Mechanical pulmonic valve thrombosis: expanding role of cardiac CT and multimodality imaging

We report a case of a 44-year-old man with a clinical history of Tetralogy of Fallot status post staged surgical correction with mechanical pulmonic valve replacement who presented with progressive exertional dyspnea in the setting of non-compliance with anticoagulation. In the context of this suggestive clinical presentation, the diagnosis of mechanical pulmonic valve thrombosis (MPVT) was made possible via multimodality imaging, including transthoracic echocardiogram and cardiac computed tomography angiography. Due to the uncommon nature of the condition, the patient was treated with systemic thrombolysis and anticoagulation using evidence-based guidelines, largely extrapolated from left-sided mechanical valve thrombosis. Our case underscores the importance of anticoagulation in MPVT and recognizing the features of MPVT on clinical history, physical examination, and multimodality imaging. It is essential to understand the pivotal role of multimodality imaging in the assessment of MPVT and realize the limitations of available data regarding the management of MPVT in the current era.     

Successful treatment of thrombotic thrombocytopenic purpura with plasmapheresis and anti-CD20 antibodies in a patient with immune thrombocytopenia and systemic lupus erythematosus: Case report

Rationale:Systemic lupus erythematosus (SLE) is an autoimmune disease of unknown etiology with diverse clinical and laboratory manifestations, including thrombocytopenia. About 25% of patients with SLE may be affected by thrombocytopenia, many of whom are asymptomatic. Some patients, however, experience platelet counts that drop quite low and predispose them to bleeding. Thrombotic thrombocytopenic purpura (TTP) is defined with a classic pentad of clinical features, such as thrombocytopenia, microangiopathic hemolytic anemia, neurological symptoms and signs, renal symptoms and signs, and fever. The association of TTP and SLE has been sporadically reported in the literature.Patient concerns and diagnosis:We describe a 16-year-old girl with SLE and immune thrombocytopenia, in whom TTP was diagnosed.Interventions and outcomes:She was treated with pulse methylprednisolone, whose platelet counts normalized after therapy with plasmapheresis and an anti-CD20 monoclonal antibody (rituximab).Conclusion:A pediatric patient with SLE and immune thrombocytopenia in whom TTP developed was treated with plasmapheresis and rituximab therapy successfully, though the patient experienced a disease relapsed after 18 months, which was controlled by the same management.     

挑战:巨噬细胞活化综合征和脓毒症相关噬血细胞淋巴组织细胞增生症

无论原发性还是继发性噬血细胞淋巴组织细胞增生症都可能是致死性的,早期发现和及时治疗是改变结局的关键.虽然在基因缺陷和免疫发病机制研究进展的基础上形成的免疫化疗和定向免疫治疗使部分患儿的生存率得到了改善,但在ICU降低噬血细胞淋巴组织细胞增生症的病死率依然面临挑战.持续发热、伴随凝血异常的特殊性肝功能不良、高甘油三脂血症、血细胞减少和异常升高的血清铁蛋白是重要的诊断线索.明确患者自然杀伤细胞在脓毒症和自身免疫性疾病不同阶段的情况将引导创新免疫介入治疗的实现.     

全身炎症评分在不可切除转移性结直肠癌患者预后评估中的价值

目的:探讨全身炎症评分（SIS）对不可切除转移性结直肠癌（mCRC）患者预后评估中的价值。方法:回顾性分析2014年1月至2016年12月中南大学湘雅医学院附属海口医院收治的130例不可切除mCRC患者的临床资料。分析SIS与不可切除mCRC患者临床病理特征的关系,采用Kaplan-Meier法进行生存分析,应用Cox...     

养阴清热 补肾化瘀中药联合CTX治疗狼疮性肾炎的临床研究

目的:观察养阴清热,补肾化瘀法联合环磷酰胺(CTX)治疗狼疮性肾炎的疗效。方法:采用随机、平行、对照比较方法,分中医组、中西医组两组,均为上海市中医医院风湿科门诊和住院病人。每组各30例,共60例,疗程6个月,对蛋白尿及免疫指标进行观察。结果:(1)总有效率分别为中医组96.7%,中西医组93.33%。(2)两组在治疗蛋白尿方面均有疗效(P0.05,P0.01)。(3)中西医结合、中医治疗均能降低血沉(P0.01,P0.05)。(4)中西医结合治疗及中医治疗均能降低dsDNA(P0.05)。中西医结合治疗能升高C4(P0.05)。结论:滋阴清热、补肾化瘀中药联合CTX能改善蛋白尿、调节免疫的作用,减少激素用量。     

