Research article

Hemorrhagic shock and encephalopathy syndrome – the markers for an early HSES diagnosis

Hiroshi Rinka¹ , Takeshi Yoshida¹ , Tetsushi Kubota¹ , Miho Tsuruwa¹ , Akihiro Fuke¹ , Akira Yoshimoto¹ , Masanori Kan¹ , Dai Miyazaki¹ , Hideki Arimoto¹ , Toshinori Miyaichi¹ , Arito Kaji¹ , Satoru Miyamoto¹ , Ichiro Kuki² and Masashi Shiomi³

¹Emergency and Critical Care Medical Center, Osaka City General Hospital, Osaka, Japan

²Children Medical Center, Division of Pediatric Neurology, Osaka, Japan

³Division of Infectious Diseases, Osaka City General Hospital, Osaka, Japan

author email corresponding author email

BMC Pediatrics 2008, 8:43doi:10.1186/1471-2431-8-43

Published:	16 October 2008

Abstract

Background

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. We reviewed the markers for an early diagnosis of HSES.

Methods

We examined the clinical, biological and radiological findings of 8 patients (4 months to 9 years old) who met the HSES criteria.

Results

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.

Conclusion

CT 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.