静脉输注免疫球蛋白治疗特发性血小板减少性紫癜

大剂量静脉输注免疫球蛋白（IVIg）治疗特发性血小板减少性紫癜（ITP）20例，15天之内100％病例血小板升至5×1010／L以上．70％病例血小板升至1×1011／L以上，与激素治疗组相比，具有血小板升速快，幅度大的优点。经激素治疗无效的患者，用大剂量IVIg治疗亦有效，使用中未发现毒副作用。     

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。     

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。     

静注人免疫球蛋白在治疗阿尔茨海默病中的应用

阿尔茨海默病(Alzheimer disease,AD)是最常见的神经退行性疾病。AD已经造成了严重的社会负担,而且目前仍无有效的方法可以治疗。近年来,使用静注人免疫球蛋白(intravenous immunoglobulin,IVIg)治疗AD被认为是一种有潜力的疗法。本文对AD、Aβ级联假说、IVIg治疗AD的作用机制以及临床试验等作一综述,并对未来IVIg治疗AD的研究进行了展望。     

妊娠合并血小板减少97例临床分析

目的探讨妊娠合并血小板减少(PT)病因及治疗预后。方法对97例妊娠合并PT患者的临床资料进行回溯性分析。结果单纯由妊娠引起49例(50.52%),重度妊高征引起31例(31.96%),特发性血小板减少性紫癜(ITP)引起13例(13.40%),合并肝功能异常4例(4.12%)。对血小板计数<50×109/L者在分娩前后短期使用肾上腺皮质激素、人免疫球蛋白(HD-IVIG)及血小板制剂,分娩方式应由产科指征决定。产后出血率为12.26%,产后出血量与血小板计数成负相关,未发现新生儿出血。结论除了针对病因治疗外,积极对症治疗亦是必需手段。     

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。     

大剂量丙种球蛋白治疗自身免疫性溶血性贫血的疗效

采用大剂量丙种球蛋白 (IVIg)对 2 8例自身免疫性溶血性贫血 (AIHA)患儿进行了临床疗效观察 ,结果在改善症状、体征及实验室检测指标等方面均明显优于对照组 ,且无复发病例     

36例婴儿肛周脓肿次性根治临床总结

目的研究一次性根治术治疗小儿肛周脓肿可行性及疗效。方法对36例一次性根治术治疗小儿肛周脓肿,观察其疗效。结果36例患儿均一次治愈,半年随访无复发,无肛门功能异常,无后遗症。结论一次性根治术治疗小儿肛周脓肿是一种很好的方法。     

赖氨酸肌醇维生素B12口服液治疗小儿厌食症的临床观察

目的评价赖氨酸肌醇维生素B12口服液治疗小儿厌食症的疗效。方法选择厌食症患儿60例,随机分为治疗组30例,用赖氨酸肌醇维生素B12治疗小儿厌食;对照组30例,只采用健康处方。临床观察并近期随访小儿食欲、体重增长以及微量元素变化情况。结果治疗组近1个月小儿在食欲、体重增长情况以及血中微量元素明显高于对照组,经统计学检验存在显著性差异(P<0.05)。结论赖氨酸肌醇维生素B12治疗小儿厌食症疗效明显。     

中西医结合治疗小儿肠系膜淋巴结炎193例分析

目的肠系膜淋巴结炎是小儿常见急腹症之一,本研究通过采用中西医结合治疗取得较好效果。本文对中西医结合治疗小儿急性肠系膜淋巴结炎临床疗效做相关探讨。方法将小儿急性肠系膜淋巴结炎患者193例随机分为中西医联合组和西药对照组,均给予头孢哌酮钠舒巴坦钠50mg/(kg.d)静脉滴注,联合组同时加用保和丸,疗程1周。注意观察临床症状和退热情况对疗效进行判断。结果经过比较,2组疗效差异显著,P<0.05。结论中西医联合治疗小儿急性肠系膜淋巴结炎疗效优于西医单独治疗,可在临床广泛推广。     

Calpain activator dibucaine induces platelet apoptosis

Calcium-dependent calpains are a family of cysteine proteases that have been demonstrated to play key roles in both platelet glycoprotein Ibα shedding and platelet activation and altered calpain activity is associated with thrombotic thrombocytopenic purpura. Calpain activators induce apoptosis in several types of nucleated cells. However, it is not clear whether calpain activators induce platelet apoptosis. Here we show that the calpain activator dibucaine induced several platelet apoptotic events including depolarization of the mitochondrial inner transmembrane potential, up-regulation of Bax and Bak, down-regulation of Bcl-2 and Bcl-X(L), caspase-3 activation and phosphatidylserine exposure. Platelet apoptosis elicited by dibucaine was not affected by the broad spectrum metalloproteinase inhibitor GM6001. Furthermore, dibucaine did not induce platelet activation as detected by P-selectin expression and PAC-1 binding. However, platelet aggregation induced by ristocetin or α-thrombin, platelet adhesion and spreading on von Willebrand factor were significantly inhibited in platelets treated with dibucaine. Taken together, these data indicate that dibucaine induces platelet apoptosis and platelet dysfunction.     

Primary Immune Thrombocytopenia and Essential Thrombocythemia: So Different and yet Somehow Similar—Cases Series and a Review of the Literature

This article collects several published cases in which immune thrombocytopenic purpura (ITP) is followed by essential thrombocythemia (ET) and vice versa. This surprising clinical condition is possible, but very rare and difficult to diagnose and manage. We have made an attempt to analyse the possible causes of the sequential appearance of ITP and ET taking into consideration the following: alteration of the thrombopoietin (TPO) receptor, the role of autoimmunity and inflammation, and cytokine modulation. A better understanding of these interactions may provide opportunities to determine predisposing factors and aid in finding new treatment modalities both for ITP and ET patients.     

