National Center for Advancing and Translational Sciences Genetic and Rare Diseases Information Center, a program of the National Center for Advancing and Translational Sciences

Hemorrhagic shock and encephalopathy syndrome


Other Names:
HSES; Hemorrhagic shock and encephalopathy syndrome
Categories:
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. Individuals with HSES have extremely high body temperatures and multiple organ failures. This condition often causes long term neurological problems or death. The cause of the HSES is unknown. There is no cure for HSES; however, different methods can be used to try to manage associated symptoms.[1][2]
Last updated: 8/26/2016
The cause of HSES is unknown.[3] Some researchers believe that this condition is caused by a complex combination of genetic and environmental factors.[4][2] 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.[1] 
Last updated: 8/26/2016

These resources provide more information about this condition or associated symptoms. The in-depth resources contain medical and scientific language that may be hard to understand. You may want to review these resources with a medical professional.

In-Depth Information

  • The Monarch Initiative brings together data about this condition from humans and other species to help physicians and biomedical researchers. Monarch’s tools are designed to make it easier to compare the signs and symptoms (phenotypes) of different diseases and discover common features. This initiative is a collaboration between several academic institutions across the world and is funded by the National Institutes of Health. Visit the website to explore the biology of this condition.
  • PubMed is a searchable database of medical literature and lists journal articles that discuss Hemorrhagic shock and encephalopathy syndrome. Click on the link to view a sample search on this topic.

Questions sent to GARD may be posted here if the information could be helpful to others. We remove all identifying information when posting a question to protect your privacy. If you do not want your question posted, please let us know. Submit a new question

  • Is there any evidence that hemorrhagic shock and encephalopathy syndrome is genetic and passed from parent to child? Are people of certain religious or ethnic backgrounds more likely to develop this syndrome? See answer


  1. Palumbo EJ. Hemorrhagic shock and encephalopathy syndrome. Merck Manual for Healthcare Professionals. May 2014; http://www.merckmanuals.com/professional/pediatrics/miscellaneous-disorders-in-infants-and-children/hemorrhagic-shock-and-encephalopathy-syndrome-hses.
  2. Ichiro Kuki, Masashi Shiomi, Shin Okazaki, Hisashi Kawawaki, Kiyotaka Tomiwa, Kiyoko Amo, Masao Togawa, Junichi Ishikawa, Hiroshi Rinka. Characteristic Neuroradiologic Features in Hemorrhagic Shock and Encephalopathy Syndrome. Journal of Child Neurology. Mar 2015; 30(4):468-475. http://www.ncbi.nlm.nih.gov/pubmed/?term=25512363.
  3. Rinka H, Yoshida T, Kubota T, Tsuruwa M, Fuke A, Yoshimoto A, Kan M, Miyazaki D, Arimoto H, Miyaichi T, Kaji A, Miyamoto S, Kuki I, Shiomi M. Hemorrhagic shock and encephalopathy syndrome--the markers for an early HSES diagnosis. BMC Pediatr. October 16, 2008; 8:43. http://www.ncbi.nlm.nih.gov/pubmed/18922188. Accessed 5/19/2009.
  4. S Sofer, B Yerushalmi, E Shahak, T Berenstein, and H Schulman. Possible aetiology of haemorrhagic shock and encephalopathy syndrome in the Negev area of Israel. Archives of Disease in Childhood. October 1996; 75:4. http://www.pubmedcentral.nih.gov/articlerender.fcgi?artid=1511758. Accessed 5/19/2009.