LONG-TERM (15-25 YEARS) OUTCOME OF CHILDHOOD HEMOLYTIC-UREMIC-SYNDROME

The short-term prognosis of the ''typical'', ''post-enteropathic'' for m of infantile HUS is usually good, with a complete recovery of renal function. However, the extent of the renal damage observed on some bio psies may raise concern for the long-term prognosis. Therefore, we stu died the outcome of 29 patients affected with classical HUS in infancy or early childhood and followed-up for 15-28 years (m = 18 yrs). Init ial renal symptoms ranged from a moderate renal failure with normal di uresis 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 t he other 11. At latest examination 10 patients had no renal abnormalit y, 12 had residual renal symptoms (hypertension in 7, proteinuria in 4 and midly reduced GFR in 1), 3 were in chronic renal failure (CRF), a nd 4 had reached end-stage renal failure (ESRF) 16-24 years after onse t; 2 of these latter 4 had a normal GFR at 10-year examination. The lo ng-term evolution was not correlated with the initial clinical severit y but appeared well correlated with the extent of the histological dam age: 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 invo lving more than 50% of glomeruli present with moderate sequelae, where as the 9 patients with TMA involving less than 50% of glomeruli are sy mptom-free or have mild sequelae. Thus, the risk of renal failure 20 y ears after a seemingly cured childhood HUS is not negligible, and rena l histology is the best indicator of long-term prognosis.