Abstract
This is a retrospective, national clinico-pathological study of past and current patients with haemolytic uraemic syndrome not associated with diarrhoea (D− HUS). Thirty-four patients were analysed and notified by members of the British Association for Paediatric Nephrology in 1998–1999. There was a 2:1 excess of males. Ten presented in infancy. The aetiology included 5 patients with complement abnormalities, 2 patients with complications of pneumococcal infection, and 2 with malignancies. Parental consanguinity was noted in 6 patients. Five children died, 9 developed chronic renal failure, and 10 end-stage renal failure. Only 7 made full recoveries. With a single exception, the pathological findings were unlike the previously reported glomerular thrombosis that is characteristic of diarrhoea-associated HUS, or HUS complicating verocytotoxin-producing Escherichia coli infection. Early and late glomerulopathy could be distinguished. Arteriolar and arterial disease was observed in 8 and 7 patients, respectively. Arterial disease correlated with a poor outcome. The pathology of D− HUS is of prognostic value, but this study was not powered to identify specific aetiological/pathological correlations.
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Acknowledgements
We wish to thank the following pathologists for their help: Dr. A.P. Boon, Dr. R.B. Hartley, Dr. A.G. Howatson, Dr. A.R. Morley, Dr. M.J. Newbould, Dr. D.S. Peat, Dr. F. Raafat, and Dr. P. Ramani.
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This paper was written on behalf of the British Association for Paediatric Nephrology
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Taylor, C.M., Chua, C., Howie, A.J. et al. Clinico-pathological findings in diarrhoea-negative haemolytic uraemic syndrome. Pediatr Nephrol 19, 419–425 (2004). https://doi.org/10.1007/s00467-003-1385-9
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DOI: https://doi.org/10.1007/s00467-003-1385-9