AN ADULT PATIENT WITH HYPERIMMUNOGLOBULEMIA-E (JOBS) SYNDROME, END-STAGE RENAL-DISEASE AND REPEATED EPISODES OF PERITONITIS

We report the first case of an adult patient with hyperimmunoglobulemi a E (Job's) syndrome, end-stage renal disease and repeated episodes of Staphylococcus peritonitis. A 42-year-old black female with end-stage renal disease was treated with hemodialysis, and later switched to pe ritoneal dialysis because of severe vascular access problems. On CAPD, she had numerous (>15) episodes of staphylococcal peritonitis. Past h istory included chronic atopic eczema and repeated sinopulmonary infec tions since childhood. She was found to have serum IgE levels greater than 10,000 units/ml. This combination of repeated infections, chronic skin condition and phenomenal elevation of IgE levels establish the d iagnosis of hyperimmunoglobulemia E (Job's) syndrome. The cause of rep eated episodes of Staphylococcal peritonitis are presumed to be relate d to impaired host defense mechanisms known to be present in patients with this disorder. As nephrologists, we should be aware that patients with impaired host defense mechanisms are highly susceptible to perit onitis in the presence of an indwelling peritoneal catheter.