=44），另选健康对照组40例，比较三组患者血清KL-6水平及肺功能在两组的差异并分析其在PM/DM-ILD组中的应用价值。结果 间质肺组患者KL-6水平明显高于无间质肺组（P<0.01）；间质肺组患者VC%pred、MVV%pred、FEV1%pred、DLCO%pred、DLCO/VA%pred、TLC%pred均低于无间质肺组（P均<0.05）；ROC曲线分析得出PM/DM患者中血清KL-6诊断ILD敏感性为84%，特异性为87.5%，DLCO%pred诊断ILD的敏感性为97.7%，特异性为57.5%，而二者联合检测敏感性为90.5%，特异性为82.5%；间质肺组血清KL-6与VC%pred、MVV%pred、FVC%pred、FEV1%pred、DLCO%pred成反比，与FEV1/FVC%pred成正比（P均<0.05）；PM/DM发生ILD的危险因素：AST降低、KL-6升高及DLCO%pred降低。结论 PM/DM患者联合检测血清KL-6和肺弥散功能可提高ILD诊断效能，KL-6和肺功能可能预测ILD的发生及疾病活动度，可指导临床。

Construction of Lentiviral Vector Containing Murine Vascular Endothelial Growth Factor Gene and Its Expression in NS-1 Murine Myeloma Cell Line

目的　构建含小鼠血管内皮生长因子（mVEGF）的重组慢病毒表达载体，包装成病毒颗粒后感染NS-1小鼠骨髓瘤细胞株，以便进一步探索VEGF在骨髓瘤病理生理机制中的作用。 方法　聚合酶链反应法扩增mVEGF基因，克隆入含嘌呤霉素抗性的pCDH慢病毒表达载体，构建出表达mVEGF的慢病毒表达载体pCDH-mVEGF；采用磷酸钙法将慢病毒系统三质粒pCDH-mVEGF、psPAX2、pMD2.G共转染293FT细胞包装病毒，分别收集转染后48 h和72 h病毒上清并感染靶细胞NS-1，初次感染72 h后开始采用嘌呤霉素筛选稳定株，筛选2周后采用ELISA法检测稳定株细胞培养上清中mVEGF的表达，建立出稳定高表达mVEGF的NS-1小鼠骨髓瘤细胞株。 结果　成功构建重组慢病毒表达质粒pCDH-mVEGF，并包装成慢病毒颗粒，感染NS-1细胞株后获得靶基因的稳定高表达。 结论　成功构建出含mVEGF的慢病毒表达载体pCDH-mVEGF，慢病毒系统能有效介导目的基因在NS-1小鼠骨髓瘤细胞株中稳定表达，病毒包装成功并能有效感染NS-1细胞，为进一步探索VEGF在骨髓瘤病理生理机制中的作用奠定了基础。

Analysis of manifestation of fundus fluorescein angiography in multile sclerosis

ObjectiveTo observe the features of the manifestations of fundus fluorescein angiography （FFA） in multiple sclerosis （MS） and their value in clinical diagnosis.MethodsThe clinical data of 42 patients (84 eyes) with MS diagnosed by magnetic resonance imaging (MRI) and examination of cerebrospinal fluid (CSF) were retrospectively analyzed. The clinical data included visual acuity, ocular fundus examined by direct ophthalmoscope after mydriasis, FFA, visual field, CSF，visual evoked potential (VEP) and MRI examination.ResultsIn 42 patients (84 eyes),the positive detectable rate of examination of direct ophthalmoscope, CSF, visual field, VEP, and MRI was 36.9%, 21.4%, 71.4%, and 83.3% respectively. Abnormal results of FFA were found in 44 eyes (52.38%), including papillitis in 4 eyes（4.76%）at the early stage with extended physiological scotoma and central scotoma; neuroretinitis in 7 eyes （8.33%）at the medium stage with central or para-central scotoma; optic atrophy in 33 eyes（39.29%） at the late stage with centripetal constriction and even tubular visual field. ConclusionThe main angiographic features of MS are papillitis, neuroretinitis and optic atrophy. The manifestations of FFA combined with the results of examination of CSF，visual field, VEP and MRI is helpful for comprehensive and exact diagnosis of MS.(Chin J Ocul Fundus Dis, 2005,21:300-302)

