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Published on: May 21, 2019
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.
The Journal of Pediatrics
|February 1, 1989
Summary
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.
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