利妥昔单抗治疗难治性特发性血小板减少性紫癜的临床观察

目的 观察利妥昔单抗治疗难治性特发性血小板减少性紫癜(RITP)的临床疗效.方法 采用利妥昔单抗治疗33例RITP患者,于治疗前后定期监测外周血小板计数、血清免疫球蛋白水平、中性粒细胞计数,观察治疗可能存在不良反应.结果 临床治疗总有效率为81.8%,不良反应轻微.结论 利妥昔单抗治疗RITP疗效确切.     

成人慢性原发性ITP患者利妥昔单抗疗效的影响因素及Plt计数预测价值探讨

目的:探讨成人慢性原发性免疫性血小板减少症(ITP)患者利妥昔单抗疗效的影响因素及血小板(Plt)数预测价值。方法:回顾性分析本院2012年1月-2016年12月收治的52例行利妥昔单抗治疗成人慢性原发性ITP患者的临床资料,其中治疗失败32例设为A组,治疗成功20例设为B组,分析影响利妥昔单抗疗效的独立危险因素,观察首次诊断骨髓CD41~+巨核细胞计数对治疗随访1年患者治疗反应率的影响,计算Plt数用于疗效预测时,效能指标及最佳截断点。结果:B组首次诊断骨髓CD41~+巨核细胞数水平高于A组(P0.05)。多因素Logistic回归模型分析结果显示,首次诊断骨髓CD41~+巨核细胞数150是影响利妥昔单抗疗效的独立危险因素(OR=5.40,95%CI:1.82-15.66,P=0.00)。首次诊断骨髓CD41~+巨核细胞数≥150组患者随访1年反应率显著高于150组(P0.05)。B组利妥昔单抗首次治疗后d 3、14、21、30、60、90、180、270和360 Plt数水平显著低于A组(P0.05)。ROC曲线分析结果显示,Plt数最佳截断点为50×10~9/L;利妥昔单抗首次治疗后d14,AUC为0.68(95%CI:0.57-0.78,P=0.00);成人慢性原发性ITP患者利妥昔单抗疗效预测敏感度和特异度分别为48.73%和87.58%;利妥昔单抗治疗后d 30和60 AUC分别为0.74(95%CI:0.64-0.87)(P=0.00)和0.93(95%CI:0.82-0.98)(P=0.00)。结论:成人慢性原发性ITP患者接受利妥昔单抗治疗后,部分可获得长期缓解,但骨髓巨核细胞数150的患者预后较差;同时根据利妥昔单抗治疗后d 14、30及60 Plt数能够有效预测患者的远期疗效,指导治疗的方案制定。     

两种小剂量利妥昔单抗治疗方案治疗原发免疫性血小板减少症疗效比较

目的 比较两种小剂量利妥昔单抗治疗方案治疗成人原发免疫性血小板减少症(ITP)的疗效。方法 51例ITP患者非随机分为两组:A组31例患者利妥昔单抗用量为100 m g/周,连续4周;B组20例患者利妥昔单抗用量为375 mg/m2,只用1次。结果 A组:总有效(OR)率和完全反应(CR)率分别为58％和29％,中位反...     

利妥昔单抗治疗难治性免疫性血小板减少性紫癜31例分析

目的 探讨利妥昔单抗(抗CD20单抗)治疗难治性免疫性血小板减少性紫癜(ITP)的安全性和有效性.方法 应用利妥昔单抗(375mg/m2,静脉输注,每周1次,连用4周)治疗31例难治性ITP患者.结果 10例获完全缓解(32.3％),13例获部分缓解(41.9％),5例微效(16.1％),3例无效(9.7％).均无明显不良反应.结论 利妥昔单抗可能是治疗难治性ITP安全、有效的药物.     

难治性免疫性血小板减少性紫癜不同治疗方法的疗效比较

目的:比较免疫性血小板减少性紫癜(ITP)不同治疗方法的疗效,探讨利妥昔单抗治疗难治性ITP的安全性和有效性.方法:125例ITP患者,分别应用糖皮质激素、丙球、达那唑、免疫抑制剂等治疗,其中5例难治性ITP患者应用利妥昔单抗375 mg·m-2,静脉输注,每周一次,连用4周.结果:ITP的一线治疗总反应率达70%.利妥昔单抗治疗的5例难治性ITP患者,2例获完全缓解(CR),1例获部分缓解(PR),1例微小反应(MR),1例没有反应.没有急性和迟发的毒性反应.结论:利妥昔单抗可能是治疗难治性ITP安全、有效的药物.     

