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Shortened Lifespan and Lethal Hemorrhage in a Hemophilia A Mouse Model
Journal article   Open access   Peer reviewed

Shortened Lifespan and Lethal Hemorrhage in a Hemophilia A Mouse Model

Janice M Staber and Molly J Pollpeter
PLoS One, Vol.11(5), pp.0154857-0154857
05/04/2016
DOI: 10.1371/journal.pone.0154857
PMCID: PMC4856382
PMID: 27144769
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Published (Version of record)CC BY V4.0 Open Access
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https://doi.org/10.1371/journal.pone.0154857View
Published (Version of record)PLoS One 11(5): e0154857.

Abstract

BACKGROUND: 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. <p>OBJECTIVE: To investigate the survival and lethal bleeding frequency in two strains of E-16 hemophilia A mice.</p> <p>METHODS: 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.</p> <p>RESULTS AND CONCLUSIONS: 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.
Hematology Hemophilia Internal Medicine Pediatrics OAfund Hemorrhage Factor VIII recombinant factor VIII SQ mice

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