title: Shortened Lifespan and Lethal Hemorrhage in a Hemophilia A Mouse Model.
abstract: Hemophilia A animal models have helped advance our understanding of factor VIII deficiency. Previously, factor VIII deficient mouse models were reported to have a normal life span without spontaneous bleeds. However, the bleeding frequency and survival in these animals has not been thoroughly evaluated.
  
  
  
  To investigate the survival and lethal bleeding frequency in two strains of E-16 hemophilia A mice.
  
  
  
  We prospectively studied factor VIII deficient hemizygous affected males (n = 83) and homozygous affected females (n = 55) for survival and bleeding frequency. Animals were evaluated for presence and location of bleeds as potential cause of death.
  
  
  
  Hemophilia A mice had a median survival of 254 days, which is significantly shortened compared to wild type controls (p < 0.0001). In addition, the hemophilia A mice experienced hemorrhage in several tissues. This previously-underappreciated shortened survival in the hemophilia A murine model provides new outcomes for investigation of therapeutics and also reflects the shortened lifespan of patients if left untreated.
authors.affiliation: Stead Family Department of Pediatrics, Carver College of Medicine, University of Iowa, Iowa City, IA, 52242, United States of America.
authors.forename: Janice M
authors.identifier: 0000-0002-2738-4206
authors.initials: JM
authors.lastname: Staber
authors.affiliation: Stead Family Department of Pediatrics, Carver College of Medicine, University of Iowa, Iowa City, IA, 52242, United States of America.
authors.forename: Molly J
authors.initials: MJ
authors.lastname: Pollpeter
mesh_terms: D000818:Animals; D004195:Disease Models, Animal; D005169:Factor VIII / Q000378:metabolism; D005260:Female; D006467:Hemophilia A / Q000378:metabolism / Q000473:pathology*; D006470:Hemorrhage / Q000378:metabolism / Q000473:pathology*; D008297:Male; D051379:Mice; D011446:Prospective Studies
chemical_list: C427184:recombinant factor VIII SQ; D005169:Factor VIII