应用丙种球蛋白结合脾切除治疗难治性血小板减少性紫癜

目的　评价静脉输注大剂量丙种球蛋白（ＩＶＩＧ）结合脾切除术治疗难治性血小板减少性紫癜的疗 效。方法　４３例均为对糖皮质激素耐药、依赖、慎用的难治性ＩＴＰ患者,术前给予丙种球蛋白４００ｍｇ／ｋｇ·ｄ,连用５ 天,用药后１０ｄ内血小板升至５０×１０９／Ｌ以上者实施脾切除术。个别病例血小板未达到此数值,术前１～２ｈ 输机采 血小板１～２ｕ,然后进行手术。结果应用ＩＶＩＧ后,３４例（７９％）血小板升至５０×１０９／Ｌ以上,其余病例血小板亦有 不同程度上升。脾切除近期疗效（术后２个月～１年）：４３例均达临床缓解,其中１３例复发,经用激素治疗后,１２例 再次达到临床缓解,１例无效。临床缓解率９７．７％。脾切除远期疗效（术后１年以上）：观察１年以上有３５例,均达 到临床缓解。其中基本治愈１８例（５１．４％）,７例复发,６例经过激素治疗再次达到临床缓解,１例无效。临床缓解率 ９７．１％。结论　静脉输往大剂量丙种球蛋白是一个有效的术前提升血小板的治疗方法,而脾切除术可使大部分难治性ＩＴＰ患者达到临床缓解。     

静脉滴注丙种球蛋白治疗小儿原发性血小板减少性紫癜的远期疗效

作者对3种不同疗法治疗小儿ITP效果进行了远期随防,结果IVIg疗效最佳,血小板数升高快,血小板抗体消失决,血清IgG水平稳定,复发率低。而其它2组不如前者。提示IVIg是目前治疗小儿ITP疗效最佳的新方法。     

慢性特发性血小板减少性紫癜发病机制的探讨

目的探讨巨核细胞负调控因子PF4和TGF-β1在CITP患者血浆中的变化,为难治性CITP的发病及治疗提供有价值的依据。方法用ELISA试剂盒检测待测血浆中PF4和TGF-β1水平,用光学显微镜分类计数标准化骨髓涂片巨核细胞。结果CITP未正规治疗组及复发难治组外周血浆中PF4和TGF-β1水平显著高于正常对照组(P<0.05);缓解组治疗后PF4和TGF-β1水平较治疗前显著下降(P<0.05),接近正常对照组;未缓解组治疗前后无明显变化(P>0.05);CITP患者治疗前骨髓涂片均以颗粒巨核细胞为主伴产板障碍;治疗后缓解组患者骨髓涂片以产板巨核细胞为主。结论巨核细胞负向调控因子如PF4及TGF-β1可能参与CITP的发病。     

静脉滴注丙种球蛋白治疗小儿原发性血小板减少性紫瘢的 …

作者对３种不同疗法治疗小儿ＩＴＰ效果进行了远期随访,结果ＩＶＩａ疗效最佳,血小板数升高快,血小板抗体消失快,血清ＩｇＧ水平稳定,复发率低。而其它２组不如前者。提示ＩＶＩｇ是目前治疗小儿ＩＴＰ疗效最佳的新方法。     

1例新生儿血小板减少性紫癜的免疫抗体分析

目的分析1例新生儿血小板减少性紫癜(NAIT)血清中免疫抗体的特异性。方法采用固相凝集法检测患儿及其母亲血清中是否存在抗父亲/抗丈夫血小板抗体,用PAK12试剂盒鉴定血清中的抗体是抗-HLA还是抗-HPA,用ELISA法(PRA)鉴定HLA抗体的特异性,用SSOP法进行父亲、母亲和患儿HLA-Ⅰ类基因分型,用PCR-SSP法进行父亲、母亲和患儿HPA抗原1-5系统基因分型。结果该例NAIT患儿血清中存在来自母体的HLA-Ⅰ类抗体和HPA抗体;HLA-Ⅰ类抗体为抗-Bw6;父亲和患儿的HLA-B位点的宽特异性均为Bw4,Bw6,母亲为Bw4;患儿HPA抗原1-5系统基因型与父亲相同,有3a,母亲没有3a。结论在对NAIT患儿进行实验室诊断时,除注意HPA抗体的作用外,也应重视HLA-Ⅰ类抗体的作用。     

