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https://www.readbyqxmd.com/read/29470256/systematic-screening-in-hereditary-hemorrhagic-telangiectasia-a-review
#1
Steven Kroon, Repke J Snijder, Marie E Faughnan, Hans-Jurgen Mager
PURPOSE OF REVIEW: Hereditary hemorrhagic telangiectasia (HHT) is an autosomal dominant inherited disease characterized by telangiectasia and arteriovenous malformations (AVMs). To date, five genetic types of HHT and one combined juvenile polyposis syndrome and HHT are known. Clinical and genetic screening of patients suspected with HHT is recommended to confirm the diagnosis and to prevent complications associated with HHT. The aim of this article is to give an overview of the evidence and to formulate a recommendation for clinicians concerning screening for HHT...
February 20, 2018: Current Opinion in Pulmonary Medicine
https://www.readbyqxmd.com/read/29469958/long-term-experience-with-intranasal-bevacizumab-therapy
#2
Johan Steineger, Terje Osnes, Ketil Heimdal, Sinan Dheyauldeen
OBJECTIVES/HYPOTHESIS: Long-term follow-up of intranasal bevacizumab therapy in hereditary hemorrhagic telangiectasia (HHT). STUDY DESIGN: Prospective, noncomparative study. METHODS: Patients treated for HHT-associated epistaxis by intranasal submucosal bevacizumab injections between June 2011 and August 2013 were included and followed prospectively. The effectiveness of the treatment was evaluated by the epistaxis severity score (ESS); the epistaxis intensity, frequency, and the need of blood transfusion (IFT) score; and hemoglobin levels...
February 22, 2018: Laryngoscope
https://www.readbyqxmd.com/read/29460088/vascular-deficiency-of-smad4-causes-arteriovenous-malformations-a-mouse-model-of-hereditary-hemorrhagic-telangiectasia
#3
Angela M Crist, Amanda R Lee, Nehal R Patel, Dawn E Westhoff, Stryder M Meadows
Hereditary hemorrhagic telangiectasia (HHT) is an autosomal dominant vascular disorder that leads to abnormal connections between arteries and veins termed arteriovenous malformations (AVM). Mutations in TGFβ pathway members ALK1, ENG and SMAD4 lead to HHT. However, a Smad4 mouse model of HHT does not currently exist. We aimed to create and characterize a Smad4 endothelial cell (EC)-specific, inducible knockout mouse (Smad4f/f ;Cdh5-CreERT2 ) that could be used to study AVM development in HHT. We found that postnatal ablation of Smad4 caused various vascular defects, including the formation of distinct AVMs in the neonate retina...
February 19, 2018: Angiogenesis
https://www.readbyqxmd.com/read/29451965/pazopanib-effective-for-bevacizumab-unresponsive-epistaxis-in-hereditary-hemorrhagic-telangiectasia
#4
Joseph G Parambil, Troy D Woodard, Omer N Koc
Hereditary hemorrhagic telangiectasia (HHT) most commonly manifests with nasal mucosal telangiectasias, and vascular endothelial growth factor (VEGF) plays a significant role in this angiodysplasia. We describe a patient with HHT with epistaxis recalcitrant to several endonasal procedures and six cycles of intravenous bevacizumab, for which he was dependent on iron infusions and packed red blood cells transfusions. He then started pazopanib at 100 mg with dramatic improvements in epistaxis and normalization of hemoglobin and iron levels, without replenishment needs for 12 months...
February 16, 2018: Laryngoscope
https://www.readbyqxmd.com/read/29450644/pi3k-akt-inhibitor-ly294002-potentiates-homoharringtonine-antimyeloma-activity-in-myeloma-cells-adhered-to-stromal-cells-and-in-scid-mouse-xenograft
#5
Ping Chen, Xiaofang Wen, Bin Wang, Diyu Hou, Hong Zou, Qin Yuan, Hui Yang, Jieqiong Xie, Huifang Huang
Homoharringtonine (HHT) is a known anti-leukemia drug that inhibits multiple myeloma (MM) cells both in vitro and in vivo. Our prior study demonstrated that the potency of HHT in MM cells was compromised significantly when myeloma cells were co-cultured with BM stromal cells. This study aimed to investigate whether PI3K/Akt inhibitor LY294002 could potentiate the antimyeloma activity of HHT against MM cells adhered to BM stromal cells and in vivo xenograft models. A co-culture system composed of MM cells and human stromal cells was employed to mimic MM cells in bone marrow niche...
