Life expectancy in patients with hereditary haemorrhagic telangiectasia.
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.
Sabbà
C
C
Department of Internal Medicine and Public Health, University of Bari, Bari, Italy. c.sabba@dimimp.uniba.it
Pasculli
G
G
Suppressa
P
P
D'Ovidio
F
F
Lenato
G Mariano
GM
Resta
F
F
Assennato
G
G
Guanti
G
G
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*