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hereditary hemorrhagic telangiectasia

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https://www.readbyqxmd.com/read/28210642/high-output-heart-failure-contributing-to-recurrent-epistaxis-kiesselbach-area-syndrome-in-a-patient-with-hereditary-hemorrhagic-telangiectasia
#1
Venugopal Brijmohan Bhattad, Jennifer N Bowman, Hemang B Panchal, Timir K Paul
Hereditary hemorrhagic telangiectasia (HHT), also known as Osler-Weber-Rendu syndrome, is a rare genetic blood disorder that leads to abnormal bleeding due to absent capillaries and multiple abnormal blood vessels known as arteriovenous malformations. A feature of HHT is high-output heart failure due to multiple arteriovenous malformations. High-output heart failure can lead to recurrent epistaxis Kiesselbach area syndrome (REKAS), further exacerbating heart failure through increased blood loss and resultant anemia...
January 2017: Journal of Investigative Medicine High Impact Case Reports
https://www.readbyqxmd.com/read/28210085/macro-and-microcirculation-patterns-of-intrahepatic-blood-flow-changes-in-patients-with-hereditary-hemorrhagic-telangiectasia
#2
Roland C Schelker, Ana P Barreiros, Christina Hart, Wolfgang Herr, Ernst-Michael Jung
AIM: To evaluated vascular dynamic processes in the liver of hereditary hemorrhagic telangiectasia (HHT) patients by ultrasound (US) considering quantitative analytic methods. METHODS: The imaging features on US and contrast-enhanced ultrasound (CEUS) in 18 patients diagnosed with HHT were retrospectively analyzed. Regarding CEUS, real-time contrast harmonic imaging and sulfur hexafluoride-filled microbubbles were used. RESULTS: HVaMs were identified in all 18 patients...
January 21, 2017: World Journal of Gastroenterology: WJG
https://www.readbyqxmd.com/read/28188364/a-survey-of-pulmonary-arteriovenous-malformation-screening-management-and-follow-up-in-hereditary-hemorrhagic-telangiectasia-centers-of-excellence
#3
Jeffrey Forris Beecham Chick, Shilpa N Reddy, Reed E Pyeritz, Scott O Trerotola
PURPOSE: To determine current practice regarding screening, management, and follow-up of pulmonary arteriovenous malformations (PAVMs), particularly those with feeding arteries <3 mm in diameter, in those patients with hereditary hemorrhagic telangiectasia (HHT) at HHT Centers of Excellence. MATERIALS AND METHODS: A 32-question survey focusing on PAVM screening, management, and follow-up was sent to 147 participants at HHT Centers worldwide. RESULTS: Thirty-three responses were received (22%); 60% from interventional radiologists and 20% from pulmonologists; 67% from the USA, 21% European, and 6% Asian...
February 10, 2017: Cardiovascular and Interventional Radiology
https://www.readbyqxmd.com/read/28175903/letter-lower-risk-of-intracranial-arteriovenous-malformation-hemorrhage-in-patients-with-hereditary-hemorrhagic-telangiectasia
#4
Elizabeth Ogando-Rivas, Jesús Quetzalcóatl Beltrán Mendoza
No abstract text is available yet for this article.
February 1, 2017: Neurosurgery
https://www.readbyqxmd.com/read/28165669/non-invasive-ct-screening-for-pulmonary-arteriovenous-malformations-in-children-with-confirmed-hereditary-hemorrhagic-telangiectasia-results-from-two-pediatric-centers
#5
Nurcan Soysal, Mélanie Eyries, Suzanne Verlhac, Virginie Escabasse, Natascha Remus, Aline Tamalet, Jean-Yves Rioux, Stéphanie Franchi-Abella, Manuela Vasile, Sarah Robert, Céline Delestrain, Isabelle Hau, Hubert Ducou-Le Pointe, Florent Soubrier, Marie-France Carette, Ralph Epaud
BACKGROUND: Hereditary hemorrhagic telangiectasia (HHT) is an autosomal dominant genetic disorder that is caused by mutations in mainly two genes, that is ENG, encoding endoglin (HHT1), or ACVRL1, encoding activin receptor-like kinase 1 (ALK-1/HHT2). HHT is characterized by recurrent epistaxis, mucocutaneous telangiectasia, and vascular visceral dysplasia responsible for visceral arteriovenous malformations (AVM). AIM: to report the experience of two university hospitals (Trousseau, Paris, and CHIC, Creteil) with screening children for HHT and pulmonary AVM (PAVM) using high resolution computed tomography (HRCT)...
