No abstract available.
Telangiectasia, Hereditary Hemorrhagic

No abstract available.
Arteriovenous Fistula* ; Telangiectasia, Hereditary Hemorrhagic*

No abstract available.
Arteriovenous Malformations ; Embolization, Therapeutic ; Telangiectasia, Hereditary Hemorrhagic

No abstract available.
Activins* ; Humans ; Telangiectasia, Hereditary Hemorrhagic* ; Telangiectasis*

No abstract available.
Activins* ; Humans ; Telangiectasia, Hereditary Hemorrhagic* ; Telangiectasis*

Hereditary hemorrhagic telangiectasia (HHT), also known as Rendu-Osler-Weber disease, is a rare autosomal dominant disorder characterized by hemorrhage from nasal, mucocutaneous and gastrointestinal telangiectasia, in addition to vascular anomalies in other organs, particularly in the pulmonary, hepatic and cerebral circulations. The most common clinical manifestations are epistaxis and gastrointestinal bleeding. Endoscopic band ligation is effective for non-variceal, non-ulcer bleeding. It has the advantage of ease of use and is relatively inexpensive. We report a case of endoscopic band ligation therapy for gastric bleeding in patient with Rendu-Osler-Weber disease.
Epistaxis ; Hemorrhage* ; Humans ; Ligation* ; Telangiectasia, Hereditary Hemorrhagic* ; Telangiectasis

High-output cardiac failure is a rare complication of hereditary hemorrhagic telangiectasia (HHT) usually caused by shunting of blood through atriovenous malformations (AVMs) in the liver. We describe two cases of high output heart failure due to large hepatic AVMs. Clinical suspicion of HHT based on detailed history taking and physical examination is essential for early detection and proper management of heart failure associated with HHT.
Heart ; Heart Failure ; Liver ; Physical Examination ; Telangiectasia, Hereditary Hemorrhagic

Pulmonary arteriovenous fistula is an uncommon capillary abnormality. It may be accompanied by neurological complications such as cerebral abscess and be associated with or without hemorrhagic telangiectasia. We recently experienced a case of cerebral abscess with pulmonary arteriovenous fistula(PAVF), which was not associated with hereditary hemorrhagic telangiectasia. We report this case with review of the related literature.
Arteriovenous Fistula* ; Brain Abscess* ; Capillaries ; Telangiectasia, Hereditary Hemorrhagic ; Telangiectasis

Adult ; Arteriovenous Malformations ; Humans ; Male ; Telangiectasia, Hereditary Hemorrhagic

BACKGROUND AND OBJECTIVES: Epistaxis is the most common symptom in patients with hereditary hemorrhagic telangiectasia (HHT). The aim of this study is to report treatment of severe epistaxis related to HHT with the septodermoplasty and to assess patient outcomes. SUBJECTS AND METHOD: Six patients with HHT who underwent septodermoplasty from 2009 to 2013 were reviewed retrospectively. We analyzed the clinical characteristics, surgical management and treatment outcomes. RESULTS: Three patients had been diagnosed with definite HHT and three patients with possible HHT. Three patients had combined abnormal vascular structures in visceral organs. The mean preoperative and postoperative hemoglobin were 7.2 g/dL and 12.8 g/dL, respectively, and visual analogue scale score for frequency and intensity of nasal bleeding remarkably decreased after septodermoplasty in all patients. CONCLUSION: Septodermoplasty was a safe and efficacious procedure for reducing the frequency and severity of bleeding in most patients with intractable epistaxis and HHT.
Epistaxis ; Hemorrhage ; Humans ; Retrospective Studies ; Telangiectasia, Hereditary Hemorrhagic*