Advances in Host Defense Mechanisms. Vol. 3. Chronic Granulomatous Disease

In the 1950s and early 1960s, there were several clinical publications describing children who had repeated bacterial infections, hypergammaglobulinemia, leukocytosis, and generalized granulomatous lesions. This disease was called "fatal chronic granulomatous disease of childhood" (FCGDC). In 1967, Paul Quie and his associates at the University of Minnesota showed that the polymorphonuclear leukocytes from FCGDC patients were able to phagocytose certain bacterial species normally but that these white blood cells lacked the ability to kill the bacteria in-tracellularly. Since that time, the ability to diagnose this infection through the use of cellular biochemical markers, and the capability of preventing infection with pro-phylactic antibiotics and of anticipating and treating infections earlier and more vigorously, has led to a better outlook for children with this condition, so FCGDC has become CGD. Along the way, our understanding of phagocytosis has been revolutionized. One could hardly find a better example of modern medicine taking advantage of "an accident of nature." In the course of looking for the intracellular biochemical defect in CGD, the normal biochemistry and physiology of intracellular bactericidal events had to be discovered. While screening children with unusual susceptibility to infection for CGD, other "accidents of nature" were discovered and their mechanisms elucidated. The methods used for study of CGD were later adopted by immunologists for the reawakening interest in antibody-mediated phagocytosis (opsonophagocytosis). I think that it can be stated that most investigators who are working on phagocytosis by white blood cells today have at some time been involved in CGD, and all owe to CGD the impetus for the development of essential research methods. Chronic Granulomatous Disease, the book, chronicles the history of this disease and, in separately authored chapters, goes into the various abnormalities found in CGD. There are chapters on the abnormalities of glycolysis in CGD, abnormalities in intracellular vacuoles, membrane potentials, and studies of inheritance. The latter is especially important as prenatal diagnosis has been reported. There are several chapters on the patient with CGD-presentation, diagnosis, and management. The authors are all investigators who have made important contributions in the areas they have been asked to cover. Figures and tables are generally excellent, coming either as reprints from standard research publications or summaries by authors who have had a long time to think about the problems. Many of the chapters, especially those dealing with intermediate biochemical metabolism and membrane physiology, are highly technical and written for people actively engaged …