High dose gammaglobulin therapy in adults with idiopathic thrombocytopenic purpura (ITP) clinical effects

Summary This study of the effect of high-dose intravenous gammaglobulins with one or two courses of therapy in 18 adults with idiopathic thrombocytopenia purpura showed a platelet rise in thirteen patients. The highest response rates were seen in splenectomized adults. In chronic patients the response was transient only. If therapy was effective, increased values of platelet-associated IgG were reduced, while shortened platelet survival times were prolonged. There was no influence of high-dose gammaglobulins on platelet function. Different 7S-preparations such as -propiolactone modified Ig, pH 4 treated Ig and reduced and alkylated Ig have comparable effects.Supported by the Deutsche Forschungsgemeinschaft (Mu 277/9-4)     

静脉丙种球蛋白加地塞米松治疗小儿特发性血小板减少性紫癜临床观察及护理

目的探讨静脉丙种球蛋白(IVIG)加地塞米松治疗小儿特发性血小板减少性紫癜(ITP)的疗效及护理。方法对34例ITP患儿随机分为激素组和静脉丙种球蛋白加地塞米松组(简称丙球组)。结果丙球组出血症状改善迅速，血小板上升时间、峰值时间较激素组明显缩短，两组比较差异有显著意义。结论静脉丙种球蛋白加地塞米松治疗TIP疗效显著。     

Can high-dose immunoglobulin therapy be indicated in neonatal rhesus haemolysis?

A male neonate with hyperbilirubinaemia and progressing anaemia due to rhesus (c+E) incompatibility received high dose intravenous gammaglobulin (ivIgG) therapy at 13 days of age. Clinical response to therapy was confirmed by a rapid decrease in serum bilirubin level, and with the prevention of a further decline of the haemoglobin level. No side-effects were noted. High dose ivIgG therapy could be an alternative to (exchange) blood transfusion in case of some haemolytic diseases of newborn.     

静脉输注丙种球蛋白防治川崎病冠状动脉病变的疗效

目的评价静脉输注丙种球蛋白(IVIG)治疗和预防川崎病(KD)冠状动脉病变(CAL)的疗效,探讨IVIG疗效的影响因素。方法对314例KD患儿的临床资料进行回顾性对比观察。按治疗将患儿分为阿司匹林(ASA) IVIG组和ASA组,观察两组CAL发生、恢复情况、不同时机不同剂量IVIG治疗KD疗效、临床及实验室指标,急性期出现CAL者分别于病程1,3,6,12个月复查。结果ASA IVIG组CAL发生率34.3%,ASA组56.0%,两组比较P<0.001。应IVIG2.0g/kg或1.0g/kg以及在病程3～10d应用IVIG,CAL发牛率低,P<0.05。22.2?L发生在IVIG治疗后;13.4?L在病程12个月仍不能恢复正常,多数为IVIG治疗开始时间超过10d者。ASA IVIG组住院时间、退热时间、总热程缩短,血小板计数、血沉、C反应蛋白显著降低(P<0.05)。IVIG耐药病例占10.5%。结论IVIG治疗可显著缩短KD病程和降低CAL发生,但对川崎病CAL防治并非人们所预期的那样有效,实际疗效需要再评价。     

The Effect of Human Gammaglobulin Derivatives on Human Lymphoblastoid Cell Lines Persistently Infected with Herpes Simplex Virus

Human lymphoblastoid cells (NC-37) were infected with two strains of herpes simplex virus type 1 (HSV-1). Persistent infections with two strains (a freshly isolated strain, Seike strain, and Miyama strain) of HSV-1 were established in NC-37 cells. In NC-37 cells infected with HSV-1 (Seike), the growth of cells was inhibited, 6–72% of viable cells were positive for HSV-antigen by fluorescent antibody technique, and the percentage of HSV-antigen positive cells seemed to be inversely related to that of viable cells. Growth of cells and infectious viruses was seen for more than 396 days without external support. NC-37 cells infected with HSV-1 were subcultured with fresh medium containing human gammaglobulin derivatives. The percentage of HSV-antigen positive cells decreased and no infectious viruses were detected in the treated cells and cultured fluids after more than 16 days. It is thought that HSV continues to associate with T-lymphocytes stimulated in vivo for a long period of time after the appearance of circulating antibody, at least for two weeks, and lymphocytes persistently infect with HSV have a relation to the patho-genesis of herpesvirus encephalitis in oider children and adults similar to the pathogenesis of SSPE. (Acta Paediatr Jpn 23(2): 201–207 1981)     

Intravenous immunoglobulin in the treatment of human immunodeficiency virus-related thrombocytopenia.

