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hereditary hemorrhagic telangiectasia

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https://www.readbyqxmd.com/read/28430541/a-comprehensive-approach-to-hepatic-vascular-disease
#1
Khaled M Elsayes, Akram M Shaaban, Sarah M Rothan, Sanaz Javadi, Beatrice L Madrazo, Rosa P Castillo, Victor J Casillas, Christine O Menias
The liver has a complex vascular supply, which involves the inflow of oxygenated blood through the hepatic artery (systemic circulation) and deoxygenated blood through the portal vein (portal circulation), as well as the outflow of deoxygenated blood through the hepatic veins to the inferior vena cava. A spectrum of vascular variants can involve the liver. Some of these variants may result in areas of enhancement that can mimic more serious pathologic conditions. In this article, the authors discuss a spectrum of variants and pathologic conditions that may involve the liver vasculature...
April 21, 2017: Radiographics: a Review Publication of the Radiological Society of North America, Inc
https://www.readbyqxmd.com/read/28427830/liver-transplantation-for-hereditary-hemorrhagic-telangiectasia-a-systematic-review
#2
REVIEW
Emanuele Felli, Pietro Addeo, François Faitot, Gennaro Nappo, Constantin Oncioiu, Philippe Bachellier
AIM: To evaluate the indications, timing and results of liver transplantation in patients affected by hereditary hemorrhagic telangiectasia (HHT), by undertaking a systematic review of the current literature. METHODS: Electronic bibliographical databases were searched on MEDLINE and Pubmed according to the PRISMA criteria. A total of 58 articles were initially found, 11 have been excluded because of single center series later included in the European Liver transplant Registry (ELTR), already reported in this study...
April 17, 2017: HPB: the Official Journal of the International Hepato Pancreato Biliary Association
https://www.readbyqxmd.com/read/28420371/effect-of-pulmonary-arteriovenous-malformations-on-the-mechanical-properties-of-the-lungs
#3
Cécile Rotenberg, Marcel Bonay, Mostafa El Hajjam, Sandra Blivet, Alain Beauchet, Pascal Lacombe, Thierry Chinet
BACKGROUND: Pulmonary arteriovenous malformations (PAVMs) are present in approximately 15-50% individuals with hereditary hemorrhagic telangiectasia (HHT). They may be isolated but more often are multiple. The goal of this study was to evaluate the influence of PAVMs on lung mechanical properties. METHODS: We reviewed the files of all adult patients (age ≥ 18 years) referred to our Center for evaluation of HHT between 2005 and 2013. The diagnosis of HHT was based on the Curacao criteria and/or the presence of a pathogenic mutation...
April 19, 2017: BMC Pulmonary Medicine
https://www.readbyqxmd.com/read/28418273/thalidomide-for-epistaxis-in-patients-with-hereditary-hemorrhagic-telangiectasia-a-preliminary-study
#4
Jia Fang, Xiaomeng Chen, Bijun Zhu, Haibo Ye, Weitian Zhang, Jian Guan, Kaiming Su
To evaluate the effectiveness of thalidomide for epistaxis in hereditary hemorrhagic telangiectasia (HHT), 7 HHT patients with recurrent epistaxis were treated with thalidomide at an initial dose of 50 mg/d, gradually increasing to 100 mg/d if needed. The Epistaxis Severity Score (ESS) was used to evaluate the treatment effects. Patients reported that epistaxis improved 1 to 3 weeks after starting thalidomide. The mean ESS before treatment, at the end of treatment, and 3 months after stopping treatment was 5...
April 1, 2017: Otolaryngology—Head and Neck Surgery
https://www.readbyqxmd.com/read/28411778/lack-of-efficacy-of-topical-timolol-for-cutaneous-telangiectasias-in-patients-with-hereditary-hemorrhagic-telangiectasia-results-of-a-pilot-study
#5
Hana Jeon, Bernard Cohen
No abstract text is available yet for this article.
May 2017: Journal of the American Academy of Dermatology
https://www.readbyqxmd.com/read/28407366/screening-children-for-pulmonary-arteriovenous-malformations-evaluation-of-18-years-of-experience
#6
Anna E Hosman, Els M de Gussem, Walter A F Balemans, Andréanne Gauthier, Cees J J Westermann, Repke J Snijder, Marco C Post, Johannes J Mager
BACKGROUND: Hereditary Hemorrhagic Telangiectasia (HHT) is an autosomal dominant disease with multi-systemic vascular dysplasia. Early diagnosis through screening is important to prevent serious complications. How best to screen children of affected parents for pulmonary arteriovenous malformations (PAVMs) is often subject to debate. Transthoracic contrast echocardiogram (TTCE) is considered optimal in screening for PAVMs in adults. Guidelines for the screening of children are not specific, reflecting the lack of scientific evidence on the best method to use...
