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C. Sabbà, G. Pasculli, P. Suppressa, F. D'Ovidio, G. Mariano Lenato, F. Resta, G. Assennato, G. Guanti, Life expectancy in patients with hereditary haemorrhagic telangiectasia, QJM: An International Journal of Medicine, Volume 99, Issue 5, May 2006, Pages 327–334, https://doi.org/10.1093/qjmed/hcl037
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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).
Discussion: 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.
