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Published on: November 20, 2015
[Hemorrhagic shock and encephalopathy syndrome in infancy and childhood]
M Kirschstein1, F Aksu, F Tegtmeyer
1Klinik für Pädiatrie, Medizinischen Universität zu Lübeck.
Insights
Hemorrhagic shock and encephalopathy syndrome (HSES) presents with severe symptoms like coma and shock in infants. This study details HSES cases, discussing its nature as a distinct disorder or a known condition with a new name.
Area of Science:
- Pediatrics
- Neurology
- Infectious Diseases
Background:
- Hemorrhagic shock and encephalopathy syndrome (HSES) is a rare but severe condition affecting infants.
- Early recognition and diagnosis are crucial for managing this potentially life-threatening illness.
Observation:
- Two infants presented with prodromal diarrhea and fever, followed by abrupt coma, seizures, shock, and hyperpyrexia.
- Clinical manifestations included bleeding, pancytopenia, and impaired liver and renal function.
Findings:
- Both infants survived but experienced significant neurologic sequelae.
- The observed clinical and laboratory findings were consistent with HSES, first described in 1983.
Implications:
- This case report contributes to understanding the clinical spectrum and diagnostic criteria for HSES.
- Further research is needed to elucidate the etiology and pathogenesis of HSES and determine if it represents a novel entity or a re-emergence of a known disorder.
Abstract:
After a few days of prodromal illness with diarrhea and fever there was an abrupt onset of coma, seizures, severe shock, and hyperpyrexia in two infants; they than developed bleeding, pancytopenia, and impairment of liver and renal function. Both infants survived with major neurologic sequelae. The clinical symptoms and laboratory parameters were compatible with hemorrhagic shock and encephalopathy syndrome which was first described in 1983. The clinical course and biochemical features of this disease and its differential diagnoses are described, and etiology and pathogenesis are discussed. Furthermore it will be discussed, whether the hemorrhagic shock and encephalopathy syndrome is actually a "new" disease or just a new label for a known disorder.
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