Survey of haemolytic‐uraemic syndrome in Queensland 1979–1995

Abstract

H aem olytic-u raem ic syndrome (HUS) is characterised by the triad of microangiopathic haemolytic anaemia, thrombocytopenia and acute renal insufficiency, and is a major cause of acute renal failure in childhood.' HUS has been broadly classified into two forms: the typical or epidemic form, also known as diarrhoeaassociated (D+) HUS, and the atypical or sporadic form, diarrhoea-negative (D-) HUS.2,3 Most children with D+ HUS recover spontaneously, whereas those with D-HUS have a poorer prognosis.v' Acute mortality ranges from 5% to 10%.4,5We conducted a retrospective survey of patients with HUS in the Brisbane area over a 16-year period. Our aim was to review the features and management of HUS and to determine whether features that differed between D+ and D-HUS were predictive of clinical course and development of chronic renal failure. Methods AbstractObjective: 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<O.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.

Enterohemorrhagic Escherichia coli (EHEC) in Pediatric Hemolytic-Uremic Syndrome: A Prospedtive Study in Germany and Austria*

“…The frequencies of hypertension (9%) and cerebral convulsions (3.1%) in the acute phase were lower than in other studies: hypertension: Australia 31-57% [33], British Isles 32% [34]; convulsions: Canada 10% [31], British Isles 19% [34]. The case fatality rate observed in this study (3.1%) was lower than in comparable studies from Belgium with 6.3% [32], Bangladesh with 15% [35] and the reported overall case fatality rate of 5% [3].…”

RBC T activation and hemolysis: implications for pediatric transfusion management

“…T activation has been detected in about one‐third of the patients in high‐risk populations such as children with NEC 30,31 or adults with sepsis in a surgical intensive care unit 3 . T activation was reported in three of seven children with diarrhea‐negative hemolytic‐uremic syndrome (HUS), all of whom had blood cultures positive for S. pneumoniae 32 …”

“…Although there are other reported cases in which plasma transfusion was temporally related to hemolysis in patients with T activation, a definite cause‐and‐effect relationship cannot be established 3,32,43,60–62 . The largest case series reported 1672 infants admitted to an intensive care unit who were screened prospectively for T activation 2 .…”

Hemolytic uremic syndrome associated with pneumococcal pneumonia in Taiwan

“…However, Streptococcus pneumoniae (S. pneumoniae) has been associated with HUS and carries a higher risk of mortality and morbidity [2]. S. pneumoniae was reported to cause 2-5% of HUS in Western countries [7,10,17,18]. There is a lack of information about S. pneumoniae-associated HUS in Asia.…”