Haemorrhagic shock encephalopathy syndrome in the British Isles

The aetiopathogenesis of haemorrhagic shock encephalopathy syndrome (HSES) remains unclear and after concern that a novel environmental agent was the cause, the British Paediatric Association and the Public Health Laboratory Service Communicable Disease Surveillance Centre in 1983 initiated surveillance of this condition in the British Isles. After 1986 cases were ascertained via the British Paediatric Surveillance Unit 'active' reporting scheme; this report presents the findings for 1985-8. Sixty five patients were reported, of whom 52 satisfied the criteria for inclusion. Of those whose outcome was known, 24 (46%) died, 18 had severe neurological damage, and six survived apparently intact. Epidemiological features of note were: the median age of 15 weeks (range 3-140); statistically significant clustering of admission times suggesting a peak onset period at night; lack of geographic clusters, of secular trends and, except for a slight excess in winter months, of seasonality. Clinical and pathological features followed a highly consistent pattern, suggesting that HSES is an individual clinical entity distinguishable from conditions with similar presentations, such as septicaemia and Reye's syndrome. There was no microbiological or epidemiological evidence to support the emergence of a novel environmental agent. Many of the features of HSES were, however, the same as those described in heat stroke and we suggest that the two conditions are the same even though there is usually no history of overt overheating.