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.
Tricia M. McKeever
Ian P. Hall
Andrew W. Fogarty
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.
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.
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.
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.
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||Oct 27, 2016|
|Publisher||American Academy of Neurology|
|Peer Reviewed||Peer Reviewed|
|Copyright Statement||Copyright information regarding this work can be found at the following address: http://eprints.nottingham.ac.uk/end_user_agreement.pdf|
Copyright information regarding this work can be found at the following address: http://eprints.nottingham.ac.uk/end_user_agreement.pdf
You might also like
Genome-Wide Association Study of Susceptibility to Idiopathic Pulmonary Fibrosis
A data analysis of the Chilean housing stock and the development of modelling archetypes
Setting the standard: The acceptability of kitchen ventilation for the English housing stock