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91财年，研究主要集中在异常患者身上 吞噬细胞功能。特别强调对病人的关注 患有儿童慢性肉芽肿性疾病(CGD)的人 宿主防御实验室的密集研究课题。其中一些 今年进行的临床观察包括 以下是肉孢子菌墨汁侵袭性感染的演示 在一位冠心病患者身上；展示了正常和不正常 中性粒细胞可以协同作用破坏烟曲霉。 白细胞应用的观察及其临床意义 CGD中的输血；CGD患者接受长时间 甲氧苄氨嘧啶-磺胺甲恶唑的长期预防性用药减少 对无过氧化氢酶阳性细菌的感染频率 真菌感染发病率增加；完成长期审查 慢性阻塞性肺疾病感染累及骨骼与麻醉的关系 干扰素治疗慢性粒细胞白血病的临床疗效观察 演示了CGD。我们继续监测和扩大这些群体 的患者以及白细胞黏附缺陷和 高免疫球蛋白-E综合征与反复感染。

The purpose of these studies was to study patients with abnormal host defense. In FY '91 studies focused mostly on patients with abnormal phagocytic cell function. Particular emphasis was given to patients with chronic granulomatous disease of childhood (CGD), who are the subject of intense study in the Laboratory of Host Defenses. Some of the clinical observations that were made this year included the following: Demonstration of invasive infection with Sarcinosporon inkin in a patient with CHD; demonstration that normal and abnormal neutrophils can cooperate to damage Aspergillus fumigatus, an observation with important clinical implications for use of white cell transfusions in CGD; demonstration that patients with CGD receiving long term prophylaxis with trimethoprim-sulfamethoxazole have reduced frequency of infection against catalase positive bacteria without increased incidence of fungal infection; completion of long term reviews of skeletal involvement by infection in CGD and anesthesia considerations in CGD; the clinical efficacy of in Gamma-interferon in CGD was demonstrated. We continue to monitor and expand these cohorts of patients as well as patients with leukocyte adhesion deficiency and the syndrome of hyperimmunoglobulin-E and recurrent infections.