稳定敲低SIGIRR的人肾小管上皮细胞的构建及其初步功能的研究

目的：建立稳定敲低单免疫球蛋白白介素1相关受体(SIGIRR)基因的人肾小管上皮细胞株(HKC),并初步研究其功能。方法针对SIGIRR基因的有效靶点设计shRNA序列,与 GV248-GFP-Puro 慢病毒载体连接产生重组体( GV248-GFP-Puro-shSIGIRR)。将测序验证正确的重组体与包装质粒(pMDL、pRev、pVSVG)共转染293T细胞进行病毒包装,收集病毒并感染HKC细胞。实时荧光定量PCR( qRT-PCR)和Western blot法分析检测HKC细胞中SIGIRR干涉效率。应用白细胞介素-1β( IL-1β)刺激成功敲低SIGIRR的HKC细胞及对照细胞, Western blot法检测其下游核转录因子NF-κB(p65)的磷酸化水平,qRT-PCR分析单核细胞趋化蛋白-1(MCP-1)、正常 T细胞表达和分泌的活化调节蛋白( RANTES) mRNA水平。结果成功构建重组慢病毒载体GV248-GFP-Puro-shSIGIRR。 qRT-PCR 和 Western blot 法均证实在HKC细胞中成功敲低SIGIRR的表达。此外, IL-1β刺激后,与对照细胞相比,敲低SIGIRR的HKC细胞( HKC/shSIGIRR)的p65磷酸化水平上调, MCP-1和 RANTES mR-NA表达水平升高。结论 HKC的炎症反应中,SIGIRR蛋白对Toll样受体/白介素1受体(TLR/IL-1R)通路起“刹车”作用。该研究为狼疮性肾炎的治疗提供了新的潜在的靶点。     

复方生地合剂治疗系统性红斑狼疮（阴虚内热型）的有效性与安全性评价

目的：评价复方生地合剂治疗阴虚内热型系统性红斑狼疮（SLE）的临床疗效及其安全性。方at：选择阴虚内热型SLE患者65例,随机分为治疗组31例和对照组34例。两组均以糖皮质激素作为基础用药,治疗组给予复方生地合剂,对照组给予硫酸羟基氯喹治疗,疗程12周。观察两组的临床疗效、系统性红斑狼疮疾病活动指数（SLEDAI）、免疫学指标及安全性指标。结果：治疗后,治疗组和对照组的总有效率分别为60．0％和56．7％,两组比较差异无统计学意义（P〉0．05）。治疗后,两组患者的SLEDAI、抗ds-DNA水平均显著降低（P〈0．01,P〈0．05）,但两组比较差异无统计学意义（P〉0．05）。治疗过程中,治疗组和对照组的不良反应事件发生率分别为10．0％和34．3％,治疗组明显低于对照组（P〈0．05）。结论：复方生地合剂对SLE有一定的免疫抑制作用,且能降低SLE的活动度,毒副反应较少。     

女性系统性红斑狼疮患者骨代谢异常的危险因素分析

目的 探讨骨代谢、骨密度与外周血淋巴细胞亚群的关系,初步探索免疫细胞在系统性红斑狼疮(systemic lupus erythematosus, SLE)骨代谢异常中的作用。方法 采用双能X线吸收法(dual energy X-ray absorptiometry, DEXA)检测SLE患者的骨密度,同日检测患者的外周血淋巴细胞亚群分布。结果 共纳入92名SLE女性患者,患者的平均年龄及病程分别为(42.1±14.1)岁、(84.4±73.0)个月。有62.0%(n=57)患者存在骨代谢异常,其中44.6%(n=41)患者为骨量减少,17.4%(n=16)患者并发骨质疏松症,后者还包括9.8%(n=9)发生脆性骨折的患者。全身骨密度最低值与年龄(r=-0.291,P=0.005)、病程(r=-0.239,P=0.022)、绝经状态(r=-0.288,P=0.005)及碱性磷酸酶(r=-0.221,P=0.033)均呈负相关。Logistics分析显示绝经状态(OR=13.0,P<0.001,95%CI 3.43～49.5)、LDL-C(OR=5.74,P=0.002,95%CI ...     

红斑狼疮并肺、胸膜改变27例临床分析

我科从１９９３年１月～１９９５年１０月收治系统性红斑狼疮患者４６例。发现肺部有不同程度改变２１例（４５．６％），胸膜腔渗出６例（１３．０％）。２７例肺、胸膜改变中Ｘ线胸部正位片提示：双肺纹理增多模糊８例（２９．６％），双肺斑片影７例（２５．９％），双侧性胸膜腔渗出液４例（４．８％），单侧性胸膜腔渗出液２例（７．４％），反复咳嗽无痰２例（７．４％）。持续性胸痛２例（７．４％）。肺部可闻湿性罗音２例（７．４％）。Ｂｃｒｎａｒｄｏ等尸检发现［１］，红斑狼疮合并肺脏病变的原因为免疫组织学检查可见肺泡膜呈颗粒状沉着，免疫组织病理学证实狼疮性肺炎系ＤＮＡ、抗ＤＮＡ抗体的免疫复合物沉积作用，导致肺实质纤维化和肺间质小血管非特异性炎症等病理改变。