February 6, 2017: Pediatric Pulmonology
https://www.readbyqxmd.com/read/28159602/it-looks-familial-hereditary-hemorrhagic-telangiectasia
#6
Binod K Khaitan, Vishal Gupta, Mamta Rai, Ajay Garg
No abstract text is available yet for this article.
January 31, 2017: American Journal of Medicine
https://www.readbyqxmd.com/read/28132533/hereditary-hemorrhagic-telangiectasia-transcatheter-intervention-and-beyond
#7
Yap-Hang Chan, Angel Chan, Ki-Wan Kelvin Chan
No abstract text is available yet for this article.
January 1, 2017: Asian Cardiovascular & Thoracic Annals
https://www.readbyqxmd.com/read/28131865/juvenile-polyposis-and-hereditary-hemorrhagic-telangiectasia-overlap-syndrome
#8
Guilherme Piovezani Ramos, Korosh Sharain, Karthik Ravi
No abstract text is available yet for this article.
January 25, 2017: Gastrointestinal Endoscopy
https://www.readbyqxmd.com/read/28116537/sinonasal-quality-of-life-outcomes-following-laser-treatment-of-epistaxis-related-to-hereditary-hemorrhagic-telangiectasia
#9
Edward C Kuan, Kevin A Peng, Christopher F Thompson, Jeffrey D Suh, Marilene B Wang
Hereditary hemorrhagic telangiectasia (HHT) is an inherited vascular disorder which manifests as recurrent, episodic, and potentially debilitating epistaxis. In this study, we aim to (1) characterize baseline sinonasal symptoms for HHT patients and to (2) analyze changes in sinonasal symptoms before and after laser surgical treatment for HHT. We performed a retrospective chart review of sinonasal outcome test-22 (SNOT-22) scores before and after one or more laser surgical treatments for HHT-related epistaxis between January 1, 2010 and December 1, 2015 at a tertiary academic medical center with an HHT Foundation-approved Center of Excellence...
January 24, 2017: Lasers in Medical Science
https://www.readbyqxmd.com/read/28115008/hereditary-hemorrhagic-telangiectasia-liver-disease-and-elevated-serum-testosterone-osler-weber-rendu-syndrome-a-case-report
#10
R Dissanayake, K P K Y M D S Wickramarathne, S N Seneviratne, S N Perera, M U J Fernando, V P Wickramasinghe
BACKGROUND: A Sri Lankan girl with hereditary hemorrhagic telangiectasia (Osler-Weber-Rendu syndrome) is described. CASE PRESENTATION: She presented with recurrent spontaneous epistaxis, pulmonary arterio venous malformation and oral telangiectasia. A diagnosis of Hereditary hemorrhagic telangiectasia (Osler-Weber-Rendu syndrome) was made based on the presence of three Curacao criteria (out of four). Evaluations of her jaundice revealed chronic parenchymal liver disease with multiple nodules in the liver with early portal hypertension...
January 23, 2017: BMC Research Notes
https://www.readbyqxmd.com/read/28114930/quality-of-life-in-patients-with-hereditary-haemorrhagic-telangiectasia-hht
#11
Roberto Zarrabeitia, Concepción Fariñas-Álvarez, Miguel Santibáñez, Blanca Señaris, Ana Fontalba, Luisa María Botella, José Antonio Parra
BACKGROUND: There are very few studies about general quality of life parameters, standards for the description of health status and comparison with general population data on patients with Hereditary hemorrhagic telangiectasia (HHT), a rare disease in which epistaxis is a cardinal symptom. PURPOSE: To assess the quality of life in a population of Spanish patients with HHT and compare it with the general population. DESIGN AND METHODS: Between January 1(st) 2005 and December 31(st) 2013, 187 adult patients diagnosed with HHT who were admitted to the HHT Unit of the Hospital Sierrallana, completed on their first visit, the EuroQol 5D-3L (five dimensions and three levels) quality of life descriptive test and the visual analog scale (VAS)...