血栓性血小板减少性紫癜患者的临床观察及护理

本文对血栓性血小板减少性紫癜患者临床观察及护理体会进行了回顾和系统的总结。认为在该病的治疗过程中,严密观察病情协助诊治,做好临床护理和血浆置换术护理工作的同时,加强安全防护、做好心理护理是保证治疗效果促进患者康复的重要措施。     

虹口区预防接种后血小板减少性紫癜4例案例分析

目的通过4例预防接种后发生的血小板减少性紫癜(idiapathic thrombocytopenit purpura,ITP)报告病例分析疫苗相关的ITP诊断标准,探讨降低ITP偶合症的相关措施。方法查阅虹口区2010~2015年疑似预防接种异常反应(adverse events following immunization,AEFI)信息管理系统、个案调查表及异常反应调查诊断资料,采用描述性方法进行分析。结果预防接种后发生4例ITP病例,经虹口区预防接种异常反应调查诊断专家组诊断,2例与疫苗接种相关,2例为偶合症。结论应对医生和家长加强预防接种后不良反应的宣传培训,发现异常及时就诊;疫苗相关的ITP诊断标准建议统一;对首次于门诊接种第2剂乙肝疫苗的儿童,医生应注意问询儿童母亲孕期健康情况和儿童有无先天畸形、感染等,以尽可能减少ITP偶合症。     

CD4+CD25+调节性细胞在特发性血小板减少性紫癜中发病机制中的作用

CD4+CD25+调节性T细胞(regulatory Tcell,Treg)是一群具有独特的免疫调节或免疫抑制作用的T细胞亚群,系维持机体自身耐受的重要组成部分,其功能紊乱或数目下降是导致自身免疫性疾病的重要原因之一。本文对CD4+CD25+Treg细胞的特征和作用、ITP的发病机制及CD4+CD25+Treg细胞在ITP发病中的作用等的研究进展作一综述。     

百、白、破三联与脊髓灰质炎糖丸联合免疫致血小板减少性紫癜一例

<正> 我们在开展儿童计划免疫门诊工作中,遇到1例由于同时接种两种疫苗而引起的不良反应,现报告如下。黄某,男,5个月,汉族。于1987年10月26日在右上臂外缘三角肌附着处,皮下注射百白破疫苗0.5ml(卫生部上海生物制品研究所制造,批号861254—4,失效期1988年6月),同时口服脊髓灰质炎活疫苗三价糖丸(简称脊灰糖丸)1粒(中国医学科学院医学生物学研究所制造,批号86—147～1,失效期1988年11月)。5个小时以后,患儿脸面出现粟粒样大小出血点,呈紫红色。24小时后,四技、胸腹背部相继出现,但不发热。48小时后去     

血小板输注无效患者的供者筛选

目的探讨解决血小板输注无效的途径,改善血小板输注效果。方法对12例血小板输注无效患者先进行HLA抗体与HPA抗体检测,然后分别在血小板供者库中找到HLA-I类位点3个以上抗原相合的供者,进行血小板交叉配合试验。结果 12例PTR患者检出HLA抗体阳性共8例,阳性率为66.7%,HPA抗体阳性1例,阳性率为8.3%,HLA抗体合并HPA抗体阳性共3例,阳性率为25.0%。对8例仅有HLA抗体的患者选择HLA-I类位点3个以上抗原相合的供者,其血小板输注有效率为87.5%,对1例有HPA抗体的患者采用血小板交叉配合试验,其血小板输注有效率33.3%。对3例有HLA与HPA联合抗体的患者,选择HLA-I类位点3个以上抗原相合的供者,进行血小板交叉配合试验,其血小板输注有效率为66.7%。结论对PTR患者进行抗体筛查,选择HLA相合的供血者及血小板交叉配合试验十分重要,是解决PTR的有效途径。     

静脉滴注丙种球蛋白治疗病毒性脑炎的临床研究

应用静注丙种球蛋白（IVIg）治疗病毒性脑炎（病脑）,同时应用血浆治疗作为对照,观察结果表明症状、体征和实验室检测结果,观察组疗效均明显优于对照组。提示IVIg治疗病脑好转快,疗效好,病死率低,后遗症少。     

