title: An evaluation of the severity and progression of epistaxis in hereditary hemorrhagic telangiectasia 1 versus hereditary hemorrhagic telangiectasia 2.
abstract: OBJECTIVE
  Hereditary hemorrhagic telangiectasia (HHT) is an autosomal dominant vascular dysplasia whose hallmark symptom is spontaneous recurrent epistaxis. Two major genetic subtypes of this syndrome are HHT1 and HHT2. Severity of epistaxis ranges from occasional low-volume bleeding to frequent large-volume hemorrhage. This study evaluated the severity and progression of epistaxis in HHT1 versus HHT2.
  
  
  METHODS
  Retrospective cohort study.
  
  
  METHODS
  A retrospective chart review was performed for 183 genotyped HHT patients seen at our center from 2010 to 2013. Data collected included epistaxis severity score (ESS), age of epistaxis onset, number and type of treatments, age at which treatments were sought, complete blood count values, ferritin, number of telangiectases, blood transfusions, iron therapy history, and patient demographics.
  
  
  RESULTS
  115 subjects with HHT2 were compared to 68 with HHT1. Subjects with HHT2 had a higher ESS compared to HHT1 (P = .043) and a later age of onset of epistaxis (P = .005). HHT2 subjects were more likely to use oral iron (P = .032) and were more likely to seek interventions to control their epistaxis (P = .029).
  
  
  CONCLUSIONS
  HHT2 is associated with more severe epistaxis and a subsequent higher rate of interventions, requiring more aggressive therapy as compared to HHT1.
  
  
  METHODS
  4.
authors.affiliation: University of Utah School of Medicine, Salt Lake City, Utah.
authors.forename: Benjamin N
authors.initials: BN
authors.lastname: Hunter
authors.affiliation: University of Utah School of Medicine, Salt Lake City, Utah.
authors.forename: Benjamin H
authors.initials: BH
authors.lastname: Timmins
authors.affiliation: Department of Pathology, University of Utah, Salt Lake City, Utah.|Department of Radiology, University of Utah, Salt Lake City, Utah.
authors.forename: Jamie
authors.initials: J
authors.lastname: McDonald
authors.affiliation: Division of Cardiovascular Medicine, Pediatric Cardiology, Molecular Medicine Program, University of Utah, Salt Lake City, Utah.
authors.forename: Kevin J
authors.initials: KJ
authors.lastname: Whitehead
authors.affiliation: Division of Otolaryngology-Head & Neck Surgery, University of Utah, Salt Lake City, Utah, U.S.A.
authors.forename: P Daniel
authors.initials: PD
authors.lastname: Ward
authors.affiliation: Division of Otolaryngology-Head & Neck Surgery, University of Utah, Salt Lake City, Utah, U.S.A.
authors.forename: Kevin F
authors.initials: KF
authors.lastname: Wilson
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D002648:Child; D002675:Child, Preschool; D015331:Cohort Studies; D018450:Disease Progression; D004844:Epistaxis / Q000209:etiology*; D005260:Female; D006801:Humans; D007223:Infant; D008297:Male; D008875:Middle Aged; D012189:Retrospective Studies; D012720:Severity of Illness Index; D013683:Telangiectasia, Hereditary Hemorrhagic / Q000150:complications*; D055815:Young Adult
keywords: HHT1; HHT2; Hereditary hemorrhagic telangiectasia; Osler-Weber-Rendu disease; Osler-Weber-Rendu syndrome; Young's procedure; epistaxis treatment; laser photocoagulation; nasal closure