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Long-term (15-25 years) outcome of childhood hemolytic-uremic syndrome
M F Gagnadoux1, R Habib, M C Gubler
1Pediatric Nephrology Department, Necker-Enfants-Malades Hospital, Paris, France.
Insights
Childhood Hemolytic Uremic Syndrome (HUS) often resolves, but kidney damage seen in biopsies can predict long-term issues. Renal histology is crucial for assessing the risk of future kidney failure after HUS.
Area of Science:
- Nephrology
- Pediatric Nephrology
- Pathology
Background:
- Infantile Hemolytic Uremic Syndrome (HUS) typically has a good short-term prognosis with renal function recovery.
- Concerns exist regarding long-term renal prognosis due to potential damage observed in kidney biopsies.
Purpose of the Study:
- To investigate the long-term outcomes of childhood HUS.
- To identify predictors of long-term renal sequelae following HUS.
Main Methods:
- A cohort of 29 patients with classical HUS in infancy/childhood was followed for 15-28 years.
- Renal biopsies were performed post-recovery to assess glomerular thrombotic microangiopathy (TMA) and cortical necrosis.
- Long-term renal function, including development of hypertension, proteinuria, chronic renal failure (CRF), and end-stage renal failure (ESRF), was evaluated.
Main Results:
- 10 patients had no renal abnormalities, 12 had residual symptoms, 3 developed CRF, and 4 reached ESRF.
- Long-term outcomes did not correlate with initial clinical severity.
- Renal histology strongly correlated with prognosis: cortical necrosis and extensive TMA (>50% glomeruli) were associated with poorer outcomes.
Conclusions:
- Childhood HUS carries a non-negligible risk of long-term renal failure, even after apparent recovery.
- Renal biopsy findings, particularly the extent of TMA and presence of cortical necrosis, are the best indicators of long-term renal prognosis.
- Close monitoring and histological assessment are vital for managing patients with a history of childhood HUS.
Abstract:
The short-term prognosis of the "typical", "post-enteropathic" form of infantile HUS is usually good, with a complete recovery of renal function. However, the extent of the renal damage observed on some biopsies may raise concern for the long-term prognosis. Therefore, we studied the outcome of 29 patients affected with classical HUS in infancy or early childhood and followed-up for 15-28 years (m = 18 yrs). Initial renal symptoms ranged from a moderate renal failure with normal diuresis to a 12-day anuria: 21 children had to be treated by peritoneal dialysis. Twenty-five patients underwent a renal biopsy shortly after recovery: lesions of glomerular thrombotic microangiopathy (TMA) were found in 14 patients, and patchy cortical necrosis was diagnosed in the other 11. At latest examination 10 patients had no renal abnormality, 12 had residual renal symptoms (hypertension in 7, proteinuria in 4 and midly reduced GFR in 1), 3 were in chronic renal failure (CRF), and 4 had reached end-stage renal failure (ESRF) 16-24 years after onset; 2 of these latter 4 had a normal GFR at 10-year examination. The long-term evolution was not correlated with the initial clinical severity but appeared well correlated with the extent of the histological damage: 10 of the 11 patients with cortical necrosis have either ESRF (4), CRF (3) or renal sequelae (3), and 4 of the 5 patients with TMA involving more than 50% of glomeruli present with moderate sequelae, whereas the 9 patients with TMA involving less than 50% of glomeruli are symptom-free or have mild sequelae. Thus, the risk of renal failure 20 years after a seemingly cured childhood HUS is not negligible, and renal histology is the best indicator of long-term prognosis.