Document Type
Article
Peer Reviewed
1
Publication Date
5-4-2016
NLM Title Abbreviation
PLoS One
Journal/Book/Conference Title
PLoS One
PubMed ID
27144769
DOI of Published Version
10.1371/journal.pone.0154857
Start Page
0154857
End Page
0154857
Total Pages
7
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.
OBJECTIVE: To investigate the survival and lethal bleeding frequency in two strains of E-16 hemophilia A mice.
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.
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.
Keywords
OAfund, Hemophilia, Hemorrhage
Grant Number
NIH HD027748-19
Journal Article Version
Version of Record
Published Article/Book Citation
Staber JM, Pollpeter MJ (2016) Shortened Lifespan and Lethal Hemorrhage in a Hemophilia A Mouse Model. PLoS ONE 11(5): e0154857. doi:10.1371/journal.pone.0154857
Rights
Copyright © 2016 Staber, Pollpeter
Creative Commons License
This work is licensed under a Creative Commons Attribution 4.0 License.
URL
https://ir.uiowa.edu/pediatrics_pubs/4
Comments
This work was supported by the University of Iowa Children’s Miracle Network (JMS) and NIH HD027748-19, the Molecular and Cellular Research to Advance Child Health (JMS). The funders had no role in study design, data collection and analysis, decision to publish, or preparation of the manuscript