CLINICAL STUDIES OF ABNORMAL HOST DEFENSE

这些研究的目的是研究宿主异常的患者 防守。在1992财年，研究的重点是吞噬细胞异常的患者 功能。特别强调慢性阻塞性肺疾病患者。 儿童肉芽肿性疾病(CGD)，他们仍然是主题 全神贯注学习。其他被研究的疾病包括 高免疫球蛋白-E反复感染综合征，中性粒细胞 特殊颗粒虚弱、白细胞黏附虚弱等 反复感染的患者不属于特定的 定义的疾病类别。临床调查包括：(A) 外科手术成功治疗皮炎外隐孢子虫感染 两性霉素B、氟胞嘧啶和白细胞输注，(B)a 详细评价CGD的泌尿系表现 证明类固醇是治疗糖尿病的重要辅助手段 CGD的泌尿学表现(C)启动4期研究 评价干扰素治疗慢性阻塞性肺疾病的远期疗效。我们将继续 监测和扩大这些患者队列，这些患者将成为 异常宿主免疫调控的长期研究 防御和开发免疫缺陷的基因疗法的研究。

The purpose of these studies was to study patients with abnormal host defense. In FY '92 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) the successful treatment of Exophiala dermatitidis infection with surgery, amphotericin B, flucytosine, and white blood cell transfusions, (b) a detailed evaluation of the urological manifestations of CGD with the demonstration that steroids are an important adjunct in the treatment of the urological manifestations of CGD (c) initiation of a phase 4 study evaluating long term effects of gamma-interferon in CGD. We continue to monitor and expand these cohorts of patients who will 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.