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Hemorrhagic
shock and encephalopathy syndrome is an extremely rare disease
characterized by acute onset of severe shock, coagulopathy,
encephalopathy, and hepatic and renal dysfunction in previously
healthy children, resulting in death or catastrophic neurologic
outcome.
Hemorrhagic
shock and encephalopathy syndrome (HSES) occurs predominantly in
infants aged 3 to 8 mo (median age, 5 mo) but was reported in a
15-yr-old. HSES resembles heatstroke, with extremely high
temperature and multiple organ dysfunction, but the cause is
unknown. Overwrapping of infants who have febrile illness has been
suggested, but evidence is slim. Other theories include a reaction
to intestinal or environmental toxins, pancreatic release of
trypsin, or an unidentified virus or bacterium. Diffuse cerebral
edema with herniation and focal hemorrhages and infarcts in the
cerebral cortex and other organs are common. The lungs and
myocardium are not primarily involved.
Symptoms
and Signs
A
prodrome of fever, upper respiratory tract symptoms, or vomiting
and diarrhea occurs in most patients. The major features are an
acute onset of encephalopathy, cerebral edema (manifested as
seizures, coma, and hypotonia), and severe shock (ie, hypotension
and poor perfusion). Other common features include hyperpyrexia
(up to 43.9° C rectally), bloody or
watery diarrhea, disseminated intravascular coagulation (DIC),
myoglobinuria, and rhabdomyolysis.
Diagnosis
Similar
symptoms can result from sepsis, Reye's syndrome, and hemolytic-uremic
syndrome. Patients require laboratory evaluation including blood
and urine culture, ABG, CBC, electrolytes, BUN, creatinine,
PT/PTT, and liver function tests. Multiple abnormalities include
metabolic acidosis, elevated liver transaminases, acute renal
failure, thrombocytopenia, falling Hct, leukocytosis,
hypoglycemia, and hyperkalemia. Bacteriologic and viral cultures
are negative. Diagnosis is by exclusion.
Prognosis
and Treatment
In
all series, > 60% of patients died,
and ≥ 70% of survivors had
severe neurologic sequelae.
Treatment
is entirely supportive. Infusions of large volumes of isotonic
solutions and blood products (fresh frozen plasma, albumin, whole
blood, packed RBCs) along with inotropic support - eg, dopamine, epinephrine) are necessary to maintain
circulation. Marked temperature elevation (eg, >
39° C) requires external cooling (see Heat
Illness: Prognosis and Treatment). Increased intracranial
pressure from cerebral edema requires intubation and
hyperventilation. DIC often progresses despite use of fresh frozen
plasma.
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