January 23, 2017: Health and Quality of Life Outcomes
https://www.readbyqxmd.com/read/28070641/influence-of-temporary-nasal-occlusion-tno-on-epistaxis-frequency-in-patients-with-hereditary-hemorrhagic-telangiectasia-hht
#12
Kornelia E C Wirsching, Frank Haubner, Thomas S Kühnel
The objective of the study was to evaluate the influence of temporary nasal occlusion (tNO) with hypoallergenic tape on the frequency and severity of epistaxis in patients with hereditary hemorrhagic telangiectasia (HHT). This prospective observational study included 20 HHT patients who were undergoing Nd:YAG laser therapy at regular intervals. Over a 3-month period, laser therapy was supplemented by tNO with hypoallergenic tape for 5 h/day on average. On a 0-10 numeric rating scale, the patients reported significantly greater satisfaction in epistaxis terms after tNO treatment, with mean scores of 5 before and 7 after 3-month tNO (p = 0...
January 9, 2017: European Archives of Oto-rhino-laryngology
https://www.readbyqxmd.com/read/28070235/bevacizumab-and-gastrointestinal-bleeding-in-hereditary-hemorrhagic-telangiectasia
#13
George Ou, Cherry Galorport, Robert Enns
We report a case of severe, refractory gastrointestinal (GI) bleeding in a patient with hereditary hemorrhagic telangiectasia (HHT) whose massive transfusion dependence was lifted shortly after treatment with bevacizumab, an anti-vascular endothelial growth factor. The patient's bleeding had been refractory to repeated endoscopic interventions, tranexamic acid, and tamoxifen. However, following treatment with bevacizumab at 5 mg/kg every other week, nearly 300 units of packed red blood cell transfusions were avoided in one year's time...
December 27, 2016: World Journal of Gastrointestinal Surgery
https://www.readbyqxmd.com/read/28059706/the-prevalence-of-malformations-of-cortical-development-in-a-pediatric-hereditary-hemorrhagic-telangiectasia-population
#14
G J Palagallo, S R McWilliams, L A Sekarski, A Sharma, M S Goyal, A J White
BACKGROUND AND PURPOSE: Brain AVM, cerebral abscess, and ischemic stroke are among the well known neurologic manifestations of hereditary hemorrhagic telangiectasia. However, recently reported data suggest an additional association with malformations of cortical development. The purpose of this study was to determine the prevalence of malformations of cortical development in a population of pediatric patients with hereditary hemorrhagic telangiectasia. MATERIALS AND METHODS: A retrospective review of brain MRIs from 116 pediatric patients was performed...
November 10, 2016: AJNR. American Journal of Neuroradiology
https://www.readbyqxmd.com/read/28032956/efficacy-and-safety-of-propranolol-for-epistaxis-in-hereditary-hemorrhagic-telangiectasia-hht-retrospective-then-prospective-study-in-a-total-of-21-patients
#15
A Contis, N Gensous, J F Viallard, C Goizet, C Léauté-Labrèze, P Duffau
Hereditary Hemorrhagic Telangiectasia (HHT) is a genetic disorder of angiogenesis associated with disabling epistaxis. Propranolol, a beta-blocker, exerts anti-angiogenic properties. In the retrospective study, nine of ten HHT patients receiving propranolol significantly improved their Epistaxis Severity Score. In the prospective study, after three months of propranolol treatment, the median duration of epistaxis per month significantly decreased as did the number of epistaxis episodes per month in 11 HHT patients...
