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

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

Grant Number

NIH HD027748-19

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

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

Creative Commons License
This work is licensed under a Creative Commons Attribution 4.0 License.

Included in

Pediatrics Commons

Share

COinS
 

URL

https://ir.uiowa.edu/pediatrics_pubs/4