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 '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.