Abstract Number: PB0702
Meeting: ISTH 2021 Congress
Background: Hereditary hemorrhagic telangiectasia (HHT) or Rendu-Osler-Weber disease is an autosomal dominant disease, penetrance related with age and with an important variability in the manifestations clinics among affected individuals, including between patients of the same family. The vascular manifestations are the presence in skin and mucous membranes telangiectasias, recurrent and spontaneous epistaxis and arteriovenous malformations on different organs.
Aims: The main objective of this investigation is to obtain epidemiological information and clinical data of patients with HHT and determine its prevalence in Costa Rica.
Methods: Data was obtained from the referral main hospitals in San Jose, Costa Rica. It was analyzed from October 1989 until 2017. Statistical support was provided by the Medical School of the University of Costa Rica utilizing the Epi.Info. 7.1 (CDC, Atlanta USA) package. The basic data were collected in a questionnaire, following the criteria of Curaçao.
Results: Approximate frequency of 2.51 cases per year was obtained, where age average of the patients was 61years, with a diagnosis of over 40 years. Geographically, 83.3% of the cases were reported in the province of San José, 8.3% in Puntarenas and 4.16% in two areas (Limón and Alajuela), Figure 1.The main reason for consultation was a history of heavy bleeding, and chronic persistent epistaxis in most patients, or upper gastro-intestinal bleeding. All cases were treated with iron replacement and/or blood products transfusion, and some with antifibrinolytic therapy or anticoagulation.
Conclusions: This is the first study of HHT in Costa Rica, there is a signicant underreporting of the disease. There is no record of the care received by HHT patients at the first and second levels of care in the rest of the country. It is important to spread knowledge and educate health personnel about this condition, to try to reduce their underreporting and improve the quality of life of patients.
To cite this abstract in AMA style:Salazar-Sanchez L, Leiva-Gabriel K, Hernandez-Salazar BI, Salazar-Chacon C. Hereditary Haemorrhagic Telangiectasia (HHT) or Rendu-Osler Weber Disease as an Important Rare Haemorrhagic Disorder in Costa Rica: A Report of Costa Rica [abstract]. Res Pract Thromb Haemost. 2021; 5 (Suppl 2). https://abstracts.isth.org/abstract/hereditary-haemorrhagic-telangiectasia-hht-or-rendu-osler-weber-disease-as-an-important-rare-haemorrhagic-disorder-in-costa-rica-a-report-of-costa-rica/. Accessed November 29, 2021.
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