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- 저자 : ( Marcelo Martin Serra ) (검색결과 1 건)
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( Cristina Maria Elizondo ),( Carla Scarano ),( Constanza Franceschini ),( Nora Angelica Fuentes ),( Hernan Diego Giunta ),( Liliana Rojas ),( Marcelo Martin Serra ) 대한내과학회 2014 대한내과학회 추계학술대회 Vol.2014 No.1
Background: Hereditary hemorrhagic telangiectasia (HHT) is an autosomal dominant systemic disorder that affects 1/5000-8000 individuals worldwide. It is characterized by recurrent epistaxis, mucocutaneous telangiectasias and arteriovenous malformations (AVMs) in organs such as lung, liver, central nervous system (CNS) and gastrointestinal tract (GI); their clinical manifestations and possible complications added, frequently alters quality of life of these patients. Objective: To identify associated factors with lower quality of life in adult patients with HHT. Methods: Cross-sectional study, from 2010 to 2013, of prospectively evaluated patients, from the institutional registry of HHT at Hospital Italiano, Buenos Aires. Quality of life (QoL) was measured with the EuroQol and visual analog scale (AVS) validated for the country. Presence of epistaxis, anemia, AVMs in CNS, lung, liver, GI tract, symptoms and related complications were evaluated. Results: 127 patients were included: 33% were men, with a median age of 47 years (IQR:16). Median for QoL measured by the VAS was 69 (ICR:20.3). From the total, 96,1% presented epistaxis, from which 33,9% had severe epistaxis; 60% had anemia, 65,15% pulmonary AVMs, 39,4% were symptomatic; 19,6% had AVMs in CNS; 73,5% had hepatic AVMs and 63% had GI AVMs from which 27,6% presented symptoms. Signifi cant association was found between de median of QoL measured by VAS with: anemia (62 vs. non-anemic 77.8, p=0.001), pulmonary AVMs (62.3 vs. 71.27 without pulmonary AVMs, p=0.056), hepatic AVMs (64.67 vs. 78.15 without AVMs, p=0.012), GI AVMs (60 vs. 70 without AVMs, p=0.043) and correlation coefficient age was -0.349 age (p<0.001). Conclusions: Factors associated to decrease QoL in HHT were anemia and hepatic, pulmonary and GI AVMs. There was no difference in QoL related to epistaxis, but this could be attributed to the low number of patients without epistaxis.
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