Haemorrhagic shock and encephalopathy syndrome: report of two cases with special reference to hypoglycaemia

Haemorrhagic shock and encephalopathy syndrome (HSES) is a devastating diso rder affecting infants. So far no cases have been reported in Switzerland. It is characterised by the abrupt onset of hyperpyrexia,shock,encephalopath y diarrhoea, disseminated intravascular coagulation (DIC) and renal and hep atic failure in previously healthy infants. Severe hypoglycaemia has been r epeatedly reported in association with HSES. However, the pathophysiology o f the hypoglycaemia is not clear. report on two infants (2 and 7 months old ) with typical HSES, both of whom were presented with nonketotic hypoglycae mia. In the first case, plasma insulin was 23 pmol/l at the time of hypo-gl ycaemia (0.1 mmol/l). In the second case, in creased values for interleukin -6 (IL-6) (319 pg/ml) and IL-8 (1382 pg/ml) were found 24 hours after admis sion, whereas IL-1 and tumour necrosis factor-alpha (TNF-alpha) were not me asurable. Alpha-l-antitrypsin was decreased (0.6 g/l). In hyperpyrexic, unc onscious and shocked infants, HSES should be considered and hypoglycaemia s hould be specifically looked for. Hypoglycaemia is not caused by hyperinsul inism but may be secondary to the release of cytokines.