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September 1982

The Syndrome of Hyperimmunoglobulinemia E and Recurrent Infections

Author Affiliations

Department of Pediatrics and Pathology University of Utah School of Medicine Salt Lake City, UT 84132

Am J Dis Child. 1982;136(9):767-771. doi:10.1001/archpedi.1982.03970450009001

The study of immunologic deficiency diseases, although seemingly consuming an inordinate amount of time, energy, and scarce research funds in proportion to the actual number of cases of any given disorder, has nevertheless shed a great deal of light on the normal person's host defense mechanism. Furthermore, considerable amounts of knowledge have spilled over into both closely related and seemingly unrelated fields of medicine from the study of these fascinating cases. Thus, such diverse disciplines as genetics, endocrinology, dermatology, neurology, embryology, nephrology, and the study of metabolism have benefited from a careful dissection of the pathophysiologic mechanisms involved in immunodeficiency states. The article by Brestel and co-workers in this issue (see p 774) is the first to describe the occurrence of a rare, severe bone dysplasia (osteogenesis imperfecta) in association with the syndrome of hyperimmunoglobulinemia E and recurrent infections (Job's syndrome). As they pointed out, disturbances in bone growth have