CLINICAL STUDIES OF ABNORMAL HOST DEFENSE

宿主防御异常的临床研究

• 批准号: 5200399
• 负责人: J I GALLIN
• 金额: --
• 依托单位: NATIONAL INSTITUTE OF ALLERGY AND INFECTIOUS DISEASES
• 依托单位国家: 美国
• 资助国家: 美国
• 起止时间: 至
• 项目状态: 未结题
• 来源: https://reporter.nih.gov/project-details/5200399
• 关键词: Actinomycetales infection; Mycobacterium; antifungal agents; chemoprevention; child (0-11); chronic granulomatous disease; clinical trials; human subject; human therapy evaluation; hyperglobulinemia; immunodeficiency; immunopathology chemotherapy; interferon gamma; liver abscess; longitudinal human study; mycosis; opportunistic infections; respiratory infections; respiratory surgery

The purpose of this project is to study patients with abnormal host defense. In FY'95 studies focussed on patients with abnormal phagocyte function. These included patients with chronic granulomatous disease of childhood (CGD), Hyperimmunoglobulin E-recurrent infection sydrome (Job's), leukocyte adhesion deficiency (LHD) and other patients with recurrent infections who do not fall into a specifically defined disease category. Currently we follow over 80 patients with CGD, 18 patients with the hyperimmunoglobulin-E recurrent infection syndrome, and over 20 patients with other phagocyte dysfunction syndromes. In addition, 12 patients are followed with disseminated multiple drug resistant-atypical mycobacteria infections, some of whom appear to have abnormalities of phagocytic cells. A phase IV study of the effect of interferon-gamma for infection prophylaxis in CGD has continued at NIH at the specific request of the FDA. No unexpected toxicities have been seen with interferon- gamma. The reduction in infections observed in the initial study has been sustained and of note is that the incidence of fungal infections in CGD patients receiving interferon-gamma is half that for those patients not receiving the drug. In view of the high incidence of fungal infections in CGD patients, we have also initiated a study designed to assess the efficacy of itraconazole in CGD. Several long term studies were completed related to specific management issues of CGD patients. These included the surgical management of pulmonary infections in CGD, and the surgical pathology of the lung in CGD. In addition we described suppurative cutaneous granulomata caused by Microascus cinereus in a patient with CGD as well as the successful treatment of severe liver abscesses with local installation of neutrophil enriched leukocytes into liver abscess. Each of these clinical studies could only be compiled at NIH where a large cohort of CGD patients could be followed for a long time. Several unique infections and their successful management were described in CGD. These include reports of successful treatment of Sarcinosporon inkin, Exophiala and other fungi.

这个项目的目的是研究宿主异常的患者。 防守。在1995财年，研究集中在吞噬细胞异常的患者 功能。这些患者包括慢性肉芽肿性疾病患者 儿童期(CGD)，高免疫球蛋白E-反复感染综合征 (JOB‘s)、白细胞黏附缺陷(LHD)等 不属于特定定义的疾病的复发性感染 类别。目前，我们跟踪调查了80多名CGD患者，其中18名患者 高免疫球蛋白-E反复感染综合征，20岁以上 有其他吞噬细胞功能障碍综合征的患者。此外，还有12个 患者被随访为播散性多药耐药-非典型 分支杆菌感染，其中一些似乎有异常的 吞噬细胞。干扰素-γ治疗慢性粒细胞白血病的IV期研究 应具体要求，美国国立卫生研究院继续在CGD进行感染预防 美国食品和药物管理局。干扰素没有出现意想不到的毒性- 伽马。在最初的研究中观察到的感染减少 值得注意的是，真菌感染的发病率在 接受干扰素-伽马治疗的CGD患者是这些患者的一半 没有接受药物治疗。鉴于真菌的高发病率 对于CGD患者的感染，我们还启动了一项旨在 评价伊曲康唑治疗慢性阻塞性肺疾病的疗效。几项长期研究 完成了与CGD患者具体管理相关的问题。 其中包括CGD肺部感染的外科治疗， CGD肺的外科病理改变。此外，我们还描述了 由灰微曲霉引起的皮肤化脓性肉芽肿 CGD患者及重症肝的成功治疗 脓肿局部安装中性粒细胞浓集的白细胞 肝脓肿。这些临床研究中的每一项只能在 NIH的一大批CGD患者可能会被长期跟踪 时间到了。几种独特的感染及其成功的管理 在CGD中描述。其中包括成功治疗的报告。 肉孢子菌、外孢子菌等真菌。