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Long-term prognosis of hemolytic uremic syndrome and effective renal plasma flow
D Hüseman1, J Gellermann, I Vollmer
1Department of Pediatric Nephrology, Charité, Humboldt University, Schumannstrasse 20-21, D-10117 Berlin, Germany.
Insights
Diarrhea-associated hemolytic uremic syndrome (D+ HUS) can lead to long-term kidney problems in children. Reduced effective renal plasma flow (ERPF) in the second year is a key indicator of poor prognosis.
Area of Science:
- Nephrology
- Pediatric Nephrology
- Renal Medicine
Background:
- Diarrhea-associated hemolytic uremic syndrome (D+ HUS) is a serious condition in children, often leading to acute renal failure.
- Long-term outcomes and risk factors for renal sequelae in D+ HUS survivors are not fully understood.
Purpose of the Study:
- To evaluate the long-term prognosis of D+ HUS in a pediatric cohort.
- To identify risk factors and predictive markers for unfavorable renal outcomes after D+ HUS.
Main Methods:
- Serial assessment of glomerular filtration rate (GFR) and effective renal plasma flow (ERPF) using (51)Cr-EDTA and (123)iodine-hippurate clearances.
- Follow-up evaluation for renal sequelae including proteinuria, hypertension, and reduced GFR.
- Statistical analysis to identify risk factors for poor outcomes.
Main Results:
- At a median follow-up of 5 years, 23% of children had renal sequelae (proteinuria, hypertension, or reduced GFR).
- Anuria >7 days and acute phase hypertension were significant risk factors for unfavorable outcomes.
- Reduced ERPF in the second year post-HUS was observed in 93% of patients with sequelae and correlated with hyperfiltration.
Conclusions:
- Loss of nephrons during acute D+ HUS may lead to hyperfiltration and progressive renal disease.
- ERPF in the second year after D+ HUS is a valuable predictor of long-term renal sequelae and prognosis.
Abstract:
The long-term prognosis of diarrhea-associated hemolytic uremic syndrome (D+ HUS) was evaluated in a cohort of 127 of 149 children who had survived the acute phase. Glomerular filtration rate (GFR) and effective renal plasma flow (ERPF) were estimated by serial (51)Cr-EDTA and (123)iodine-hippurate clearances. All children had acute renal failure during the initial phase and 74% of patients were dialyzed. During the 1st year, mean GFR and ERPF increased continuously until a plateau was reached. In the 2nd year after the diagnosis of HUS, GFR was below 80 and ERPF below 515 ml/min per 1. 73 m(2) in 16% and 47% of patients, respectively. At the end of a median follow-up of 5.0 (range 2.0-13.2) years, the proportion of children with renal sequelae such as proteinuria >/=300 mg/l, hypertension, or a GFR <80 ml/min per 1.73 m(2) was 23%. Anuria of more than 7 days' duration and hypertension during the acute phase were statistically significant risk factors for an unfavorable outcome. A reduced ERPF in the 2nd year was found in 93% of patients with sequelae. Mean filtration fraction (SD) in these patients was 0. 26 (+/-0.07) versus 0.19 (+/-0.05) in patients without sequelae (P<0. 0001). These data suggest that loss of nephrons during the acute phase may implicate hyperfiltration in the residual functioning kidney mass leading to progressive renal disease. ERPF in the 2nd year after D+ HUS may serve as an excellent parameter to detect patients with a high risk of an unfavorable long-term outcome.