国内止血药酚磺乙胺的合成工艺概述

酚磺乙胺是通过促进凝血过程而发挥作用。临床上用于预防和治疗外科手术出血过多，血小板减少性紫癜或过敏性紫癜以及其它原因引起的出血。本品是一种合成止血药，可与其他类型止血药合用。     

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。     

Platelet-activating factor type activity in plasma from patients with septicemia and other diseases

The purpose of the present study was to determine whether increased levels of platelet-activating factor (PAF) type activity can be detected in plasma from patients with septicemia and other diseases. A level of PAF below 0.5 ng/mL of plasma was considered normal. We found that plasma from a patient with adverse anaphylactoidic reaction to intravenous analgetics contained 2.1 ng PAF/mL. In seven patients with septicemia, including urosepsis, endocarditis and peritonitis, and with positive blood culture, increased plasma PAF levels (1–20 ng PAF/mL) were observed. Other patients with clinical indications of septicemia had negative blood cultures and/or increased levels of C-reactive protein (CRP). Yet, in the plasma from these patients, no increased PAF levels were detected under the assay conditions used. Two patients with allergic asthma, requiring treatment with steroids, had no measurable plasma PAF. In the plasma from a patient with idiopathic thrombocytopenic purpura (ITP) only an “endogenous” inhibitor of PAF induced platelet aggregation was initially observed. In spite of this, the patient responded to treatment with the PAF antagonist WEB 2086 with a dramatic increase in platelet count (Lohmannet al., Lancet ii, 1147, 1988). Thereafter, also increased PAF levels (3.3 ng PAF/mL) were detected in plasma, although some “endogenous” inhibitor of PAF was still present. In conclusion, increased PAF levels in plasma from patients support a role of PAF in certain human disease states, such as in anaphylactoid reaction, sepsis and septic shock. The type, relevance and specificity of endogenous inhibitors of PAF deserve further study. Based on a paper presented at the Third International Conference on Platelet-Activating Factor and Structurally Related Alkyl Ether Lipids, Tokyo, Japan, May 1989.     

Placental Complement Activation in Fetal and Neonatal Alloimmune Thrombocytopenia: An Observational Study

Fetal and neonatal alloimmune thrombocytopenia (FNAIT) is a disease that causes thrombocytopenia and a risk of bleeding in the (unborn) child that result from maternal alloantibodies directed against fetal, paternally inherited, human platelet antigens (HPA). It is hypothesized that these alloantibodies can also bind to the placenta, causing placental damage. This study aims to explore signs of antibody-mediated placental damage in FNAIT. We performed a retrospective study that included pregnant women, their newborns, and placentas. It comprised 23 FNAIT cases, of which nine were newly diagnosed (14 samples) and 14 were antenatally treated with intravenous immune globulins (IVIg) (21 samples), and 20 controls, of which 10 had anti-HLA-class I antibodies. Clinical information was collected from medical records. Placental samples were stained for complement activation markers (C1q, C4d, SC5b-9, and mannose-binding lectin) using immunohistochemistry. Histopathology was examined according to the Amsterdam criteria. A higher degree of C4d deposition was present in the newly diagnosed FNAIT cases (10/14 samples), as compared to the IVIg-treated FNAIT cases (2/21 samples, p = 0.002) and anti-HLA-negative controls (3/20 samples, p = 0.006). A histopathological examination showed delayed maturation in four (44%) placentas in the newly diagnosed FNAIT cases, five (36%) in the IVIg-treated FNAIT cases, and one in the controls (NS). C4d deposition at the syncytiotrophoblast was present in combination with low-grade villitis of unknown etiology in three newly diagnosed FNAIT cases that were born SGA. We conclude that a higher degree of classical pathway-induced complement activation is present in placentas from pregnancies with untreated FNAIT. This may affect placental function and fetal growth.     

Efficacy of Polyvalent Human Immunoglobulins in an Animal Model of Neuromyelitis Optica Evoked by Intrathecal Anti-Aquaporin 4 Antibodies

Neuromyelitis Optica Spectrum Disorders (NMOSD) are associated with autoantibodies (ABs) targeting the astrocytic aquaporin-4 water channels (AQP4-ABs). These ABs have a direct pathogenic role by initiating a variety of immunological and inflammatory processes in the course of disease. In a recently-established animal model, chronic intrathecal passive-transfer of immunoglobulin G from NMOSD patients (NMO-IgG), or of recombinant human AQP4-ABs (rAB-AQP4), provided evidence for complementary and immune-cell independent effects of AQP4-ABs. Utilizing this animal model, we here tested the effects of systemically and intrathecally applied pooled human immunoglobulins (IVIg) using a preventive and a therapeutic paradigm. In NMO-IgG animals, prophylactic application of systemic IVIg led to a reduced median disease score of 2.4 on a 0–10 scale, in comparison to 4.1 with sham treatment. Therapeutic IVIg, applied systemically after the 10th intrathecal NMO-IgG injection, significantly reduced the disease score by 0.8. Intrathecal IVIg application induced a beneficial effect in animals with NMO-IgG (median score IVIg 1.6 vs. sham 3.7) or with rAB-AQP4 (median score IVIg 2.0 vs. sham 3.7). We here provide evidence that treatment with IVIg ameliorates disease symptoms in this passive-transfer model, in analogy to former studies investigating passive-transfer animal models of other antibody-mediated disorders.