HEMORRHAGIC-SHOCK AND ENCEPHALOPATHY SYNDROME - AN UNUSUAL CAUSE OF SUDDEN-DEATH IN CHILDREN

Hemorrhagic shock and encephalopathy syndrome (HSES) is a sudden-onset symptom complex occurring in previously healthy infants and children. It was first described in 1983 in the United Kingdom in 10 infants. S ubsequently, >140 cases have been reported worldwide, although no case s have been previously reported in the forensic literature. Typically the child presents with fever, shock, encephalopathy with coma and sei zures, evidence of hemorrhage, and diarrhea. Laboratory investigation reveals falling hemoglobin and platelet counts, renal impairment, evid ence of disseminated intravascular coagulation, metabolic acidosis, an d raised serum transaminases. Microbiological cultures are uniformly n egative. The condition has a high mortality and morbidity. The etiolog y is unknown and may be multifactorial. However, hyperpyrexia appears to play a central role in pathogenesis. The diagnosis of HSES in the d eceased child is one of exclusion and requires a careful antemortem hi story as well as a thorough autopsy with toxicological and microbiolog ical investigations. A case of HSES is reported and the literature rev iewed.