Clinico-pathological findings in diarrhoea-negative haemolytic uraemic syndrome.
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.
Taylor
C M
CM
Department of Nephrology, The Birmingham Children's Hospital, Birmingham, B4 6NH, UK. cm.taylor@bch.nhs.uk
Chua
C
C
Howie
A J
AJ
Risdon
R A
RA
D001158:Arteries / Q000473:pathology*; D002675:Child, Preschool; D003967:Diarrhea / Q000473:pathology; D005260:Female; D006463:Hemolytic-Uremic Syndrome / Q000401:mortality / Q000473:pathology* / Q000503:physiopathology*; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D007676:Kidney Failure, Chronic / Q000209:etiology; D008297:Male; D011379:Prognosis; D012189:Retrospective Studies