Thrombotic Complications in Multiple Myeloma: A Case Report and the Literature Review

目的　提高对多发性骨髓瘤合并动静脉血栓形成的临床诊治水平。 方法　报道1例表现为反复胸腔积液，同时合并反复、多处静脉和动脉血栓形成患者的临床资料，并复习文献。 结果　该例62岁老年女性患者，合并右侧下肢静脉、肾动脉、脑动脉、颈动脉、肺动脉血栓反复形成，检查后明确诊断为多发性骨髓瘤IgG λ型、原发性淀粉样变，合并多处动静脉血栓形成，给予美法仑+泼尼松（MP）方案化疗和抗血小板、抗凝治疗后症状改善，随访13个月病情稳定，无新发血栓形成。 结论　多发性骨髓瘤可能合并动静脉血栓形成等不典型表现，需要进一步提高认识。

Daily nursing for multiple myeloma: whole life cycle health management based on the doctor-nurse-patient integration model

With the continuous development of new drugs and immunotherapy, the survival period of patient with multiple myeloma (MM) is continuously prolonged, and the disease is becoming chronic. Due to the involvement of multiple systems and numerous complications, the daily nursing for MM faces significant challenges. The doctor-nurse-patient integration model and the whole life cycle health management model for daily nursing of MM are expected to reduce the social burden related to diseases, improve patients’ quality of life, and reduce medical costs. This article provides a review on three aspects of MM doctor-nurse-patient integration, whole life cycle health management, and daily health management involving multiple systems.

无环鸟苷致多发性骨髓瘤合并肾功能不全患者精神障碍一例

生物学检测技术在多发性骨髓瘤临床应用中的意义

多发性骨髓瘤（MM），又称浆细胞骨髓瘤，是起源于浆细胞克隆扩张的高异质性的肿瘤，是血液系统第二大常见的恶性疾病，然而临床上对其认识至今仍止于表面。目前，现代生物学检测技术在肿瘤中的应用已能帮助我们从新的角度与层面去深入认识和了解MM，这将有希望改变目前临床上千篇一律的诊治模式，指导更为个体化的评估及治疗。

Efficacy and safety analysis of glucocorticoid and tacrolimus in the treatment of anti-synthetase syndrome-associated interstitial lung disease

Objective To evaluate the efficacy and safety of glucocorticoids (GC) monotherapy and GC combined with tacrolimus (TAC) therapy in patients with anti-synthetase syndrome-associated interstitial lung disease (ASS-ILD). Methods Through retrospective analysis and propensity score matching (PSM) analysis, the 2-year progression-free survival (PFS) and related side effects of ASS-ILD patients in TAC+GC group and GC monotherapy group were compared. Predictors associated with PFS were analyzed with COX. Results The 2-year PFS rate of TAC+GC group was better than that of GC group [P=0.0163; hazard ratio (HR) 0.347]; Univariate and multivariate analysis of the COX regression model for 2-year PFS in the two groups suggested that creatine kinase level (P=0.0019, HR 1.002) and initial treatment selection [(TAC+GC) vs. GC, P=0.0197, HR 0.207] were independent predictors of PFS; PSM analysis showed that the 2-year PFS rate of TAC+GC group (54.5%) was higher than that of GC group (18.2%) (P=0.0157, HR 0.275). In terms of adverse effect, there was no significant increase in GC+TAC group compared with GC group. Conclusion Compared with GC monotherapy, initial TAC+GC treatment significantly prolonged PFS in ASS-ILD patients and did not increase the incidence of drug-related complications.