2017
DOI: 10.1007/s10620-017-4719-3
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Gastrointestinal Manifestations of Hereditary Hemorrhagic Telangiectasia (HHT): A Systematic Review of the Literature

Abstract: Hereditary hemorrhagic telangiectasia (HHT), also called Osler–Weber–Rendu syndrome, is an autosomal dominant genetic disease that affects the vasculature of numerous organs. The prevalence of HHT is estimated to be between 1.5 and 2 persons per 10,000. While there is still much to learn about this condition, there is an increasing understanding its underlying pathophysiology, genetic basis, presentations, and management. Recognizing that the clinical manifestations of HHT can involve a number of organ systems… Show more

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Cited by 92 publications
(49 citation statements)
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“…Many cases of anemia are misattributed to overt epistaxis instead of attributing them to GI bleeding among patients with HHT [16,31]. In fact, we have not found significant differences in epistaxis severity measured by the ESS between patients with and without GI involvement.…”
Section: Discussioncontrasting
confidence: 65%
“…Many cases of anemia are misattributed to overt epistaxis instead of attributing them to GI bleeding among patients with HHT [16,31]. In fact, we have not found significant differences in epistaxis severity measured by the ESS between patients with and without GI involvement.…”
Section: Discussioncontrasting
confidence: 65%
“…Telangiectasias in the gastrointestinal tract are present in more or less 15 to 30% of the patients, typically after the age of 40 16 . They are present in the stomach, the small and large intestine.…”
Section: Clinical Manifestationsmentioning
confidence: 99%
“…GI bleeding can be stopped by either resection or embolectomy. Unfortunately, HHT is not preventable [13].…”
Section: Hereditary Hemorrhagic Telangiectasia (Hht)mentioning
confidence: 99%