title: Heterogeneity of atypical haemolytic uraemic syndromes.
abstract: 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 D-HUS, 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.
authors.affiliation: University Children's Hospital, Nephrology Unit, Zurich, Switzerland.
authors.forename: T J
authors.initials: TJ
authors.lastname: Neuhaus
authors.forename: S
authors.initials: S
authors.lastname: Calonder
authors.forename: E P
authors.initials: EP
authors.lastname: Leumann
mesh_terms: D000208:Acute Disease; D000293:Adolescent; D002648:Child; D002675:Child, Preschool; D003967:Diarrhea / Q000209:etiology; D005260:Female; D005500:Follow-Up Studies; D006463:Hemolytic-Uremic Syndrome / Q000150:complications / Q000175:diagnosis* / Q000628:therapy; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D007676:Kidney Failure, Chronic / Q000209:etiology; D008297:Male; D011379:Prognosis; D012008:Recurrence; D006435:Renal Dialysis