ABSTRACT

The implications of new information on host defense problems in pulmonary disease are far-reaching. This chapter reviews current information on those inherited immune defects which lead to pulmonary disease, and focuses on certain genetic problems. Serum antibodies play at least four major roles in bacterial infections: toxin neutralization; bacteriolysis in the presence of complement; opsonization of bacteria prior to phagocytosis; and formation of immune complexes which generate chemotactic cleavage products of complement. It is involved in both classical and alternate pathways and its absence leads to defects in opsonization and phagocytosis of bacteria, in Chemotaxis, and in complement-mediated lysis. V Host defense defects usually are manifested via a final common pathway of severe infection. It is hoped that the examples selected emphasize the need for understanding of both basic genetic mechanisms and of specific genetic defects in the study of pulmonary disease.