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Atypical hemolytic-uremic syndrome: a comparison with postdiarrheal disease
R L Siegler1, A T Pavia, F L Hansen
1Department of Pediatrics, Division of Nephrology, University of Utah School of Medicine, Salt Lake City, USA.
The Journal of Pediatrics
|April 1, 1996
Summary
Atypical hemolytic-uremic syndrome (HUS) in the western US presents with milder acute kidney injury and similar outcomes to typical HUS, except for patients with recurrent disease.
Area of Science:
- Nephrology
- Pediatric Nephrology
- Epidemiology
Background:
- Hemolytic-uremic syndrome (HUS) is a severe condition characterized by microangiopathic hemolytic anemia, thrombocytopenia, and acute kidney injury.
- Distinguishing between atypical (nondiarrheal) HUS and classic postdiarrheal HUS is crucial for understanding disease variations and outcomes.
Purpose of the Study:
- To compare epidemiologic, laboratory, clinical, and outcome variables between atypical (nondiarrheal) HUS and classic postdiarrheal HUS.
- To identify specific differences in disease presentation and patient outcomes between the two forms of HUS.
Main Methods:
- A retrospective review of 28 episodes of atypical HUS in 22 children was conducted.
- These cases were compared with 266 episodes of typical postdiarrheal HUS in 265 children over a 24-year period.
Main Results:
- Atypical HUS constituted 9.5% of all HUS episodes, with 18% experiencing recurrences.
- Patients with atypical HUS showed significantly lower white blood cell counts, serum creatinine, and blood urea nitrogen levels, along with less frequent oliguria, anuria, and need for dialysis.
- No deaths occurred in the atypical HUS group, compared to 3.4% in the typical HUS group; however, no significant differences in end-stage renal disease or chronic renal sequelae were observed, except in recurrent atypical HUS cases.
Conclusions:
- Atypical HUS in the western United States is associated with milder acute nephropathy compared to classic postdiarrheal HUS.
- Outcomes for atypical HUS are generally favorable, comparable to typical HUS, particularly for non-recurrent cases.
- Patients with recurrent atypical HUS may be at higher risk for end-stage renal disease, warranting further investigation.
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