Ga. Khan et N. Bank, AN ADULT PATIENT WITH HYPERIMMUNOGLOBULEMIA-E (JOBS) SYNDROME, END-STAGE RENAL-DISEASE AND REPEATED EPISODES OF PERITONITIS, Clinical nephrology, 41(4), 1994, pp. 233-236
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.