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Otol."],"published-print":{"date-parts":[[2016,8]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec id=\"S0022215116008422_sec_a1\"><jats:title>Objective:<\/jats:title><jats:p>To determine whether patients with hereditary haemorrhagic telangiectasia were being screened according to international guidelines, and to review recent evidence in order to provide up-to-date guidelines for the initial systemic management of hereditary haemorrhagic telangiectasia.<\/jats:p><\/jats:sec><jats:sec id=\"S0022215116008422_sec_a2\"><jats:title>Methods:<\/jats:title><jats:p>A retrospective case note analysis was conducted, assessing patients in terms of screening for: genetics, cerebral arteriovenous malformations, pulmonary and hepatic arteriovenous malformations, and gastrointestinal telangiectasia. Databases searched included Medline, the Cumulative Index to Nursing and Allied Health Literature, and Embase.<\/jats:p><\/jats:sec><jats:sec id=\"S0022215116008422_sec_a3\"><jats:title>Results:<\/jats:title><jats:p>Screening investigations were most frequently performed for hepatic arteriovenous malformations and least frequently for genetics. Recent data suggest avoiding routine genetic and cerebral arteriovenous malformation screening because of treatment morbidities; performing high-resolution chest computed tomography for pulmonary arteriovenous malformation screening; using capsule endoscopy (if possible) to reduce complications from upper gastrointestinal endoscopy; and omitting routine liver enzyme testing in favour of Doppler ultrasound.<\/jats:p><\/jats:sec><jats:sec id=\"S0022215116008422_sec_a4\"><jats:title>Conclusion:<\/jats:title><jats:p>Opportunities for systemic arteriovenous malformation screening are frequently overlooked. 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