February 15, 2018: Annals of Hematology
https://www.readbyqxmd.com/read/29438260/endothelial-cell-biology-of-endoglin-in-hereditary-hemorrhagic-telangiectasia
#6
Wade W Sugden, Arndt F Siekmann
PURPOSE OF REVIEW: Mutations in the Endoglin (Eng) gene, an auxiliary receptor in the transforming growth factor beta (TGFβ)-superfamily signaling pathway, are responsible for the human vascular disorder hereditary hemorrhagic telangiectasia (HHT) type 1, characterized in part by blood vessel enlargement. A growing body of work has uncovered an autonomous role for Eng in endothelial cells. We will highlight the influence of Eng on distinct cellular behaviors, such as migration and shape control, which are ultimately important for the assignment of proper blood vessel diameters...
February 12, 2018: Current Opinion in Hematology
https://www.readbyqxmd.com/read/29427063/functional-brain-abnormalities-in-major-depressive-disorder-using-the-hilbert-huang-transform
#7
Haibin Yu, Feng Li, Tong Wu, Rui Li, Li Yao, Chuanyue Wang, Xia Wu
Major depressive disorder is a common disease worldwide, which is characterized by significant and persistent depression. Non-invasive accessory diagnosis of depression can be performed by resting-state functional magnetic resonance imaging (rs-fMRI). However, the fMRI signal may not satisfy linearity and stationarity. The Hilbert-Huang transform (HHT) is an adaptive time-frequency localization analysis method suitable for nonlinear and non-stationary signals. The objective of this study was to apply the HHT to rs-fMRI to find the abnormal brain areas of patients with depression...
February 9, 2018: Brain Imaging and Behavior
https://www.readbyqxmd.com/read/29410309/gastroprotective-effects-of-hwanglyeonhaedok-tang-against-helicobacter-pylori-induced-gastric-cell-injury
#8
Hee-Seon Park, Charith Ub Wijerathne, Hye-Yun Jeong, Chang-Seob Seo, Hyekyung Ha, Hyo-Jung Kwun
ETHNOPHARMACOLOGICAL RELEVANCE: Helicobacter pylori, which is found in the stomachs of approximately half of the world's population, has been associated with the development of chronic gastritis and gastric cancer. Hwanglyeonhaedok-tang (HHT) is a popular traditional medicine for the therapies of gastric ulcers and gastritis. AIM OF THE STUDY: The emerging resistance of H. pylori to antibiotics arouses requirement on alternative nonantibiotic-based therapies. In the present study, we investigated the anti-inflammatory activity and anti-microbial activity of HHT against H...
February 1, 2018: Journal of Ethnopharmacology
https://www.readbyqxmd.com/read/29398197/clinical-presentation-and-treatment-paradigms-in-patients-with-hereditary-hemorrhagic-telangiectasia-and-spinal-vascular-malformations
#9
Ilyas Eli, Nicholas T Gamboa, Evan J Joyce, Min S Park, Philipp Taussky, Richard H Schmidt, William T Couldwell, Jamie McDonald, Kevin J Whitehead, M Yashar S Kalani
Hereditary hemorrhagic telangiectasia (HHT) is an autosomal dominant disorder that causes angiodysplasia and results in mucocutaneous telangiectasias and arteriovenous malformations of organs. Although central nervous system vascular malformations can occur anywhere along the neuraxis, spinal vascular malformations are rare. We present our experience with the presentation and management of spinal vascular malformations in patients with HHT. Of the more than 800 patients with the diagnosis of HHT screened at our institution from 1995 through 2017, four patients with spinal vascular malformations (age range 1 month-77 years; 2 male, 2 female) were identified, three of whom came to clinical attention after significant neurological deterioration from previously unknown malformations...
February 2, 2018: Journal of Clinical Neuroscience: Official Journal of the Neurosurgical Society of Australasia
https://www.readbyqxmd.com/read/29397826/-mechanism-of-mtor-pathway-in-k562-cell-apoptosis-induced-by-homoharringtonine
#10
Yi-Han Ding, Jing-Jing Wu, Qian Wang, Zhi-Kui Deng, Yu-Feng Li
OBJECTIVE: To investigate the effect of homoharringtonine (HHT) on proliferation and apoptosis of CML cell line K562 cells and to explore its possible mechanism through mTOR pathway. METHODS: K562 cells were cultured with different concentrations of HHT or in its combination with mTOR inhibitor rapamycin (RAPA) for 24 hours. The cell viability was analyzed by CCK-8 assay, the cell apoptosis was detected by flow cytometry, the expressions of BCL-6, Caspase-3 and mTOR signal pathway related proteins was assayed by Western blot, the expression of BCL-6 mRNA was determined by RT-PCR...