小剂量利妥昔单抗治疗复发难治性原发免疫性血小板减少症的临床观察

目的 探讨小剂量利妥昔单抗治疗复发难治性原发免疫性血小板减少症(ITP)的疗效及安全性.方法 研究纳入20例复发难治性ITP患者,给予利妥昔单抗100 mg静脉滴注,每周1次,连用4周,动态观察血常规、肝肾功能及凝血功能.采用流式细胞术检测治疗前后CD3+、CD4+、CD8+、CD19+淋巴细胞数.免疫比浊法定量检测治疗前后血清免疫球蛋白(IgG、lgM、IgA)水平.用ELISA方法检测血小板膜糖蛋白抗体.治疗前后各项检测指标比较采用配对t检验.结果 治疗后中位起效时间为18d,PLT达峰值时间为(24±7)d.治疗后PLT[(124±106)×109/L]显著高于治疗前[(13±5)×109/L](P＜0.01).11例(55％)患者达完全反应(CR),4例(20％)有效(R),5例(25％)无效(NR).中位疗效持续时间为8(5～23)个月.治疗前后外周血WBC、HGB、血清免疫球蛋白以及CD3+、CD4+、CD8+淋巴细胞数无明显变化,CD19+淋巴细胞数治疗后[(50.53±29.11)×106/L]较治疗前[(125.65±14.12)×106/L]明显下降(P＜0.01).3例患者治疗前血小板自身抗体检测阳性,治疗后均为阴性.1例患者在首次输注利妥昔单抗后发生轻微不良反应.结论 小剂量利妥昔单抗是一种治疗复发难治性ITP安全有效的药物,但其最佳用药方案、长期疗效以及不良反应有待临床进一步观察验证.     

成年人原发免疫性血小板减少症的二线治疗方案选择

原发免疫性血小板减少症(ITP)为以血小板数目减少为特征的自身免疫性疾病.近年来,成年人ITP在发病机制、诊断、治疗等方面均获得重大进展,尤其是利妥昔单抗、血小板生成素(TPO)及血小板生成素受体激动剂(TPORA)在临床成年人ITP治疗中的广泛应用,使成年人ITP二线治疗方案的选择不再局限于脾切除术.笔者主要探讨成年人ITP患者二线治疗方案的选择,为采取一线治疗方案治疗失败的ITP患者提供个体化治疗方案.     

小剂量利妥昔单抗在特发性血小板减少性紫癜病人治疗中的观察及护理

目的探讨小剂量利妥昔单抗治疗特发性血小板减少性紫癜(ITP)的护理方法。方法采用小剂量利妥昔单抗治疗ITP患者31例,用药前30min给予预处理,及时准确用药,监测生命体征,观察并及时处理不良反应。结果 31例患者中完全缓解(CR)6例,有效(R)13例,无效(NR)12例,不良反应4例。结论小剂量利妥昔单抗是一种新的治疗方法,应用过程中密切观察、有效护理,对于减轻不良反应、提高疗效非常重要。     

小剂量利妥昔单抗治疗继发于结缔组织病的血小板减少症七例临床研究

目前,继发于结缔组织病(CTD)的血小板减少症被归人继发性免疫性血小板减少症(sITP)的范畴[1].相对于原发免疫性血小板减少症(ITP),继发于结缔组织病的免疫性血小板减少症(CTD-sITP)发病机制更为复杂[2],但两者的发病机制也存在一些共性,例如都存在血小板糖蛋白抗体以及T、B淋巴细胞的免疫耐受损伤[2-3].利妥昔单抗作为一种抗CD20的嵌合型单克隆抗体,具有B淋巴细胞清除作用.标准剂镀和小剂量利妥昔单抗治疗ITP疗效较好[4-7].     

利妥昔单抗治疗婴儿难治性自身免疫性溶血性贫血伴假性血小板减少一例报告附文献复习

自身免疫性溶血性贫血(AIHA)系体内B淋巴细胞免疫调节紊乱,产生自身抗体和(或)补体,并结合于红细胞膜上,致红细胞破坏加速而引起的一组溶血性贫血.婴儿难治性AIHA症状重,病程迁延,病死率高,治疗目标是迅速控制溶血[1-2].利妥昔单抗治疗成人原发免疫性血小板减少症(ITP)、AIHA及Evans综合征安全有效[3].但利妥昔单抗在婴儿患者应用少,既往仅国外有报道,且均为非前瞻性非随机对照的小样本研究,国内尚未见报道,其在婴儿患者中的最适剂量、有效性及安全性尚不明确.我们报告1例利妥昔单抗治疗成功的难治性AIHA伴假性血小板减少患儿,并进行文献复习.     

