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1991财政年度，研究主要集中在异常 吞噬细胞功能 特别强调的是， 儿童慢性肉芽肿病（CGD）， 在宿主防御实验室里进行深入研究的课题。 一些 今年进行的临床观察包括 以下：Sarcinosporon inkin侵袭性感染的证明 在冠心病患者中，显示正常和异常 中性粒细胞可以合作破坏烟曲霉菌， 对使用白色细胞具有重要临床意义的观察结果 CGD中的输血;证明CGD患者接受长期 使用甲氧苄啶-磺胺甲恶唑的长期预防治疗 感染过氧化氢酶阳性细菌的频率， 真菌感染发生率增加;完成长期审查 感染累及骨骼的CGD和麻醉 CGD中的注意事项; γ-干扰素在CGD中的临床疗效 CGD得到证实。 我们继续监测和扩大这些队列 以及白细胞粘附缺陷患者， 高免疫球蛋白-E综合征和复发性感染。

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.