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hereditary hemorrhagic telangiectasia

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https://www.readbyqxmd.com/read/28339142/selective-effects-of-oral-anti-angiogenic-tyrosine-kinase-inhibitors-on-an-animal-model-of-hereditary-hemorrhagic-telangiectasia
#1
Yong Hwan Kim, Mi-Jung Kim, Se-Woon Choe, Dennis Sprecher, Young Jae Lee, S Paul Oh
BACKGROUND: Epistaxis and gastrointestinal (GI) tract hemorrhages are common symptoms of aged hereditary hemorrhagic telangiectasia (HHT) patients that result in anemia. Clinical as well as animal studies have suggested that vascular endothelial growth factor (VEGF) neutralizing antibodies lessen hemorrhage associated with adult onset arteriovenous malformations (AVMs). OBJECTIVES: The goal of this study is to evaluate potential therapeutic effects of oral delivery of four anti-angiogenic tyrosine-kinase inhibitors (TKIs) in the development of adult onset AVMs in a murine model of HHT...
March 24, 2017: Journal of Thrombosis and Haemostasis: JTH
https://www.readbyqxmd.com/read/28314138/depression-and-post-traumatic-stress-disorder-in-individuals-with-hereditary-hemorrhagic-telangiectasia-a-cross-sectional-survey
#2
Shruti Chaturvedi, Marianne Clancy, Nicole Schaefer, Olalekan Oluwole, Keith R McCrae
INTRODUCTION: Hereditary hemorrhagic telangiectasia (HHT) is characterized by frequent severe bleeding, particularly epistaxis, and life-threatening complications including stroke, brain abscess and heart failure. The psychological impact of HHT is not known. We conducted this cross sectional study to determine the prevalence of depression and post-traumatic stress disorder (PTSD) related to HHT. METHODS: A survey tool comprising demographic and clinical information and two validated self-administered questionnaires, the PTSD checklist for DSM-5 (PCL-5) and Beck Depression Inventory-II (BDI-II), was distributed to individuals with HHT...
March 9, 2017: Thrombosis Research
https://www.readbyqxmd.com/read/28298578/hereditary-hemorrhagic-telangiectasia-management
#3
Jeffrey Peterson
Hereditary hemorrhagic telangiectasia (HHT) is an inherited disorder that can lead to frequent and severe sequelae. Although the condition has no cure, many of its physical symptoms can be managed to improve the quality of life and lower the risk of life-threatening complications. This article discusses HHT presentation, genetic screening, diagnosis, and management. The management of HHT can involve interventional radiology techniques, particularly for patients with pulmonary arteriovenous malformations.
January 2017: Radiologic Technology
https://www.readbyqxmd.com/read/28298363/mutual-interaction-between-endothelial-cells-and-mural-cells-enhances-bmp9-signaling-in-endothelial-cells
#4
Yuki Tachida, Nanae Izumi, Toyo Sakurai, Hideki Kobayashi
Hereditary hemorrhagic telangiectasia is characterized by the formation of abnormal vascular networks and caused by the mutation of genes involved in BMP9 signaling. It is also known that the interaction between endothelial cells (ECs) and mural cells (MCs) is critical to maintain vessel integrity. However, it has not yet fully been uncovered whether the EC-MC interaction affects BMP9 signaling or not. To elucidate this point, we analyzed BMP9 signaling in a co-culture of several types of human primary culture ECs and MCs...
March 15, 2017: Biology Open
https://www.readbyqxmd.com/read/28297555/epistaxis-grading-in-osler-s-disease-comparison-of-comprehensive-scores-with-detailed-bleeding-diaries
#5
Thomas Parzefall, Axel Wolf, Klemens Frei, Alexandra Kaider, Dominik Riss
BACKGROUND: Use of reliable grading scores to measure epistaxis severity in hereditary hemorrhagic telangiectasia (HHT) is essential in clinical routine and for scientific purposes. For practical reasons, visual analog scale (VAS) scoring and the Epistaxis Severity Score (ESS) are widely used. VAS scores are purely subjective, and a potential shortcoming of the ESS is that it is based on self-reported anamnestic bleeding data. The aim of this study was to validate the level of correlation between VAS scores, the ESS, and actual bleeding events, based on detailed epistaxis diaries of patients...
March 2017: International Forum of Allergy & Rhinology
https://www.readbyqxmd.com/read/28267932/hemoglobin-is-a-vital-determinant-of-arterial-oxygen-content-in-hypoxemic-patients-with-pulmonary-arteriovenous-malformations
#6
Abeer Rizvi, Patricia Macedo, Lydia Babawale, Hannah C Tighe, J Michael B Hughes, James E Jackson, Claire L Shovlin
RATIONALE: Arterial partial pressure of oxygen (PaO2), and oxygen saturation (SaO2) are commonly measured in respiratory practice, but arterial oxygen content (CaO2) refers to the volume of oxygen delivered to the tissues per unit blood volume. CaO2 is calculated from SaO2 and the hemoglobin concentration in blood, recognizing that each gram of hemoglobin can transport approximately 1.34mls of oxygen when fully saturated. OBJECTIVES: To prospectively evaluate serial changes in CaO2 in man, incorporating and excluding dynamic changes to oxygenation and hemoglobin parameters that may occur during life...
March 7, 2017: Annals of the American Thoracic Society
https://www.readbyqxmd.com/read/28210642/high-output-heart-failure-contributing-to-recurrent-epistaxis-kiesselbach-area-syndrome-in-a-patient-with-hereditary-hemorrhagic-telangiectasia
#7
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
#8
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
#9
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
#10
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
#11
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
#12
Binod K Khaitan, Vishal Gupta, Mamta Rai, Ajay Garg
No abstract text is available yet for this article.
February 1, 2017: American Journal of Medicine
https://www.readbyqxmd.com/read/28132533/hereditary-hemorrhagic-telangiectasia-transcatheter-intervention-and-beyond
#13
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
#14
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
#15
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...
April 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
#16
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
#17
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
#18
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
#19
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
#20
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
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