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Links Between Strokes and Hereditary Hemorrhagic Telangiectasia: A Population-Based Study

  • Farah N. Chowdhury (a1), G. Sanjaya Chandrarathne (a1), Kristopher D. Masilamani (a1) (a2), Jennifer T. N. LaBranche (a1), Shaun Malo (a3), Lawrence W. Svenson (a3) (a4) (a5) (a6), Thomas Jeerakathil (a7) and Dilini P. Vethanayagam (a1) (a8)...

Abstract

Background

Hereditary hemorrhagic telangiectasia (HHT) is a disease of abnormal vasculature where patients are predisposed to strokes of multiple etiologies. We assessed yearly stroke incidence among Albertans with HHT and compared with the general population. Given the tendency for stroke in HHT patients, we expected HHT patients to have higher stroke incidence, in particular at younger ages.

Methods

Population-based administrative health data on inpatient and ambulatory admissions were extracted over a 16-year period using International Classification of Diseases (ICD)-9 and ICD-10, Canada codes. We analyzed overall occurrence of strokes in Alberta by age, gender, stroke subtype, and diagnosis of HHT.

Results

The age-standardized incidence rate of stroke in HHT was 450 per 100,000 compared with 260 per 100,000 in the general population with a rate ratio of 1.73 (95% confidence interval (CI) [1.046-2.842]). This study found a higher HHT prevalence in Alberta (1 in 3800) compared to the world average of 1 in 5000. Women were also more likely to be diagnosed with HHT, with a 3.25:1 female gender preponderance in the yearly incidence.

Interpretation

This study not only shows that HHT patients are at higher risk of having a stroke but also quantifies that risk using an age-adjusted metric in Alberta. This province has a higher than expected disease burden of HHT, with the majority of cases affecting women. Our study found that acute ischemic strokes and transient ischemic attacks are far more common than hemorrhage in HHT. As HHT is a rare, multi-system, chronic disease, these patients should be referred to an HHT Centre of Excellence.

Les liens existant entre les AVC et la maladie de Rendu-Osler-Weber: une étude basée sur la population albertaine.

Contexte

La maladie de Rendu-Osler-Weber (MROW) est une maladie caractérisée par une anomalie du système vasculaire. Les patients qui en sont atteints deviennent prédisposés à être victimes d’AVC présentant de multiples étiologies. À cet égard, nous avons cherché à évaluer l’incidence annuelle d’AVC au sein de patients albertains atteints de la MROW comparativement à l’incidence annuelle d’AVC au sein de la population de cette province. Étant donné la prédisposition évoquée ci-haut, nous nous attendions à ce que l’incidence d’AVC chez des patients atteints de cette maladie soit plus élevée, particulièrement à un plus jeune âge.

Méthodes

Des données administrative liées à la santé et basées sur une population déterminée ont été extraites au moyen des codes ICD-9 et ICD-10-CA. Elles concernaient des patients hospitalisés et ambulatoires au cours d’une période de 16 ans. Nous avons ensuite analysé l’incidence d’ensemble des AVC en Alberta en fonction de l’âge, du sexe, des sous-types d’AVC et de l’établissement préalable d’un diagnostic de MROW.

Résultats

Le taux d’incidence normalisé selon l’âge des AVC chez les patients atteints de la MROW était de 450 pour 100 000 comparativement à 260 pour 100 000 au sein de la population générale. Le ratio des taux, lui, était de 1,73 (IC 95 % [1,046;2,842]). Notre étude a aussi constaté une prévalence plus élevée de la MROW en Alberta (1 sur 3 800) comparativement à la moyenne mondiale, qui est de 1 sur 5 000. Fait à souligner, les femmes étaient plus susceptibles de recevoir un diagnostic de MROW (prépondérance féminine de 3,25:1 sur une base annuelle).

Interprétation

Cette étude a montré que les patients atteints de la MROW étaient non seulement plus à risque d’être victimes d’un AVC mais a aussi quantifié ce risque au moyen d’un outil de mesure ajusté pour l’Alberta en fonction de l’âge. Cette province donne à voir une incidence plus élevée de MROW que ce à quoi on pouvait s’attendre, la majorité des patients étant de sexe féminin. Notre étude a en outre montré que les AVC ischémiques aigus et les accidents ischémiques transitoires (AIT) sont nettement plus courants que les hémorragies lorsqu’on est atteint de la MROW. Considérant qu’il s’agit d’une maladie chronique rare de nature multi-systémique, ceux et celles qui en sont atteints devraient être dirigés vers un centre d’excellence spécialisé dans son traitement.

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Copyright

Corresponding author

Correspondence to: Dilini P. Vethanayagam, 3-105 Clinical Sciences Building, University of Alberta, 11350-83 Avenue, Edmonton, Alberta, Canada T6G 2G3. Email: dilini@ualberta.ca

References

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