April 13, 2017: Pediatric Pulmonology
https://www.readbyqxmd.com/read/28394685/a-case-report-of-hereditary-hemorrhagic-telangiectasia-diagnosed-in-a-podiatric-medical-patient
#7
Kunal Amin, Douglas Mckay
Hereditary hemorrhagic telangiectasia (HHT), which is also known as Osler-Weber-Rendu syndrome, is a group of related disorders characterized by the development of arteriovenous malformations. These malformations occur in almost all organs but predominantly in the skin, intestines, liver, lungs, and brain. This is a case report of a patient with cutaneous manifestations of HHT in the lower extremities as diagnosed by his podiatric physician. To our knowledge, the literature does not present any case reports in which cutaneous manifestations of the lower extremities followed by a further work-up allowed a diagnosis of HHT...
March 2017: Journal of the American Podiatric Medical Association
https://www.readbyqxmd.com/read/28387092/-hereditary-hemorrhagic-telangiectasia-and-pulmonary-arteriovenous-malformations-in-children
#8
M Thimmesch, K El Abd, D Brisbois, J Khamis, M Lewin, P Philippet
Hereditary hemorrhagic telangiectasia is a constitutional vascular dysplasia characterized by chronic epistaxis, mucocutaneous and visceral telangiectasias and arteriovenous malformations. Apart from family screenings, the disease is rarely diagnosed during the pediatric age given the late advent of typical clinical symptoms. Nevertheless, arteriovenous malformations are sometimes already present at a young age with significant morbidity risk. Therefore, it is important to establish an early diagnosis. We describe two pediatric cases of hereditary hemorrhagic telangiectasia and pulmonary arteriovenous malformations with divergent clinical presentation...
December 2016: Revue Médicale de Liège
https://www.readbyqxmd.com/read/28375020/pulmonary-hypertension-prevalence-and-prognosis-in-a-cohort-of-patients-with-hereditary-hemorrhagic-telangiectasia-undergoing-embolization-of-pavms
#9
Mwelwa Chizinga, Anastasiia A Rudkovskaia, Katharine Henderson, Jeffrey Pollak, Guadalupe Garcia-Tsao, Lawrence H Young, Wassim H Fares
No abstract text is available yet for this article.
April 4, 2017: American Journal of Respiratory and Critical Care Medicine
https://www.readbyqxmd.com/read/28360478/hereditary-hemorrhagic-telangiectasia-with-hemothorax-in-pregnancy
#10
Sagar Raiya, Amita Athavale, Jairaj Nair, Hemant Deshmukh
No abstract text is available yet for this article.
March 2017: Lung India: Official Organ of Indian Chest Society
https://www.readbyqxmd.com/read/28348038/bone-morphogenetic-proteins-in-vascular-homeostasis-and-disease
#11
Marie-José Goumans, An Zwijsen, Peter Ten Dijke, Sabine Bailly
It is well established that control of vascular morphogenesis and homeostasis is regulated by vascular endothelial growth factor (VEGF), fibroblast growth factor (FGF), Delta-like 4 (Dll4), angiopoietin, and ephrin signaling. It has become clear that signaling by bone morphogenetic proteins (BMPs), which have a long history of studies in bone and early heart development, are also essential for regulating vascular function. Indeed, mutations that cause deregulated BMP signaling are linked to two human vascular diseases, hereditary hemorrhagic telangiectasia and pulmonary arterial hypertension...
March 27, 2017: Cold Spring Harbor Perspectives in Biology
https://www.readbyqxmd.com/read/28348036/tgf-%C3%AE-signaling-in-control-of-cardiovascular-function
#12
Marie-José Goumans, Peter Ten Dijke
Genetic studies in animals and humans indicate that gene mutations that functionally perturb transforming growth factor β (TGF-β) signaling are linked to specific hereditary vascular syndromes, including Osler-Rendu-Weber disease or hereditary hemorrhagic telangiectasia and Marfan syndrome. Disturbed TGF-β signaling can also cause nonhereditary disorders like atherosclerosis and cardiac fibrosis. Accordingly, cell culture studies using endothelial cells or smooth muscle cells (SMCs), cultured alone or together in two- or three-dimensional cell culture assays, on plastic or embedded in matrix, have shown that TGF-β has a pivotal effect on endothelial and SMC proliferation, differentiation, migration, tube formation, and sprouting...
March 27, 2017: Cold Spring Harbor Perspectives in Biology
https://www.readbyqxmd.com/read/28339142/selective-effects-of-oral-anti-angiogenic-tyrosine-kinase-inhibitors-on-an-animal-model-of-hereditary-hemorrhagic-telangiectasia
#13
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
#14
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
#15
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
#16
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
#17
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
#18
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
#19
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
#20
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
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