title: The challenge of managing hemophilia A and STEC-induced hemolytic uremic syndrome.
abstract: BACKGROUND
  The hemolytic uremic syndrome (HUS) is a thrombotic microangiopathy leading to acute kidney injury in children. In most cases it is triggered by an infection caused by Shiga-like toxin-producing Escherichia coli (STEC). Endothelial damage plays a central role in the pathogenesis of disease. Hemophilia A is a genetic disorder leading to factor VIII (FVIII) deficiency, an important factor in the coagulation system.
  
  
  METHODS
  Here we describe a hemophilia A patient who developed HUS due to a STEC O26 infection. The patient developed not only acute kidney injury, but also severe gastro-intestinal and neurological complications. Increased amounts of recombinant FVIII (rFVIII) had to be administered during the acute phase of the disease to reach acceptable blood levels of FVIII, in order to control the hemorrhagic colitis and to prevent severe neurological complications.
  
  
  CONCLUSIONS
  The patient's treatment schedule of rFVIII during the HUS period was a serious challenge, and we cannot exclude that it contributed to the severity of the HUS by enhancing the thrombotic microangiopathic process. The role of factor VIII administration in the severe outcome of this disease is discussed.
authors.affiliation: Department of Pediatric Nephrology, Radboud University Nijmegen Medical Centre, P.O. Box 9101, 6500 HB Nijmegen, the Netherlands.
authors.forename: Dineke
authors.initials: D
authors.lastname: Westra
authors.forename: Eiske M
authors.initials: EM
authors.lastname: Dorresteijn
authors.forename: Auke
authors.initials: A
authors.lastname: Beishuizen
authors.forename: Lambert P W J
authors.initials: LP
authors.lastname: van den Heuvel
authors.forename: Paul P T
authors.initials: PP
authors.lastname: Brons
authors.forename: Nicole C A J
authors.initials: NC
authors.lastname: van de Kar
mesh_terms: D001002:Anuria / Q000209:etiology; D002675:Child, Preschool; D003029:Coagulants / Q000627:therapeutic use; D003251:Constriction, Pathologic / Q000209:etiology / Q000601:surgery; D004830:Epilepsy, Tonic-Clonic / Q000209:etiology; D004927:Escherichia coli Infections / Q000150:complications*; D005169:Factor VIII / Q000627:therapeutic use; D006471:Gastrointestinal Hemorrhage / Q000209:etiology*; D006440:Hemofiltration; D006463:Hemolytic-Uremic Syndrome / Q000382:microbiology / Q000628:therapy*; D006467:Hemophilia A / Q000150:complications / Q000175:diagnosis / Q000188:drug therapy*; D006801:Humans; D045823:Ileus / Q000209:etiology; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D010956:Plasmapheresis; D012007:Rectum; D054323:Shiga-Toxigenic Escherichia coli; D012810:Sigmoid Diseases / Q000209:etiology / Q000601:surgery
chemical_list: D003029:Coagulants; C577506:recombinant factor VIII N8; D005169:Factor VIII