Abstract
BackgroundThe hemorrhagic shock and encephalopathy syndrome (HSES) is a devastating disease that affects young children. The outcomes of HSES patients are often fatal or manifesting severe neurological sequelae. We reviewed the markers for an early diagnosis of HSES.MethodsWe examined the clinical, biological and radiological findings of 8 patients (4 months to 9 years old) who met the HSES criteria.ResultsAlthough cerebral edema, disseminated intravascular coagulopathy (DIC), and multiple organ failure were seen in all 8 cases during their clinical courses, brain computed tomography (CT) scans showed normal or only slight edema in 5 patients upon admission. All 8 patients had normal platelet counts, and none were in shock. However, they all had severe metabolic acidosis, which persisted even after 3 hours (median base excess (BE), -7.6 mmol/L). And at 6 hours after admission (BE, -5.7 mmol/L) they required mechanical ventilation. Within 12 hours after admission, fluid resuscitation and vasopressor infusion for hypotension was required. Seven of the patients had elevated liver enzymes and creatine kinase (CK) upon admission. Twenty-four hours after admission, all 8 patients needed vasopressor infusion to maintain blood pressure.ConclusionCT scan, platelet count, hemoglobin level and renal function upon admission are not useful for an early diagnosis of HSES. However, the elevated liver enzymes and CK upon admission, hypotension in the early stage after admission with refractory acid-base disturbance to fluid resuscitation and vasopressor infusion are useful markers for an early HSES diagnosis and helpful to indicate starting intensive neurological treatment.
Highlights
The hemorrhagic shock and encephalopathy syndrome (HSES) is a devastating disease that affects young children
The biological investigations performed for all 8 patients included white blood cell (WBC) counts, C-reactive protein (CRP), platelet counts, hemoglobin, asparate aminotransferase (AST), and alanine aminotransferase (ALT), creatinine, base excess, creatine kinase (CK), and cell counts of cerebrospinal fluid (CSF)
When such a patient presents with elevated liver enzymes and CK upon admission, hypotension within 24 hours after admission, with refractory acid-base disturbance and an abnormally high serum lactate level, even with fluid-resuscitate and/or vasopressor infusion, these signs may be useful markers for an early HSES diagnosis and indicators to start intensive neurological treatment
Summary
The hemorrhagic shock and encephalopathy syndrome (HSES) is a devastating disease that affects young children. The outcomes of HSES patients are often fatal or manifesting severe neurological sequelae. Since the original description of the hemorrhagic shock and encephalopathy syndrome (HSES) by Levin et al [1], numerous cases have been reported in the literature. The etiology of HSES remains unknown, this syndrome is associated with acute onset of encephalopathy, shock, watery diarrhea, severe disseminated intravascular coagulopathy (DIC), and renal and hepatic dysfunction. All 8 patients needed mechanical ventilation due to coma and/or seizure. As some authors have defined the HSES criteria [2,3,4], patients meeting them will usually have very poor prognoses with a fatal course or severe neurological sequelae. Our experience suggests that early detection of HSES plays an important role in survival and the reduction of neurological sequelae
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