BACKGROUND: Atypical @entity2142 (aHUS) accounts for    10% of all cases of @entity11849 and is often due to @entity465 . The short-term outcomes for this disease are established, but there are limited long-term data. The long-term outcomes of a comprehensive nationwide cohort of @entity1 with aHUS are presented here. METHODS: The Australian Paediatric Surveillance Unit prospectively collected data on all cases of @entity11849 in @entity1 seen by paediatricians between 1994 and 2001. @entity1 with aHUS were followed-up with a written questionnaire to the treating clinician at 1   year and again before transition to adult services or at last known follow-up. RESULTS: There were 146 reported cases of @entity11849 , of which 14 were aHUS. Ten @entity1 required dialysis at first presentation, including two who died and three who did not recover renal function. The disease was relapsing in all but one who survived the presenting episode, with most relapses occurring in the first 12   months. At 1   year, one @entity1 was lost to follow-up. Nine of the remaining 11 @entity1 were dialysis dependent. Thirteen kidneys were transplanted into eight @entity1 . There was disease recurrence in eight kidneys, which resulted in graft loss in seven. There were three further deaths 1.7, 6.7 and 16.1   years after the initial presentation. Five @entity1 developed @entity16 and two had @entity299 , largely at the time of onset of the disease. CONCLUSIONS: aHUS is a rare but devastating disease with very high mortality and morbidity that extends beyond the initial presentation period.

The long-term outcomes of atypical XXXX : a national surveillance study.

@entity1 :: ('9606', 'Species') :: ['children', 'Patients', 'patients', 'child'] @entity16 :: ('MESH:D009422', 'Disease') :: ['neurological complications'] @entity11849 :: ('MESH:D065766', 'Disease') :: ['HUS'] @entity465 :: ('MESH:D007153', 'Disease') :: ['complement dysregulation'] @entity299 :: ('MESH:D006331', 'Disease') :: ['cardiac complications'] @entity2142 :: ('MESH:D006461', 'Disease') :: ['haemolytic uraemic syndrome']
@entity2142 :: (MESH:D006461,Disease) :: ['haemolytic uraemic syndrome']
