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. Subsequently, > 140 cases have been reported worldwide, although no cases have been previously reported in the forensic literature. Typically the child presents with fever, shock, encephalopathy with coma and seizures, evidence of hemorrhage, and diarrhea. Laboratory investigation reveals falling hemoglobin and platelet counts, renal impairment, evidence of disseminated intravascular coagulation, metabolic acidosis, and raised serum transaminases. Microbiological cultures are uniformly negative. The condition has a high mortality and morbidity. The etiology is unknown and may be multifactorial. However, hyperpyrexia appears to play a central role in pathogenesis. The diagnosis of HSES in the deceased child is one of exclusion and requires a careful antemortem history as well as a thorough autopsy with toxicological and microbiological investigations. A case of HSES is reported and the literature reviewed.
Little
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D
Department of Forensic Medicine, Westmead Hospital, New South Wales, Australia.
Wilkins
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B
D001927:Brain Diseases / Q000209:etiology* / Q000473:pathology; D002675:Child, Preschool; D003645:Death, Sudden / Q000209:etiology* / Q000473:pathology; D003937:Diagnosis, Differential; D004660:Encephalitis / Q000209:etiology* / Q000473:pathology; D017809:Fatal Outcome; D005260:Female; D006801:Humans; D012771:Shock, Hemorrhagic / Q000209:etiology* / Q000473:pathology; D013577:Syndrome