儿童遗传性心律失常的诊治进展

回顾在遗传性心律失常领域最新发表的相关研究,主要关注与儿童心源性猝死关系密切的离子通道病,包括长QT综合征（LQTS）、短QT综合征（SQTS）、Brugada综合征（BrS）和儿茶酚胺敏感性多形性室性心动过速（CPVT）,总结它们在发病机制及诊治方面的进展。     

The acoustic characteristics of the skull

Many investigators have stated that the difficulties of imaging with acoustical energy through the skull result from the marked attenuation of the energy by the skull. In the literature measurements of total attenuation have been confused with those for absorption.Measurements made by us show that absorption by compact bone varies between 2–3 dB cm^?1 MHz^?1 and, in the low megaHertz region appears to be directly proportional to frequency.It has also between shown that the convoluted inner surface of the ivory bone of the inner table of the skull may degrade the collimation and directionality of the beam by refraction.Cancellous bone, such as is present in the dipole of the skull, greatly attenuates the energy. It is postulated that this largely results from scattering. It is also postulated that the energy propagates through cancellous bone as two components, one in the soft tissues and the other partly in the bony spicules. Observations suggest that attenuation due to scattering much more markedly affects the latter of these components and scatters more greatly the higher frequencies in a pulse of broad bandwidth.The energy in each component has varying propagation paths so that the later cycles in the pulse of each component are subject to increasing interference as a result of the variations in propagation times. The two components moreover may have different propagation times so that interference may occur between the pulses of each component as well.All of these phenomena degrade the collimation, coherence, directionality, beam width, pulse length, frequency and other properties of the ultrasonic energy upon which imaging through the skull depends.The interference effects described above are least for the first cycle in the pulse which usually is not the cycle of highest amplitude. Since, in the free field, most of the energy is concentrated around the beam axis, most of the energy in the field which is deflected from its normal propagation path is deflected away from the beam axis. Thus the directionality of the beam is least degraded in the beam axis. The effects of the skull in degrading the properties of the ultrasonic pulse would therefore be lessened if the amplitude of the first cycle of the pulse and the directionality of its energy could be used for imaging.     

Organ Preservation: Current Concepts and New Strategies for the Next Decade

SUMMARY: Organ transplantation has developed over the past 50 years to reach the sophisticated and integrated clinical service of today through several advances in science. One of the most important of these has been the ability to apply organ preservation protocols to deliver donor organs of high quality, via a network of organ exchange to match the most suitable recipient patient to the best available organ, capable of rapid resumption of life-sustaining function in the recipient patient. This has only been possible by amassing a good understanding of the potential effects of hypoxic injury on donated organs, and how to prevent these by applying organ preservation. This review sets out the history of organ preservation, how applications of hypothermia have become central to the process, and what the current status is for the range of solid organs commonly transplanted. The science of organ preservation is constantly being updated with new knowledge and ideas, and the review also discusses what innovations are coming close to clinical reality to meet the growing demands for high quality organs in transplantation over the next few years.     

2017年呼吸疾病主要进展

2017年,国内外学者在呼吸系统疾病的临床和基础领域均进行了深入研究,不仅对相关指南进行了更新,并且针对一些临床热点、难点问题达成专家共识,现就2017年呼吸疾病相关进展作一简单介绍。     

家族性噬血细胞性淋巴组织细胞增生症1例并文献复习

目的加强对家族性噬血细胞性淋巴组织细胞增生症（familially hemophagocytic lymphohistiocytosis,FHL）的认识。方法报道确诊为FHL的新病例1例,结合国内外报道的FHL的病例,对该病的临床特点进行汇总分析。结果FHL2常与PRF1基因突变相关,约20%~40%的患者存在穿孔素基因突变。结论对于有阳性家族史,基因诊断明确,应尽早行化疗或者造血干细胞移植。若无家族史,未发现与继发性HLH相关的原发病因,可考虑行基因筛查以明确是否存在FHL的可能。     

COVID‐19 and COVID‐19 vaccine‐related dermatological reactions: An interesting case series with a narrative review of the potential critical and non‐critical mucocutaneous adverse effects related to virus,therapy, and the vaccination

This narrative review article was conducted to lay out a summarized but exhaustive review of current literature over mucocutaneous manifestations in 4 dimensions of SARS‐CoV‐2 pandemic: virus itself, treatment‐related, vaccine‐induced, and alteration of chronic dermatologic diseases following infection. Virus and vaccine‐related were mainly self‐limited and non‐severe. Treatment‐related reactions could be life‐threatening.     

