Reversal of central nervous system involvement in hemolytic uremic syndrome by use of plasma exchanges.
A 12-year-old girl presented with weakness, diplopia, and lethargy after a prodrome of gastroenteritis. Laboratory studies were compatible with a diagnosis of hemolytic uremic syndrome. She developed seizures that were controlled by diphenylhydantoin and valium. In spite of peritoneal dialysis and fresh frozen plasma infusions, she progressed to a left hemiplegia associated with a brain scan finding of decreased blood flow in the right middle cerebral artery perfusion area. A 5 liter whole blood exchange transfusion did not improve the neurological status or low platelet count. Daily plasma exchanges with fresh frozen plasma replacement resulted in normal platelet count within 48 hours and was followed by progressive improvement in neurological status. Platelet agglutinating factor decreased to control levels. A repeat brain scan was normal.
D000941:Antigens / Q000032:analysis; D002648:Child; D005169:Factor VIII / Q000032:analysis; D005260:Female; D006429:Hemiplegia / Q000209:etiology / Q000628:therapy*; D006463:Hemolytic-Uremic Syndrome / Q000150:complications / Q000628:therapy*; D006801:Humans; D010951:Plasma Exchange; D010972:Platelet Activating Factor / Q000032:analysis; D012640:Seizures / Q000209:etiology / Q000628:therapy; D014841:von Willebrand Factor / Q000276:immunology
D000941:Antigens; D010972:Platelet Activating Factor; C029417:Von Willebrand antigen; D014841:von Willebrand Factor; D005169:Factor VIII