重症肌无力患儿免疫状态及静注免疫球蛋白的疗效

目的观察重症肌无力（ＭＧ）患儿机体免疫状态,比较静脉注射免疫球蛋白（ＩＶＩｇ）与免疫调节疗法 对ＭＧ的疗效。方法对８３例ＭＧ患儿分别于治疗前和缓解后,采用单项免疫扩散法检测血清免疫球蛋白（Ｉｇ）,用 ＥＩＪＳＡ法检测乙酸胆碱受体抗体（ＡｃｈＲａｂ）和可溶性白细胞介素－２受体（ＳＩＬ－２Ｒ）,以２４名健康儿童为对照组;并 对上述患儿中的６８例采用免疫调节疗法,２５例采用ＩＶＩｇ疗法。结果初发和复发的ＭＧ患儿ＩｇＧ、ＡｃｈＲａｂ、ＳＩＬ－２Ｒ 明显高于对照组（Ｐ＜０．００１）,缓解后与对照组差异无显著意义（Ｐ＞０．０５）;ＩＶＩｇ治疗组和免疫调节治组疗缓解率均 为９２％,复发率ＩＶＩｇ组为８％,免疫调节组为１８％,两组比较差异有显著意义（Ｐ＜０．０５）。结论ＩｇＧ、ＡｃｈＲａｂ、ＳＩＬ－ ２Ｒ随ＭＧ的病情而变化,可作为预测ＭＧ病情的依据;ＩＶＩｇ疗法在降低复发率方面优于免疫调节疗法。     

Novel Pyrimidines as Multitarget Protein Tyrosine Kinase Inhibitors for the Treatment of Idiopathic Pulmonary Fibrosis (IPF)

A new class of pyrimidine derivatives were identified as potent protein tyrosine kinase (PTK) inhibitors for the treatment of idiopathic pulmonary fibrosis (IPF). Most of these small-molecule inhibitors displayed strong enzymatic activity against BTK and JAK3 kinases at concentrations lower than 10 nM. The representative compound N-(3-((5-chloro-2-(4-((1-morpholino)acetylamino)phenylamino)-4-pyrimidinyl)amino)phenyl)acrylamide ( 6 a ) also exhibited high inhibitory potency toward both BTK and JAK kinase families, as well as ErbB4, at a concentration of 10 nM, achieving rates of inhibition higher than 57 %. Additionally, in vivo biological evaluations showed that 6 a can remarkably decrease the severity of IPF disease. All these investigations suggested that the multi-PTK inhibitor 6 a may serve as a promising agent for the treatment of IPF.     

Assessment of Brain-Derived Neurotrophic Factor (BDNF) Concentration in Children with Idiopathic Nephrotic Syndrome

Idiopathic nephrotic syndrome (INS) is a chronic disease affecting children in early childhood. It is characterized by proteinuria, hypoalbuminemia, edema and hyperlipidemia. To date, the diagnosis is usually established at an advanced stage of proteinuria. Therefore, new methods of early INS detection are desired. This study was designed to assess brain-derived neurotrophic factor (BDNF) as a potential marker in the early diagnosis of INS. The study group included patients with a diagnosis of idiopathic nephrotic syndrome (n = 30) hospitalized in Clinical Hospital No. 1 in Zabrze, from December 2019 to December 2021. Our study shows that serum BDNF concentration decreased and urine BDNF concentration increased in a group of patients with INS, compared with healthy controls. Such outcomes might be related to loss of the BDNF contribution in podocyte structure maintenance. Moreover, we anticipate the role of BDNF in urine protein concentration increase, which could be used as a direct predictor of urine protein fluctuations in clinical practice. Moreover, the ROC curve has also shown that serum BDNF and urine BDNF levels might be useful as an INS marker.     

