Hemorrhagic shock and encephalopathy syndrome

Other Names: HSES; Hemorrhagic shock and encephalopathy syndrome

Hemorrhagic shock and encephalopathy syndrome (HSES) is a rare disease that occurs suddenly in previously healthy children. This condition is characterized by severe shock, impairment of the blood's ability to clot (coagulopathy), abnormal brain function or structure (encephalopathy), and liver and kidney dysfunction. Most cases of HSES occur in infants from age 3 to 8 months of age, although it can also occur in older children.

Cause

The cause of HSES is unknown. Some researchers believe that this condition is caused by a complex combination of genetic and environmental factors. Researchers have proposed various factors that may contribute to the development of this condition, including infection, exposure to [[toxins in the environment, and overwrapping of infants with a fever.

Symptoms This condition is characterized by severe shock, impairment of the blood's ability to clot (coagulopathy), abnormal brain function or structure (encephalopathy), and liver and kidney dysfunction. Most cases of HSES occur in infants from age 3 to 8 months of age, although it can also occur in older children. Individuals with HSES have extremely high body temperatures and multiple organ failures. This condition often causes long term neurological problems or death.

Diagnosis

CT scan, platelet count, hemoglobin level and renal function upon admission are not useful for an early diagnosis of HSES. However, the elevated liver enzymes and CK upon admission, hypotension in the early stage after admission with refractory acid-base disturbance to fluid resuscitation and vasopressor infusion are useful markers for an early HSES diagnosis and helpful to indicate starting intensive neurological treatment.

Treatment

There is no cure for HSES; however, different methods can be used to try to manage associated symptoms. Controlling brain edema might be the most important therapy to help HSES patients survive.

NIH genetic and rare disease info

• NIH info on Hemorrhagic shock and encephalopathy syndrome

Hemorrhagic shock and encephalopathy syndrome is a rare disease.

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