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A review of extrarenal involvement in diarrhoea-associated haemolytic-uraemic syndrome (HUS) is based on 64 of our autopsied patients and an update of the literature. Large bowel pathology was the commonest (29 cases), followed by the central nervous system (21 cases), the heart (19 cases) and the pancreas (19 cases). The severity of systemic involvement was associated with the magnitude of renal compromise and the prognosis of the acute phase. Diarrhoea-associated HUS is described as a multiorgan entity, due to extensive microvascular damage and thrombosis. At present mortality during the acute phase is not confined to renal failure; systemic involvement can also lead to death.

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Renal biopsies from 19 boys and 11 girls, most with moderate or severe forms of hemolytic uremic syndrome (HUS) of the classic diarrhea-associated type, were analyzed as part of their long-term follow-up. Patients were biopsied because of late or persistent proteinuria, hypertension, and prolonged renal failure. The median length of follow-up was 11.2 years (range 0.9-22.0 years). Four histological groups were identified: focal segmental glomerulosclerosis and hyalinosis (FSGSH) (17 patients), diffuse mesangial proliferative glomerulonephritis (DMPGN) (9 patients), diffuse glomerulosclerosis (2 patients), and minimal glomerular changes (2 patients). The median interval between the onset of disease and renal biopsy was significantly shorter in DMPGN than in FSGSH (P < 0.001). The pathological findings may be the expression of two different stages of the same dynamic process: a regular sequence of glomerular lesions consisting of early DMPGN, followed by FSGSH. This lesion would ultimately lead to the final stage of global glomerulosclerosis. At the last examination, only a quarter of the patients had normal renal function. These observations also confirm that prolonged oligoanuria during the acute stage of HUS frequently results in an unfavorable long-term prognosis.

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Eighteen records from children with renal transplants (RT) and classical haemolytic-uraemic syndrome (HUS) were reviewed. The mean oliguric period was 17.9 +/- 7.5 days; the interval between acute phase and end-stage renal disease (ESRD) was 9.3 +/- 5.2 years. HUS was the most frequent cause of renal transplantation (23.4%). There were no significant differences between patients with HUS and controls (children with RT but without HUS), regarding renal function, frequency of rejections, renal survival (HUS 65%, controls 57%) or patient survival (94.4% and 96.6%, respectively) after 9 years. None had clinical or histopathological evidence of HUS recurrence in the allograft. Of all children with living-related donors (LRD), renal survival after 3 years was longer for those who received cyclosporin A (CSA) (HUS and controls 86%) than for those who did not receive it (HUS 50%, controls 53%). Classical HUS is a frequent cause of ESRD in Argentina. The duration of the acute oliguric period is a good predictor of the likelihood of progression to chronicity. In the classical form of HUS there is no recurrence in the allograft. CSA and LRD can be used without risk in renal transplantation of children with classical HUS.

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