The purpose of this project is to study patients with abnormal host defense. In FY'95 studies focussed on patients with abnormal phagocyte function. These included patients with chronic granulomatous disease of childhood (CGD), Hyperimmunoglobulin E-recurrent infection sydrome (Job's), leukocyte adhesion deficiency (LHD) and other patients with recurrent infections who do not fall into a specifically defined disease category. Currently we follow over 80 patients with CGD, 18 patients with the hyperimmunoglobulin-E recurrent infection syndrome, and over 20 patients with other phagocyte dysfunction syndromes. In addition, 12 patients are followed with disseminated multiple drug resistant-atypical mycobacteria infections, some of whom appear to have abnormalities of phagocytic cells. A phase IV study of the effect of interferon-gamma for infection prophylaxis in CGD has continued at NIH at the specific request of the FDA. No unexpected toxicities have been seen with interferon- gamma. The reduction in infections observed in the initial study has been sustained and of note is that the incidence of fungal infections in CGD patients receiving interferon-gamma is half that for those patients not receiving the drug. In view of the high incidence of fungal infections in CGD patients, we have also initiated a study designed to assess the efficacy of itraconazole in CGD. Several long term studies were completed related to specific management issues of CGD patients. These included the surgical management of pulmonary infections in CGD, and the surgical pathology of the lung in CGD. In addition we described suppurative cutaneous granulomata caused by Microascus cinereus in a patient with CGD as well as the successful treatment of severe liver abscesses with local installation of neutrophil enriched leukocytes into liver abscess. Each of these clinical studies could only be compiled at NIH where a large cohort of CGD patients could be followed for a long time. Several unique infections and their successful management were described in CGD. These include reports of successful treatment of Sarcinosporon inkin, Exophiala and other fungi.
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