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Surgery of hereditary hemorrhagic telangiectasia with severe refractory gastrointestinal bleeding: A case report of a rare condition

INTRODUCTION: Hereditary hemorrhagic telangiectasia (HHT) is an autosomal dominant genetic disease that affects the vasculature of numerous organs. HHT is characterized by the presence of multiple arteriovenous malformations that lack intervening capillaries and result in direct connections between...

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Autores principales: Lim, Dae Ro, Kim, Da Bin, Kim, Hee Kyung, Shin, Eung Jin
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Elsevier 2020
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7317164/
https://www.ncbi.nlm.nih.gov/pubmed/32698275
http://dx.doi.org/10.1016/j.ijscr.2020.06.057
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author Lim, Dae Ro
Kim, Da Bin
Kim, Hee Kyung
Shin, Eung Jin
author_facet Lim, Dae Ro
Kim, Da Bin
Kim, Hee Kyung
Shin, Eung Jin
author_sort Lim, Dae Ro
collection PubMed
description INTRODUCTION: Hereditary hemorrhagic telangiectasia (HHT) is an autosomal dominant genetic disease that affects the vasculature of numerous organs. HHT is characterized by the presence of multiple arteriovenous malformations that lack intervening capillaries and result in direct connections between arteries and veins. PRESENTATION OF CASE: A 58-year-old South Korean female was admitted presenting with severe anemia, dizziness, and intermittent hematochezia. The patient had experienced chronic severe refractory gastrointestinal bleeding for years. She had an intermittent spontaneous epistaxis, telangiectasias in the oral cavity (tongue), and hepatic arteriovenous malformations and gastrointestinal telangiectasia. She underwent a segmental resection of a segment of the ileum and cecum (about 77 cm). After surgery, the patient experienced no more drops in hemoglobin count or symptoms of anemia and melena. She is living a normal life. CONCLUSION: The present case report is a surgical case of undiagnosed HHT with chronic refractory gastrointestinal bleeding. Surgical treatment is rarely required for gastrointestinal bleeding unless nonsurgical methods such as endoscopy/colonoscopy and angiography have failed.
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spelling pubmed-73171642020-06-30 Surgery of hereditary hemorrhagic telangiectasia with severe refractory gastrointestinal bleeding: A case report of a rare condition Lim, Dae Ro Kim, Da Bin Kim, Hee Kyung Shin, Eung Jin Int J Surg Case Rep Article INTRODUCTION: Hereditary hemorrhagic telangiectasia (HHT) is an autosomal dominant genetic disease that affects the vasculature of numerous organs. HHT is characterized by the presence of multiple arteriovenous malformations that lack intervening capillaries and result in direct connections between arteries and veins. PRESENTATION OF CASE: A 58-year-old South Korean female was admitted presenting with severe anemia, dizziness, and intermittent hematochezia. The patient had experienced chronic severe refractory gastrointestinal bleeding for years. She had an intermittent spontaneous epistaxis, telangiectasias in the oral cavity (tongue), and hepatic arteriovenous malformations and gastrointestinal telangiectasia. She underwent a segmental resection of a segment of the ileum and cecum (about 77 cm). After surgery, the patient experienced no more drops in hemoglobin count or symptoms of anemia and melena. She is living a normal life. CONCLUSION: The present case report is a surgical case of undiagnosed HHT with chronic refractory gastrointestinal bleeding. Surgical treatment is rarely required for gastrointestinal bleeding unless nonsurgical methods such as endoscopy/colonoscopy and angiography have failed. Elsevier 2020-06-13 /pmc/articles/PMC7317164/ /pubmed/32698275 http://dx.doi.org/10.1016/j.ijscr.2020.06.057 Text en © 2020 The Author(s) http://creativecommons.org/licenses/by/4.0/ This is an open access article under the CC BY license (http://creativecommons.org/licenses/by/4.0/).
spellingShingle Article
Lim, Dae Ro
Kim, Da Bin
Kim, Hee Kyung
Shin, Eung Jin
Surgery of hereditary hemorrhagic telangiectasia with severe refractory gastrointestinal bleeding: A case report of a rare condition
title Surgery of hereditary hemorrhagic telangiectasia with severe refractory gastrointestinal bleeding: A case report of a rare condition
title_full Surgery of hereditary hemorrhagic telangiectasia with severe refractory gastrointestinal bleeding: A case report of a rare condition
title_fullStr Surgery of hereditary hemorrhagic telangiectasia with severe refractory gastrointestinal bleeding: A case report of a rare condition
title_full_unstemmed Surgery of hereditary hemorrhagic telangiectasia with severe refractory gastrointestinal bleeding: A case report of a rare condition
title_short Surgery of hereditary hemorrhagic telangiectasia with severe refractory gastrointestinal bleeding: A case report of a rare condition
title_sort surgery of hereditary hemorrhagic telangiectasia with severe refractory gastrointestinal bleeding: a case report of a rare condition
topic Article
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7317164/
https://www.ncbi.nlm.nih.gov/pubmed/32698275
http://dx.doi.org/10.1016/j.ijscr.2020.06.057
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