Burkitt's lymphoma of maxillary gingiva: A case report

Burkitt's lymphoma(BL) is an aggressive form of nonHodgkin's B-cell lymphoma with three variants namely endemic, sporadic, and immunodeficiency-associated types. It is endemic in Africa and sporadic in other parts of the world. While the endemic form is widely reported to occur in early childhood and commonly involves the jaw bones, the sporadic form typically presents as an abdominal mass. This presentation reports a rare case of sporadic form of BL clinically manifesting as a generalized gingival enlargement in an immunocompetent adult male which demonstrated an aggressive behavior. The patient reported with a prominent anterior gingival swelling of 6 mo duration which slowly enlarged in size and associated with multiple lymph node involvement. Microscopic examination of the lesion using H, E and immunohistochemical diagnosis confirmed the diagnosis as BL. The patient succumbed to the disease before any therapy could be instituted. Since a wide array of causes can be attributed to gingival enlargements, it is necessary to consider malignancies as one of the important differential diagnosis so as to facilitate the need for appropriate diagnosis and prompt treatment.     

Real‐life effectiveness of biological therapies on symptoms in severe asthma with comorbid CRSwNP

BackgroundWe aimed to evaluate the effectiveness of different antibody therapies on nasal polyp symptoms in patients treated for severe asthma.MethodsWe performed a retrospective analysis of patients with severe asthma and comorbid CRSwNP who were treated with anti‐IgE, anti‐IL‐5/R or anti‐IL‐4R. CRSwNP symptom burden was evaluated before and after 6 months of therapy.ResultsFifty patients were included hereof treated with anti‐IgE: 9, anti‐IL‐5/R: 26 and anti‐IL‐4R: 15 patients. At baseline median SNOT‐20 was similar among groups (anti‐IgE: 55, anti‐IL‐5/R: 52 and anti‐IL‐4R: 56, p = 0.76), median visual analogue scale (VAS) for nasal symptoms was 4, 7 and 8 (p = 0.14) and VAS for total symptoms was higher in the anti‐IL‐4R group (4, 5 and 8, p = 0.002). After 6 months SNOT‐20 improved significantly in all patient groups with median improvement of anti‐IgE: −8 (p < 0.01), anti‐IL‐5/R: −13 (p < 0.001) and anti‐IL‐4R: −18 (p < 0.001), with larger improvement in the anti‐IL‐4R group than in anti‐IgE (p < 0.001) and anti‐IL‐5/R (p < 0.001) groups. VAS nasal symptoms improved by median anti‐IgE: 0 (n.s.), anti‐IL‐5/R: −1 (p < 0.01) and anti‐IL‐4R: −3 (p < 0.001), VAS total symptoms by anti‐IgE: −1 (n.s.), anti‐IL‐5/R: −2 (p < 0.001) and anti‐IL‐4R: −2 (p < 0.001).ConclusionsTreatment by all antibodies showed effectiveness in reducing symptoms of CRSwNP in patients with severe asthma, with the largest reduction observed in anti‐IL‐4R‐treated patients.     

经颅黑质超声诊断帕金森病的临床评价

目的 通过对帕金森病患者及正常对照组进行经颅超声(transcranial sonography,TCS)检查,结合帕金森病患者的Hoehn Yahr（H Y）分级及帕金森病统一评分量表评分结果,了解我国大陆地区帕金森病患者有无黑质(substantia nigra, SN)回声增强表现。方法 对入选对象进行TCS检查,并对检查结果进行评价。结果 帕金森病患者 SN阳性率明显高于正常对照者（P＜0.05）。帕金森病SN异常组 H Y 分期明显高于帕金森病SN正常组(P＜0.05),表明帕金森病患者SN高回声面积与H Y分期相关。TCS检查对帕金森病的敏感性为80.5%,特异性为79.9%。结论 我国帕金森病患者SN强回声检出率显著高于对照组,说明我国帕金森病患者也存在SN回声增强这一现象,与国内外报道相一致。TCS检查对帕金森病的诊断具有一定的意义,敏感性及特异性较高。