Hemorrhagic Shock And Encephalopathy Syndrome (Hses)

Hemorrhagic shock and encephalopathy syndrome is a rare disease, which is characterized by the acute onset of a severe shock, coagulopathy, encephalopathy and hepatic or renal dysfunction in a previously healthy child. This disease leads to death or catastrophic neurological sequelae. The diagnosis is made by exclusion of diseases, particularly sepsis, Reye’s syndrome, hemolytic uremic syndrome and thrombotic thrombocytopenia. Treatment is supportive.

The hemorrhagic shock and encephalopathy syndrome (HSES) is most common in children 3-8 months (the median age is 5 months). It was also observed even at fifteen year old. Given the extremely high temperature and multiple organ disorders is reminiscent of heat stroke HSES. but the cause is unknown. Excessive wrapping of children who have a febrile infection was suspected, but the evidence for this is lacking. Other theories include a reaction on an intestinal toxins, environmental toxins, release from the pancreas of trypsin or an unknown virus or bacterium. Many researchers suspect a genetic defect underlies. A diffuse cerebral edema with obstruction, focal hemorrhages and infarcts in the cerebral cortex and other organs are common. The lungs and the myocardium are not primarily affected.

Hemorrhagic shock and encephalopathy syndrome is a rare disease, which is characterized by the acute onset of a severe shock, coagulopathy, encephalopathy and hepatic or renal dysfunction in a previously healthy child. This disease leads to death or catastrophic neurological sequelae. The diagnosis is made by exclusion of diseases, particularly sepsis, Reye’s syndrome, hemolytic uremic syndrome and thrombotic thrombocytopenia. Treatment is supportive. The hemorrhagic shock and encephalopathy syndrome (HSES) is most common in children 3-8 months (the median age is 5 months). It was also observed even at fifteen year old. Given the extremely high temperature and multiple organ disorders is reminiscent of heat stroke HSES. but the cause is unknown. Excessive wrapping of children who have a febrile infection was suspected, but the evidence for this is lacking. Other theories include a reaction on an intestinal toxins, environmental toxins, release from the pancreas of trypsin or an unknown virus or bacterium. Many researchers suspect a genetic defect underlies. A diffuse cerebral edema with obstruction, focal hemorrhages and infarcts in the cerebral cortex and other organs are common. The lungs and the myocardium are not primarily affected. Symptoms and signs In most patients, there is the prodromal fever, symptoms of infection of the upper respiratory tract, vomiting and diarrhea. The main symptoms are acute onset of encephalopathy, cerebral edema (in the form of seizures, coma and hypotension) and severe shock (u. A. Hypotension and hypoperfusion). Other common symptoms include fever (up to 43.9 ° C rectally), bloody and watery diarrhea, disseminated intravascular coagulation (DIC), myoglobinuria and rhabdomyolysis. Diagnostic laboratory tests like symptoms can range from a sepsis, Reye’s syndrome, a hemolytic-uremic syndrome, thrombotic thrombocytopenia and familial or acquired erythrophagozytischen lymphohistiocytosis caused. Patients require laboratory tests, including cultures of blood, urine, stool, sputum and cerebrospinal fluid, and blood gas analysis, blood count with differential diagnosis, platelets, electrolytes, urea nitrogen, creatinine, PT / PTT, liver function tests, C-reactive protein, creatine kinase, lactate, fibrin, fibrin degradation products, electroencephalogram, ferritin and lipids and head CT and EEG. The multiple pathological findings include metabolic acidosis, an increase in liver enzymes, DIC, elevated creatine kinase, acute renal failure, thrombocytopenia, a falling hematocrit, hypoglycemia, leukocytosis, and hyperkalemia. The bacteriological and viral cultures are negative. The diagnosis is a diagnosis of exclusion. Forecast In all test series died more than 60% of patients and more than 70% of the survivors had severe neurologic sequelae. Early detection and treatment are important to minimize morbidity and mortality. Treatment Supportive Treatment is entirely supportive. Large amounts of intravenous fluids and blood products (FFP, albumin, red blood cell concentrates, complete blood products) along with inotropic drugs (eg. As dopamine, epinephrine) must be given in order to obtain the cycle upright. A balance of acid-base abnormalities may be required. An increase in body temperature> 39 ° C makes an external cooling required (s. Treatment). Intubation and hyperventilation are necessary in order to reduce an increased intracranial pressure due to cerebral edema. DIC, despite administration of fresh frozen plasma is often a progressive character.

Health Life Media Team

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