CLINICAL STUDIES OF ABNORMAL HOST DEFENSE

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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.