SURVEY OF HEMOLYTIC-UREMIC SYNDROME IN QUEENSLAND 1979-1995

Objective: To review the clinical course of haemolytic-uraemic syndrom e (HUS) in children admitted to Brisbane children's hospitals between April 1979 and October 1995. Design: Retrospective case survey. Settin g: Royal Children's Hospital and Mater Misericordiae Children's Hospit al (the two major children's hospitals in Brisbane). Subjects: All chi ldren hospitalised for HUS. Outcome measures: Clinical and laboratory features on presentation (including typical [diarrhoea-positive, D+] o r atypical [diarrhoea-negative, D-] presentation), clinical course, tr eatment and features on subsequent outpatient follow-up (1, 3, 6 and 1 2 months later), renal outcome on long term follow-up (3-16 years late r). Results: 55 children (aged 2 months to 13 years) were hospitalised for HUS, but no epidemic was detected. Seven children (13%) had D- pr esentations, including three (5%) with T-activation caused by pneumoco ccal pneumonia. Thrombocytopenia was more severe and prolonged in D- p atients (P<0.01). Major complications occurred only in the D+ group (o ne patient died, and two had recurrences). Chronic renal failure was s ignificantly more likely in patients with prolonged oliguria or hypert ension 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 prognos is than D+ HUS. Pneumococcal-associated T-activation is an important c ause of D- HUS and should be actively sought to allow for appropriate therapy.