Novel Insight in Idiopathic Normal Pressure Hydrocephalus (iNPH) Biomarker Discovery in CSF

Idiopathic normal pressure hydrocephalus (iNPH) is a potentially reversible neurological disease, causing motor and cognitive dysfunction and dementia. iNPH and Alzheimer’s disease (AD) share similar molecular characteristics, including amyloid deposition, t-tau and p-tau dysregulation; however, the disease is under-diagnosed and under-treated. The aim was to identify a panel of sphingolipids and proteins in CSF to diagnose iNPH at onset compared to aged subjects with cognitive integrity (C) and AD patients by adopting multiple reaction monitoring mass spectrometry (MRM-MS) for sphingolipid quantitative assessment and advanced high-resolution liquid chromatography–tandem mass spectrometry (LC–MS/MS) for proteomic analysis. The results indicated that iNPH are characterized by an increase in very long chains Cer C22:0, Cer C24:0 and Cer C24:1 and of acute-phase proteins, immunoglobulins and complement component fragments. Proteins involved in synaptic signaling, axogenesis, including BACE1, APP, SEZ6L and SEZ6L2; secretory proteins (CHGA, SCG3 and VGF); glycosylation proteins (POMGNT1 and DAG1); and proteins involved in lipid metabolism (APOH and LCAT) were statistically lower in iNPH. In conclusion, at the disease onset, several factors contribute to maintaining cell homeostasis, and the protective role of very long chains sphingolipids counteract overexpression of amyloidogenic and neurotoxic proteins. Monitoring specific very long chain Cers will improve the early diagnosis and can promote patient follow-up.     

The High Temperature Treatment of Trinitrotoluene (TNT) and Cyclotrimethylene-Trinitramine (RDX) with Ozone and Ultrasound

This paper summarizes the results of research on the treatment of synthetically prepared aqueous solutions of trinitrotoluene (TNT) and cyclotrimethylene–trinitramine (RDX) in the weight ratio 70%/30% representing a typical munitions wastewater, by a combination of ozone and ultrasound. A parametric study investigated the relationship among the variables: (1) initial solution pH (5.84 → 10.0); (2) ultrasound power level (5 → 50 watts at 852.0 to 863.0 kHz);(3) ultrasound frequency level (60.6 → 1,007.0 kHz); (4) solution concentration (70/30 mg/l TNT/RDX solutions volumetrically diluted with distilled water in the ratios 1/0, 1/1, 1/3); (5) reaction temperature (25 → 59°C). Removal rates of both TNT and TOC increased directly with increases in reaction temperature and initial solution pH. Likewise, increased sound power level produced enhanced system kinetic responses; however, these were attributed to reaction mass temperature increases. Ultrasound was found to inhibit reaction kinetics at high temperatures and pH because it promoted radical–radical extinguishment reaction.     

静注甲型H1N1流感人免疫球蛋白的研制

目的研制高效价静注甲型H1N1流感人免疫球蛋白。方法用甲型H1N1流感疫苗(简称甲流)对固定供血浆者按疫苗说明书免疫后2周开始采集原料血浆,采用血凝抑制法检测原料血浆和制品的甲流抗体效价;采用柱层析法从高效价甲流免疫血浆中提取富含IgM免疫球蛋白的样品,模拟低温乙醇蛋白分离法工艺从小量混合血浆分离IgG样品,测定血浆和提取样品的甲流抗体效价,分析甲流中和抗体效价与IgG、IgM含量的对应关系,判断甲流中和抗体的免疫球蛋白类型;按本公司静脉注射人免疫球蛋白(Intravenous immunoglobulin,IVIG)生产工艺制备静注甲型H1N1流感人免疫球蛋白,并与普通IVIG及相应合并血浆的甲流抗体效价进行比较。结果筛选到甲流抗体效价≥320 HU/ml的合格血浆9 866袋,合格率达33.5%,其中抗体效价≥640 HU/ml的血浆占合格血浆的49%,血浆质量符合《中国药典》三部(2010版)"血液制品生产用人血浆规程"要求;甲流抗体效价与IgG含量呈相应比例关系,而与IgM含量无关,甲流中和抗体的免疫球蛋白类型以IgG为主;制备的甲流人免疫球蛋白制品的甲流中和抗体效价达2 560 HU/ml,为普通IVIG的197倍,其他质量指标符合《中国药典》三部(2010版)"静注人免疫球蛋白制检规程"要求。结论成功研制了静注甲型H1N1流感人免疫球蛋白,在甲流疫情得到有效控制的情况下,仍具有战略储备意义。