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Complications and mortality in hereditary hemorrhagic telangiectasia: a population-based study

Donaldson, J.W.; McKeever, Tricia M.; Hall, Ian P.; Hubbard, R.B.; Fogarty, Andrew W.

Complications and mortality in hereditary hemorrhagic telangiectasia: a population-based study Thumbnail


Authors

J.W. Donaldson

Tricia M. McKeever

Ian P. Hall

R.B. Hubbard

Andrew W. Fogarty



Abstract

OBJECTIVES:

Studies report that the risks of significant neurologic complications (including stroke, cerebral abscess, and migraine) and hemorrhagic sequelae are high in patients with hereditary hemorrhagic telangiectasia (HHT), and that life expectancy in this cohort is reduced. However, most published cohorts derive from specialist centers, which may be susceptible to bias.
METHODS:

We used a population-based approach to estimate the risks of developing neurologic and hemorrhagic complications of HHT, the association of a diagnosis of HHT with common cardiovascular and malignant comorbidities, and also long-term survival of those with the disease.
RESULTS:

From a UK primary care database of 3.5 million patients (The Health Improvement Network), we identified 675 cases with a diagnosis of HHT and compared them with 6,696 controls matched by age, sex, and primary care practice. Risks of stroke (odds ratio [OR] 1.8, 95% confidence interval [CI] 1.2-2.6), cerebral abscess (OR 30.0, CI 3.1-288), and migraine (OR 1.7, CI 1.3-2.2) were elevated over controls. Bleeding complications including epistaxis (OR 11.6, CI 9.1-14.7) and gastrointestinal hemorrhage (OR 6.1, CI 2.8-13.4) were more common in cases with HHT. Survival of cases with HHT was poorer than controls with a hazard ratio for death of 2.0 (CI 1.6-2.6) and a median age at death 3 years younger.
CONCLUSIONS:

Patients with HHT are at substantially increased risk of serious neurologic and hemorrhagic complications of the disease. Because a diagnosis of HHT is associated with a significantly poorer survival compared with those who have no disease, evaluation of new strategies to improve clinical management is required.

Citation

Donaldson, J., McKeever, T. M., Hall, I. P., Hubbard, R., & Fogarty, A. W. (2015). Complications and mortality in hereditary hemorrhagic telangiectasia: a population-based study. Neurology, 84(18), https://doi.org/10.1212/WNL.0000000000001538

Journal Article Type Article
Acceptance Date Jan 27, 2015
Publication Date May 5, 2015
Deposit Date Oct 27, 2016
Publicly Available Date Mar 29, 2024
Journal Neurology
Print ISSN 0028-3878
Electronic ISSN 1526-632X
Publisher American Academy of Neurology
Peer Reviewed Peer Reviewed
Volume 84
Issue 18
DOI https://doi.org/10.1212/WNL.0000000000001538
Public URL https://nottingham-repository.worktribe.com/output/752657
Publisher URL http://www.neurology.org/content/84/18/1886

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