Life expectancy in patients with hereditary haemorrhagic telangiectasia

Authors: Sabbà, C.; Pasculli, G.; Suppressa, P.; D'Ovidio, F.; Lenato, G. Mariano; Resta, F.; Assennato, G.; Guanti, G.

Source: QJM: An International Journal of Medicine, Volume 99, Number 5, May 2006 , pp. 327-334(8)

Publisher: Oxford University Press

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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.

Document Type: Research article

DOI: http://dx.doi.org/10.1093/qjmed/hcl037

Publication date: 2006-05-01

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QJM is a long-established, leading general medical journal. It focuses on internal medicine and publishes peer-reviewed articles which promote medical science and practice. Published monthly, QJM includes original papers, editorials, reviews, commentary papers to air controversial issues, and a correspondence column.