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lüll Heterogeneity of atypical haemolytic uraemic syndromes Neuhaus TJ; Calonder S; Leumann EPArch Dis Child 1997[Jun]; 76 (6): 518-21Atypical, 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.|Acute Disease[MESH]|Adolescent[MESH]|Child[MESH]|Child, Preschool[MESH]|Diarrhea/etiology[MESH]|Female[MESH]|Follow-Up Studies[MESH]|Hemolytic-Uremic Syndrome/complications/*diagnosis/therapy[MESH]|Humans[MESH]|Infant[MESH]|Infant, Newborn[MESH]|Kidney Failure, Chronic/etiology[MESH]|Male[MESH]|Prognosis[MESH]|Recurrence[MESH]|Renal Dialysis[MESH] |