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[Changing clinical course of hemolytic uremic syndrome in children]
A Zurowska1, Z Gockowska, P Czarniak
1Klinika Nefrologii Dzieciecej Akademii Medycznej w Gdańsku.
Insights
Hemolytic-uremic syndrome (HUS) incidence decreased, while childhood survival rates dramatically improved. However, long-term consequences remain severe, with many children experiencing impaired kidney function.
Area of Science:
- Pediatric Nephrology
- Epidemiology
- Clinical Outcomes
Context:
- Hemolytic-uremic syndrome (HUS) is a significant cause of acute renal failure in children.
- Understanding temporal trends in HUS incidence, survival, and sequelae is crucial for pediatric nephrology.
- This study analyzes HUS cases over three decades in a defined geographical region.
Purpose:
- To compare the incidence, early survival rates, and long-term consequences of childhood hemolytic-uremic syndrome (HUS) across the 1970s, 1980s, and 1990s.
- To identify prognostic markers for long-term outcomes in pediatric HUS.
- To assess the evolving clinical significance of HUS in pediatric acute renal failure.
Summary:
- 196 children with HUS (94% typical D+ HUS) were treated between 1972-1999, with 87% requiring peritoneal dialysis.
- Incidence of HUS as a cause of acute renal failure decreased from 58% in the 1970s to 30% in the 1990s.
- Survival rates improved significantly from 80% in the 1970s to 97% in the 1990s, despite which 68% of patients had severe long-term outcomes, including renal impairment.
Impact:
- Improved early survival rates for childhood HUS are evident, yet long-term renal sequelae remain a major concern.
- The duration of anuria serves as a critical marker for poor long-term prognosis in pediatric HUS.
- The clinical focus for HUS is shifting towards managing severe, persistent long-term consequences despite better short-term outcomes.
Abstract:
An incidence of hemolytic-uraemic syndrome as a cause of the acute renal failure in childhood, its early survival rate and long-term sequela quences were compared in the 1970s, 1980s, and 1990s in the same geographical area. 196 children with HUS were treated between 1972-1999. 94% had a typical D+ HUS. The majority were of patients severely ill with 87% requiring peritoneal dialysis of an average 14 days duration. The children were grouped into three 9-year periods for comparison (1972-1980, 1981-1989, 1990-1999). 105 patients were followed for 2-27 years (mean 1 years). An incidence of HUS as a cause of ARF in childhood ranged from 58% and 50% in the 1970s and 1980s to 30% in the past decade. With time survival rate has improved dramatically over the years from 80% and 75% to 97% in the 1990s. Deo spite better short term outcome the late consequences of HUS are serious. The longer the follow-up, the greater percentage of patients with severe outcome. At last follow-up only 32% children showed full recovery. 33% are on renal replacement therapy or have impaired renal function. The severity of the initial lesions to the kidney measured by the duration of anuria is a marker of poor late prognosis. With improving early outcome the clinical significance of HUS is focusing on its severe long-term consequences.