CLINICAL STUDIES OF ABNORMAL HOST DEFENSE

这些研究的目的是研究异常宿主的患者， 防御 在1993财政年度，研究集中在吞噬细胞异常的患者， 功能 特别强调的是， 儿童肉芽肿性疾病（CGD），谁继续成为主题 紧张的学习。 研究的其他疾病包括 高免疫球蛋白E复发性感染（Job's）综合征，中性粒细胞 特异性颗粒缺陷、白细胞粘附缺陷等 复发性感染的患者不属于特定的 定义疾病类别。 临床研究包括：（a） 描述一名患有“白细胞渗漏综合征”的患者， 以坏疽性脓毒症为特征，其中循环中性粒细胞 自发释放大量的特定颗粒内容物到 血浆（B）发现慢性感染 缺乏中性粒细胞亚群的非典型分枝杆菌 结合单克隆抗体C10，和（c）继续一个阶段 4项研究评价干扰素-γ在CGD中的长期作用。我们 继续监测和扩大这些患者队列， 模型的免疫操纵的长期研究， 异常宿主防御和研究开发免疫的基因治疗 缺陷

The purpose of these studies was to study patients with abnormal host defense. In FY '93 studies focused on patients with abnormal phagocyte function. Particular emphasis was given to patients with chronic granulomatous diseases of childhood (CGD), who continue to be the subject of intense study. Other diseases that were studied include the Hyperimmunoglobulin-E recurrent infection (Job's) syndrome, neutrophil specific granule deficiency, leukocyte adhesion deficiency and other patients with recurrent infections who do not fall into a specifically defined disease category. Clinical investigations included: (a) description of a patient with "leaky leukocyte syndrome," a new disorder characterized by pyoderma gangrenosum in which circulating neutrophils spontaneous release enormous amounts of specific granule contents into the plasma (b) discovery of a patient whith chronic infection with atypical mycobacteria who has absence of a subpopulation of neutrophils that bind the monoclonal antibody C10, and (c) continuation of a phase 4 study evaluating long term effects of interferon-gamma in CGD. We continue to monitor and expand these cohorts of patients who serve as models for long term studies of the immunological manipulation of the abnormal host defenses and for studies developing gene therapy of immune deficiencies.