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Hemolytic-uremic syndrome

Hemolytic-uremic syndrome. Corrigan JJ, Jr., Boineau FG. Pediatrics in Review 2001;22(11):365-369.

ABSTRACT:

This paper reviews the definitions, epidemiology, pathogenesis, clinical picture, management, and prognosis of hemolytic uremic syndrome (HUS). Prognosis varies by type of HUS. Typical, diarrhea-positive (D+) HUS, associated with infectious agents, has a more favorable outcome than atypical, sporadic D- HUS. Recurrences associated with D+ HUS are uncommon. The early mortality rate of D+ HUS is about 5%. Another 5% of affected persons develop acute renal failure and anuria, requiring life-long dialysis. Long-term complications include proteinuria, reduced glomerular filtration rate, hypertension, and late development of end-stage renal disease (ESRD). Many HUS-affected individuals studied have experienced initial complete recovery, but developed complications years later. ESRD develops in approximately 10% to 15% of patients followed for 15 to 25 years. Children who have D+ HUS should be followed for many years. Long-term prognosis can be predicted by renal biopsy performed in the initial illness period. Children who have patchy renal cortical necrosis are more likely to develop ESRD or chronic renal failure.