Fourteen patients with sexually transmitted human immunodeficiency virus (HIV)-related immune thrombocytopenia were treated with intravenous gammaglobulin (IVIG). The patients were treated with a uniform program consisting of 1 g/kg of IVIG on day 1 and day 2, followed by 1 g/kg on day 15. Most patients had pretreatment bleeding symptoms, which included petechiae, spontaneous and traumatic ecchymoses, gum bleeding, and epistaxis. Median baseline platelet count was 17,000/mm3 (range 3-61,000/mm3). After the infusion of the IGIV, all patients had a resolution of their bleeding by day 8. The median maximum platelet count achieved with the IGIV was 220,000/mm3 (range 76-426,000/mm3). No patient achieved either a sustained complete or partial remission after the conclusion of the IVIG therapy. Toxicities were minimal with the majority being headache and nausea. In conclusion, patients with sexually transmitted HIV infection and immune thrombocytopenia respond favorably to IVIG. This treatment should be considered as first-line therapy for patients with HIV-related immune thrombocytopenia who require immediate but temporary increase in their platelet count, attributable to symptoms or signs of clinical bleeding or because of the need for an invasive procedure.     

The use of intravenous gammaglobulin in the treatment of typical hemolytic uremic syndrome

Nine children with acute typical post-diarrhea hemolytic uremic syndrome (HUS) were treated with intravenous gammaglobulin (IVIG). These children were compared to nine children with HUS who did not receive IVIG. The use of IVIG did not appear to have a beneficial effect on eight of the nine treated children. There were no significant differences found in the duration of hemorrhagic colitis, thrombocytopenia, elevation of the white blood count (WBC), anuria, dialysis, or hospitalization, or the presence of a central nervous system complication or pancreatitis. Although no significant difference was found in the duration of thrombocytopenia, there was a trend towards a longer duration of thrombocytopenia in children treated with IVIG (P=0.13). One child demonstrated both an increase in her platelet count and a decrease in her WBC count within 24 h of receiving her first dose of IVIG.     

120例川崎病临床分析

目的 探讨川崎病(KD)的临床特征、实验室检查、冠脉损害的危险因素及治疗方法.方法 对本院儿科2000年8月至2008年10月诊治的120例KD患儿资料进行回顾性分析和总结.结果 120例KD中典型99例,不典型21例,男83例,女37例.平均发病年龄2岁7个月.合并冠脉损害者32例,其中冠脉扩张23例,冠脉瘤9例.对合并冠脉损害者随访6个月至3年,完全恢复正常者29例.结论 不典型KD冠脉损害率高,KD发病10d内用丙种球蛋白效果好,2g/kg单次注射明显优于0.4g·kg-1·d-1连用5d的疗法,WBC升高,ALB降低,男性及发热时间长,是KD合并冠脉损害的危险因素.     

中西医结合治疗小儿急性病毒性脑炎临床观察

目的 探讨治疗急性病毒性脑炎的有效，安全的治疗方法。方法 采用广羚散，丙种球蛋白联合应用，观察小儿急性病毒性脑炎76例疗程及治疗效果，并与对照组50例比较。结果 治疗组在疗程及疗效方面明显优于对照组（P＜0．01），未见明显副作用。结论：广羚散，丙种球蛋白联合治疗小儿急性病毒性脑炎是一种安全有效疗程短的方法，能否减少后遗症的发生有待进一步观察。     

Diagnosis and treatment of primary Sjögren syndrome-associated peripheral neuropathy: a six-case series

Objectives

The clinical and therapeutic aspects of primary Sjögren syndrome (PSS) in patients with peripheral neuropathy were analyzed and the specifics of individual case studies are discussed.

Methods

We retrospectively studied six patients (four women, two men; mean age 64.5 years) presenting with PSS with peripheral neurological involvement over a five-year period (2008–2012). All patients had neurological examinations, including nerve conduction studies, somatosensory evoked potentials, and sural nerve biopsies. Treatment regimens included corticosteroids, intravenous gammaglobulin, or immunosuppressive treatment.

Results

Peripheral neuropathy was observed in six (7.9 %) of 76 patients with SS as the underlying disease; three were cases of multiple mononeuropathy, two cases had sensory ataxic neuropathy, one of which was autonomic neuropathy, and one case was diagnosed as painful sensory neuropathy without sensory ataxia. Four of the six patients were diagnosed with SS after the onset of neurological symptoms. Individual peripheral neuropathies had distinct neurological, electrophysiological, and pathological characteristics. The effect of steroids and intravenous gammaglobulin differed depending on the case.

Conclusions

In PSS patients, a precise diagnosis is important, because the therapeutic strategy and response varies depending on the type of neuropathy. In clinical practice, it is important to consider a diagnosis of SS when patients present with peripheral neuropathy.