title: Life expectancy in patients with hereditary haemorrhagic telangiectasia.
abstract: BACKGROUND
  There are few data on life expectancy in patients with hereditary haemorrhagic telangiectasia (HHT), a disorder with life-threatening complications.
  
  
  METHODS
  Seventy HHT patients provided data on age and age at death of their HHT-affected parent, which was compared with that of the parent's non-affected partner.
  
  
  RESULTS
  At the time of the study, 40 HHT parents (57.1%) vs. 36 (51.4%) non-HHT parents had died (p = 0.404). Median age at death was lower in HHT vs. non-HHT parents (63.2 vs. 70.0 years, respectively). The mortality of HHT parents showed an early peak in the under 50s and a late peak at 60-79 years. HHT was the main risk factor influencing life expectancy after 30 years (p < 0.05). No differences in survival probability were found in HHT patients with respect to sex (p = 0.37), or ENG vs. ALK-1 genotype (p < 0.9).
  
  
  CONCLUSIONS
  Life expectancy appears to be significantly lower in HHT patients than in their partners. Prevention of HHT complications with screening programs could increase life expectancy.
authors.affiliation: Department of Internal Medicine and Public Health, University of Bari, Bari, Italy. c.sabba@dimimp.uniba.it
authors.forename: C
authors.initials: C
authors.lastname: Sabbà
authors.forename: G
authors.initials: G
authors.lastname: Pasculli
authors.forename: P
authors.initials: P
authors.lastname: Suppressa
authors.forename: F
authors.initials: F
authors.lastname: D'Ovidio
authors.forename: G Mariano
authors.initials: GM
authors.lastname: Lenato
authors.forename: F
authors.initials: F
authors.lastname: Resta
authors.forename: G
authors.initials: G
authors.lastname: Assennato
authors.forename: G
authors.initials: G
authors.lastname: Guanti
mesh_terms: D000328:Adult; D000367:Age Factors; D000368:Aged; D000369:Aged, 80 and over; D002423:Cause of Death; D005260:Female; D006801:Humans; D007558:Italy / Q000453:epidemiology; D008017:Life Expectancy; D008297:Male; D008875:Middle Aged; D012189:Retrospective Studies; D013683:Telangiectasia, Hereditary Hemorrhagic / Q000401:mortality*