December 29, 2016: Clinical Otolaryngology
https://www.readbyqxmd.com/read/28031662/hereditary-hemorrhagic-telangiectasia-and-myocardial-infarction
#16
Efrén Martínez-Quintana, Fayna Rodríguez-González, Silvia Gopar-Gopar
Hereditary hemorrhagic telangiectasia, also known as Osler-Weber-Rendu syndrome, is an autosomal dominant genetic disorder that leads to epistaxis, gastrointestinal bleeding, iron deficiency anemia, and arteriovenous malformations at the lungs, the liver, and the brain. However, due to its rarity and its unspecific findings on routine examinations, diagnosis is not easy unless suspected due to hypoxemia or paradoxical embolism. We present a case of a 46-year-old-woman with hereditary hemorrhagic telangiectasia and hypoxemia who presented a myocardial infarction secondary to paradoxical embolism through pulmonary arteriovenous malformations...
December 2016: International Journal of Angiology: Official Publication of the International College of Angiology, Inc
https://www.readbyqxmd.com/read/28008625/arterial-ligation-versus-embolization-in-epistaxis-management-counterintuitive-national-trends
#17
Michael J Sylvester, Sei Y Chung, Luis A Guinand, Aparna Govindan, Soly Baredes, Jean Anderson Eloy
OBJECTIVES/HYPOTHESIS: Arterial ligation and embolization are treatment modalities indicated in severe and refractory epistaxis. The purpose of this study was to examine temporal trends and compare outcomes in treatment of hospitalized epistaxis patients with ligation or embolization. METHODS: This retrospective cohort analysis utilized the 2008 to 2013 National Inpatient Sample to identify patients admitted with a primary diagnosis of epistaxis, and an associated procedure code for ligation or embolization...
December 23, 2016: Laryngoscope
https://www.readbyqxmd.com/read/28004998/de-novo-vascular-lesions-after-liver-transplant-in-a-case-with-hereditary-hemorrhagic-telangiectasia-and-fibropolycystic-liver-disease-long-term-follow-up-with-magnetic-resonance-imaging-and-magnetic-resonance-angiography
#18
Sila Ulus, Aydan Arslan, Ercan Karaarslan, Yilmaz Cakaloglu
We report a case of a 62-year-old woman who received a liver transplant 19 years previously for end-stage liver disease due to hereditary hemorrhagic telangiectasia and fibropolycystic liver disease. During long-term follow-up 8 years after the liver transplant, de novo vascular lesions were detected with magnetic resonance imaging and magnetic resonance angiography. Hepatic vascular lesions had slowly progressed, despite no symptoms. To our knowledge, there are few reports in the English literature of de novo vascular lesions after liver transplant in patients with hepatic telangiectasias...
December 22, 2016: Experimental and Clinical Transplantation
https://www.readbyqxmd.com/read/27997001/-a-rare-cardiomegaly
#19
Bruno De Piccoli, Marino Cibin, Marta Possamai, Giuseppe Favretto
We present the case of a patient who came to our attention for enlargement of the cardiac silhouette on chest X-ray. Echocardiography showed moderate diastolic overload of both ventricles with enhanced cardiac output without valvular disease or cardiac shunt that could account for this cardiomegaly. A subsequent abdominal echocardiographic exploration showed an angiomatous transformation of the liver due to diffuse lacunar enlargement of hepatic portal vein branches and arterial-venous shunts. Computed tomography and magnetic resonance imaging confirmed the echocardiographic findings...
November 2016: Giornale Italiano di Cardiologia
https://www.readbyqxmd.com/read/27895866/liver-findings-in-patients-with-hereditary-hemorrhagic-telangiectasia
#20
Wenyan Song, Dawei Zhao, Hongjun Li, Jinli Ding, Ning He, Yu Chen
BACKGROUND: Hereditary hemorrhagic telangiectasia (HHT), also known as Osler-Weber-Rendu syndrome, is a rare autosomal dominant genetic vascular dysplasia. OBJECTIVES: The aim is to characterize the liver involvement of HHT by multi-detector helical computed tomography. PATIENTS AND METHODS: Through the use of multi-detector helical computed tomography, scan data derived from 12 cases of HHT were retrospectively studied, and an abdominal scan was performed on all of the patients...
October 2016: Iranian Journal of Radiology: a Quarterly Journal Published By the Iranian Radiological Society
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