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An epistaxis severity score for hereditary hemorrhagic telangiectasia

Authors

  • Jeffrey B. Hoag MD, MS,

    Corresponding author
    1. Department of Medicine , the Drexel University College of Medicine, Philadelphia, Pennsylvania
    • Division of Pulmonary, Critical Care, and Sleep Medicine, Drexel University College of Medicine, 245 N. 15th St., Mail Stop 107, NCB 12th Floor, Philadelphia, PA 19102
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  • Peter Terry MD, MA,

    1. Department of Medicine , The Johns Hopkins University School of Medicine, Baltimore, Maryland, U.S.A.
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  • Sally Mitchell MD,

    1. Department of Interventional Radiology , The Johns Hopkins University School of Medicine, Baltimore, Maryland, U.S.A.
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  • Douglas Reh MD,

    1. Department of Otolaryngology , The Johns Hopkins University School of Medicine, Baltimore, Maryland, U.S.A.
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  • Christian A. Merlo MD, MPH

    1. Department of Medicine , The Johns Hopkins University School of Medicine, Baltimore, Maryland, U.S.A.
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  • This project was provided by the HHT Foundation International, Inc. The authors have no other funding, financial relationships, or conflicts of interest to disclose.

Abstract

Objectives/Hypothesis:

Hereditary hemorrhagic telangiectasia (HHT)-related epistaxis leads to alterations in social functioning and quality of life. Although more than 95% experience epistaxis, there is considerable variability of severity. Because no standardized method exists to measure epistaxis severity, the purpose of this study was to determine factors associated with patient-reported severity to develop a severity score.

Study Design:

Prospective, survey-based study.

Methods:

HHT care providers and a focus group of patients were interviewed to determine epistaxis-associated factors. From this, an electronic survey was developed and administered to patients with HHT. Descriptive analyses were performed with calculations of means and medians for continuous and proportions for categorical variables. Multiple ordinal logistic and linear regression models were developed to determine risk factors for epistaxis severity.

Results:

Nine hundred respondents from 21 countries were included. Eight hundred fifty-five (95%) subjects reported epistaxis. The mean (standard deviation) age was 52.1 (13.9) years, and 61.4% were female. Independently associated risk factors for self-reported epistaxis severity included epistaxis frequency (odds ratio [OR] 1.57), duration (OR 2.17), intensity (OR 2.45), need for transfusion (OR 2.74), anemia (OR 1.44), and aggressiveness of treatment required (OR 1.53, P < .001 for all).

Conclusions:

Risk factors for increasing epistaxis severity in patients with HHT include frequency, duration, and intensity of episodes; invasiveness of prior therapy required to stop epistaxis; anemia; and the need for blood transfusion. From these factors, an epistaxis severity score will be presented. Laryngoscope, 2010

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