Chronic granulomatous disease (CGD) is a group of inherited disorders in which neutrophils and other phagocytes have a defective respiratory burst oxidase. These individuals lack an essential host microbial pathway and develop recurrent, severe bacterial and fungal infections. These patients also suffer from the formation of chronic inflammatory granulomas in many tissues. The genes affected in the 4 known genetic subgroups of CGD have now been identified and cloned. Because the genes have been identified, the potential for gene therapy exists. This study will examine patients who have defects in the gp91phox gene which is present in the X-linked form of chronic granulomatous disease. The purpose of this study is to determine the toxicity associated with infusing peripheral blood progenitor cells into which has been inserted a correct copy of the gp91phox gene. The gene correction is performed using retroviral vectors. The process of gene transduction utilized a new technology, the use of recombinant fibronectin fragments. Therefore, an additional objective of the study is to determine the toxicities which may result by the use of fibronectin in the transduction process.
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