Survey of haemolytic-uraemic syndrome in Queensland 1979-1995

Objective: To review the clinical course of haemolytic-uraemic syndrome (HUS) in children admitted to Brisbane children's hospitals between April 1979 and October 1995. Design: Retrospective case survey. Setting: Royal Children's Hospital and Mater Misericordiae Children's Hospital (the two major children's hospitals in Brisbane). Subjects: All children hospitalised for HUS. Outcome measures: Clinical and laboratory features on presentation (including typical [diarrhoea-positive, D+] or atypical [diarrhoea-negative, D-] presentation), clinical course, treatment and features on subsequent outpatient follow-up (1, 3, 6 and 12 months later), renal outcome on long term follow-up (3-16 years later). Results: 55 children (aged 2 months to 13 years) were hospitalised for HUS, but no epidemic was detected. Seven children (13%) had D- presentations, including three (5%) with T-activation caused by pneumococcal pneumonia. Thrombocytopenia was more severe and prolonged in D- patients (P<0.01). Major complications occurred only in the D+ group (one patient died, and two had recurrences). Chronic renal failure was significantly more likely in patients with prolonged oliguria or hypertension in the acute illness and proteinuria or hypertension on follow-up. Conclusions: The clinical course and outcome in childhood HUS vary greatly and D- HUS is not invariably associated with a poorer prognosis than D+ HUS. Pneumococcal-associated T-activation is an important cause of D- HUS and should be actively sought to allow for appropriate therapy.