Dwyer J M, Erlendsson K
Department of Clinical Immunology, Yale University School of Medicine, New Haven, CT.
Pediatr Infect Dis J. 1988 May;7(5 Suppl):S30-3.
Although bacterial infections predominate in patients with hypogammaglobulinemia, patients who do not produce normal amounts of immunoglobulin also have an increased incidence of viral infections. This is particularly true of infections with enteroviruses. Echovirus encephalitis has been a major problem for patients with hypogammaglobulinemia. Neurologic damage, frequently resulting in death, has been common in such patients. Because there is an obligatory extracellular phase in the cell to cell spread of enteroviruses, therapy with immunoglobulin has been attempted. In certain patients intravenous and intrathecal gammaglobulin has temporarily halted progression of the disease, but no patients have been cured by this approach. In this report we detail treatment of three children with X-linked hypogammaglobulinemia who had encephalitis caused by echovirus infections. Despite doses of intravenous immunoglobulin that maintained the patients' IgG levels within the normal range, their condition deteriorated in all cases. Treatment with intraventricular immunoglobulin was then tried. In all three cases cerebrospinal fluid protein levels and cell counts returned to normal after this treatment and the echoviruses can no longer be isolated from the cerebrospinal fluid. Follow-up time has ranged from 18 months to 4 years. Ommaya reservoirs were placed into the lateral ventricle of each patient and concentrated (6%) immunoglobulin (Sandoglobulin) was injected into the reservoir on a daily basis. On Days 1 through 7 of the regimen patients were given 120, 300, 450, 510, 540 and 600 mg of IgG, respectively. Patients then received 300 mg daily for periods ranging from 1 week to 1 month. Cultures of cerebrospinal fluid removed from the reservoir were repeatedly analyzed to determine the need for further treatment. Clinically the patients improved markedly.(ABSTRACT TRUNCATED AT 250 WORDS)
尽管低丙种球蛋白血症患者中细菌感染占主导,但免疫球蛋白产生量不正常的患者病毒感染发生率也会增加。肠道病毒感染尤其如此。埃可病毒脑炎一直是低丙种球蛋白血症患者的主要问题。神经系统损害,常导致死亡,在这类患者中很常见。由于肠道病毒在细胞间传播存在一个必需的细胞外阶段,因此尝试用免疫球蛋白进行治疗。在某些患者中,静脉注射和鞘内注射丙种球蛋白暂时阻止了疾病进展,但尚无患者通过这种方法治愈。在本报告中,我们详细介绍了三名患有X连锁低丙种球蛋白血症且因埃可病毒感染导致脑炎的儿童的治疗情况。尽管静脉注射免疫球蛋白的剂量使患者的IgG水平维持在正常范围内,但所有病例中患者的病情仍恶化。随后尝试了脑室内注射免疫球蛋白治疗。在所有三例病例中,治疗后脑脊液蛋白水平和细胞计数恢复正常,且脑脊液中不再能分离出埃可病毒。随访时间为18个月至4年。在每位患者的侧脑室置入奥马亚贮液器,每天向贮液器中注入浓缩(6%)免疫球蛋白(桑球蛋白)。在治疗方案的第1至7天,患者分别接受120、300、450、510、540和600毫克的IgG。患者随后每天接受300毫克,持续1周至1个月。反复分析从贮液器中取出的脑脊液培养物,以确定是否需要进一步治疗。临床上患者有明显改善。(摘要截断于250字)