-by Rinka, Hiroshi et al. (2008); Published by Biomed Central Paediatrics
Hemorrhagic shock and encephalopathy syndrome (HSES) is a rare but devastating disorder in the young children. It is a disease of unknown aetiology and presents as acute onset of encephalopathy, shock, watery diarrhea, severe disseminated intravascular coagulopathy (DIC), and renal and hepatic dysfunction. Patients manifesting the condition have very poor prognosis with a fatal course or severe neurologic sequelae.
The cause of such clinical picture and outcome may be partly attributed to the delay in diagnosis and therefore, delay in management. HSES may be mistaken for another disease of the nervous system, especially in its initial stage. At times clinicians misdiagnose then, provide a management inconsistent with HSES. Then they may wonder why the symptoms just cannot be controlled. By the time distinctive signs and symptoms of HSES appear, multiple organ failure has occurred. At this point modifying treatment may be too late, and a patient may have died. This elaborates tremendous loss of time, resources, and even life. And so the investigators of the research emphasized a need of early detection of HSES. Resolution of such need will definitely improve survival and reduce neurological sequelae.
The research is a retrospective study made in 2008. The authors of the journal recalled the course of hospitalization of eight patients, who met the classical HSES criteria. These patients were admitted to Intensive Care Unit (ICU), between November 2001 to August 2007. Patients’ age range from four months to nine years old at the time of admission.
The investigators examined the clinical, biological, and radiological findings of the patients/ subjects. The results were summarized by the investigators as follows:
“Although 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 (Rinka et al, 2007).”
Most of the classical signs and symptoms of HSES, as detected by different laboratory and physical examinations are absent initially. CT scan, platelet count, haemoglobin level, and renal function are not useful therefore in early diagnosis. However, the elevated liver enzymes and CK upon admission, hypotension in early stage after admission, acid-base disturbances, and vasopressor infusion are useful markers. This fact is very helpful in starting neurological treatment even before severe neurological manifestations occur.
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