Heterogeneity of atypical haemolytic uraemic syndromes
University Children's Hospital, Nephrology
Unit, Steinwiesstrasse 75, CH-8032 Zurich, Switzerland
Correspondence to: Dr Neuhaus.
Accepted 20 February 1997
Atypical, non-diarrhoea associated haemolytic uraemic syndrome
(D
HUS) is a heterogeneous disorder with a generally poor outcome, although this view has now been questioned. The clinical and laboratory features of 23 children with DHUS, representing a third of all patients with HUS seen during the last 26 years, were examined. The
median age was 4.9 years (range 3 days-13.8 years). Twenty one
children (91%) survived the initial phase. All patients except six
infants aged <18 months required dialysis (74%). Hypertension (43%),
cardiomyopathy (43%), and cerebral convulsions (48%) were common.
Nineteen (83%) children were followed up for a median period of 5.5 years (range 0.5-23.4). Only five (26%) patients, among them four
infants, recovered completely. Six (32%) patients had one to 10 recurrences, including two siblings with neonatal onset, and eight
(42%) developed end stage renal failure. Five children underwent
cadaveric renal transplantation, with recurrence and subsequent graft
failure in two. Four children died, resulting in an overall mortality
of 26%. Atypical HUS is heterogeneous with regard to epidemiology,
pathophysiology, and outcome. Children with a recurrent, familial, or
neonatal course have worse outcomes; in contrast, infants not requiring
dialysis in the acute phase have a better prognosis.
© 1997 by Archives of Disease in Childhood
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