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

机译:失血性休克和脑病综合征–早期HSES诊断的标志

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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.
机译:背景技术失血性休克和脑病综合征(HSES)是一种破坏性疾病,影响幼儿。 HSES患者的结局通常是致命的或表现出严重的神经系统后遗症。我们回顾了HSES的早期诊断标记。方法我们检查了8例符合HSES标准的患者(4个月至9岁)的临床,生物学和放射学发现。结果尽管在临床过程中所有8例患者均出现脑水肿,弥散性血管内凝血病(DIC)和多器官功能衰竭,但5例入院时脑计算机断层扫描(CT)扫描显示正常或仅有轻度水肿。所有8例患者血小板计数均正常,无休克。然而,他们都患有严重的代谢性酸中毒,甚至在3小时后仍然持续(中位数碱过量(BE),-7.6 mmol / L)。入院后6小时(BE,-5.7 mmol / L)需要机械通气。入院后12小时内,需要进行液体复苏和输注降压药以治疗低血压。七名患者入院时肝酶和肌酸激酶(CK)升高。入院后二十四小时,所有八名患者都需要输注血管加压药以维持血压。结论入院时CT扫描,血小板计数,血红蛋白水平和肾功能均不能早期诊断HSES。然而,入院时肝酶和CK升高,入院后早期低血压,难治性酸碱对液体复苏的干扰以及升压药的输注是早期HSES诊断的有用标志物,有助于指示开始加强神经治疗。

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