Haemorrhagic shock and encephalopathy

Abstract

Two infants are described with a fulminant disorder characterised by profound circulatory collaps and shock, generalised convulsions and unremitting coma, bleeding due to severe DIC, fever, diarrhoea, metabolic acidosis and renal and hepatic failure. Both infants died shortly after onset of the symptoms. Autopsy mainly revealed haemorrhages in different organs, anoxaemic lesions in the brain and a normal structure of liver and pancreas. No causative agent could be demonstrated. We believe that both patients suffered from haemorrhagic shock and encephalopathy, a mostly fatal disorder which has recently been described. Although the clinical and biochemical features are very distinctive, this syndrome is probably heterogeneous and its differentiation from some other disorders may be difficult. Its pathogenesis is unknown but there are some indications that intravascular activity of trypsin may play a role. During a study of the two families we obtained abnormal results of immunologic tests in most members: the interpretation of this finding remains conjectural. Haemorrhagic shock and encephalopathy may occur more frequently than the restricted literature on this subject suggests. Future studies will have to deal with the question of identity and pathogenesis.

Haemorrhagic shock encephalopathy syndrome in the British Isles.

“…A child had to be under 16 years of age with acute onset of: (1) encephalopathy, (2) shock, (3) disseminated intravascular coagulation, (4) diarrhoea (may be bloody), (5) falling haemoglobin concentration and platelet counts, (6) acidosis, (7) raised hepatocellular enzymes, (8) renal function impairment, and (9) negative cultures of blood and cerebrospinal fluid.…”

Developing a mouse model of acute encephalopathy using low-dose lipopolysaccharide injection and hyperthermia treatment

“…2,10 In severe cases, diffuse necrotic changes and brain liquefaction, a condition termed as "respirator brain," are evident. 8,[11][12][13][14][15][16][17] In addition, both ANE and HSES lead to the failure of multiple organs associated with disseminated intravascular coagulation, including the liver and kidney. 2 Although it has been hypothesized that upregulation of proinflammatory cytokines is the key factor of AE, 18 its pathogenesis and neurophysiology remain largely unclear.…”