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Hemorrhagic shock and encephalopathy: clinical, pathologic, and biochemical features
M Levin1, J R Pincott, M Hjelm
1Infectious Diseases Unit, Hospital for Sick Children, London, United Kingdom.
Insights
Hemorrhagic shock and encephalopathy syndrome (HSES) in children presents with shock, coma, and bleeding. Genetic factors, particularly alpha 1-antitrypsin variants, may contribute to this severe disorder.
Area of Science:
- Pediatric Critical Care
- Neurology
- Biochemistry
Background:
- Hemorrhagic shock and encephalopathy syndrome (HSES) is a severe pediatric condition with poorly understood etiology.
- Previous studies suggest a complex interplay of factors contributing to HSES.
Purpose of the Study:
- To comprehensively define the clinical, pathological, and biochemical characteristics of HSES.
- To investigate potential underlying biochemical abnormalities and genetic predispositions in HSES patients.
Main Methods:
- Retrospective analysis of 25 children diagnosed with HSES.
- Clinical data review, postmortem examinations, plasma biochemical assays, and genetic analysis of alpha 1-antitrypsin phenotypes in relatives.
Main Results:
- All patients exhibited shock, coma, bleeding (or disseminated intravascular coagulation), elevated hepatic enzymes, acidosis, and renal impairment.
- Mortality was high (20/25 deaths), and survivors had significant neurological damage.
- Postmortem findings included microthrombi, hemorrhages, liver necrosis, and cerebral edema.
- Decreased protease inhibitors (alpha 1-antitrypsin, alpha 2-macroglobulin) and increased proteolytic enzymes were common.
- An overrepresentation of uncommon alpha 1-antitrypsin variants (MZ, MS, MC) was observed in relatives, with abnormal accumulation in patients' livers.
Conclusions:
- HSES is a devastating syndrome with high mortality and severe neurological sequelae.
- No infectious cause was identified.
- Defects in protease inhibitor production or release, potentially linked to alpha 1-antitrypsin genetic variants, may play a role in HSES pathogenesis.
Abstract:
To further define the clinical, pathologic, and biochemical features of hemorrhagic shock and encephalopathy syndrome, we studied 25 affected children (aged 3 months to 14 years) admitted to a single center between 1982 and 1985. A prodromal illness comprising vomiting, diarrhea, listlessness, and fever was present in 84% of the cases. Acute onset of shock, convulsions and coma, bleeding (or laboratory evidence of disseminated intravascular coagulation), elevated plasma activity of hepatic enzymes, acidosis, and impaired renal function was present in every case. Twenty patients died, and all the survivors are neurologically damaged. At postmortem examination, intravascular microthrombi coexisting with hemorrhages and petechiae were found in most organs. Centrilobular liver necrosis and cerebral edema were prominent features. No microbiologic cause for the disorder was identified, but decreased plasma levels of the protease inhibitors alpha 1-antitrypsin and alpha 2-macroglobulin, together with increased levels of circulating proteolytic enzymes, were frequently present. An overrepresentation of the uncommon variant phenotypes of alpha 1-antitrypsin was found in first-degree relatives of affected patients (four had the MZ phenotype, and one each the MS or MC phenotype, of 19 relatives studied). Abnormal accumulation of alpha 1-antitrypsin was detected immunohistochemically in the livers of six of the patients. Defective protease inhibitor production or release may be involved in the pathogenesis of the disorder.
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