February 2018: Zhongguo Shi Yan Xue Ye Xue za Zhi
https://www.readbyqxmd.com/read/29395350/intravenous-bevacizumab-for-refractory-hereditary-hemorrhagic-telangiectasia-related-epistaxis-and-gastrointestinal-bleeding
#11
Vivek N Iyer, Dinesh R Apala, Bibek S Pannu, Aditya Kotecha, Waleed Brinjikji, Michael D Leise, Patrick S Kamath, Sanjay Misra, Kebede H Begna, Rodrigo Cartin-Ceba, Hilary M DuBrock, Michael J Krowka, Erin K O'Brien, Rajiv K Pruthi, Darrell R Schroeder, Karen L Swanson
OBJECTIVE: To present a multiyear clinical experience with intravenous bevacizumab for the management of severe gastrointestinal bleeding and/or epistaxis in patients with hereditary hemorrhagic telangiectasia (HHT). PATIENTS AND METHODS: All patients treated with intravenous bevacizumab for severe hereditary hemorrhagic telangiectasia-related bleeding from June 1, 2013, through January 31, 2017, were included in this report. Severity of epistaxis (determined using the Epistaxis Severity Score questionnaire); hemoglobin, iron, and ferritin levels; and quality of life data were collected serially in all patients...
January 9, 2018: Mayo Clinic Proceedings
https://www.readbyqxmd.com/read/29393992/medical-treatment-of-epistaxis-in-hereditary-hemorrhagic-telangiectasia-an-evidence-based-review
#12
Ashleigh A Halderman, Matthew W Ryan, Christopher Clark, Raj Sindwani, Douglas D Reh, David M Poetker, Rosangela Invernizzi, Bradley F Marple
BACKGROUND: Hereditary hemorrhagic telangiectasia (HHT) is an autosomal dominant hereditary disorder resulting in vascular dysplasia and formation of arteriovenous malformations. Recurrent epistaxis is a hallmark of the disease. An array of medical therapies are used in this patient population, but robust evidence-based recommendations regarding the medical treatment of epistaxis are lacking. This systematic review was performed to look at the current literature and make meaningful evidence-based recommendations...
February 2, 2018: International Forum of Allergy & Rhinology
https://www.readbyqxmd.com/read/29390976/probiotics-and-infective-endocarditis-in-patients-with-hereditary-hemorrhagic-telangiectasia-a-clinical-case-and-a-review-of-the-literature
#13
Evangelo Boumis, Alessandro Capone, Vincenzo Galati, Carolina Venditti, Nicola Petrosillo
BACKGROUND: In the last decades, probiotics have been widely used as food supplements because of their putative beneficial health effects. They are generally considered safe but rare reports of serious infections caused by bacteria included in the definition of probiotics raise concerns on their potential pathogenic role in patients with particular predisposing factors. Patients with hereditary hemorrhagic telangiectasia (HHT) are exposed to infections because of telangiectasias and arteriovenous malformations (AVMs)...
February 1, 2018: BMC Infectious Diseases
https://www.readbyqxmd.com/read/29356703/home-hearing-test-within-subjects-threshold-variability
#14
Robert H Margolis, Gene Bratt, M Patrick Feeney, Mead C Killion, George L Saly
BACKGROUND: The Home Hearing Test (HHT) is an automated pure-tone threshold test that obtains an air conduction audiogram at five test frequencies. It was developed to provide increased access to hearing testing and support home telehealth programs. PURPOSE: Test and retest thresholds for 1000-Hz stimuli were analyzed to determine intrasubject variability from two independent data sets. RESEARCH DESIGN: Prospective, repeated measures. STUDY SAMPLE: In the Veterans Affairs (VA) study, results from 26 subjects 44 to 88 years of age (mean = 65) recruited from the Nashville VA audiology clinic were analyzed...
January 18, 2018: Ear and Hearing
https://www.readbyqxmd.com/read/29351229/joint-bearing-and-range-estimation-of-multiple-objects-from-time-frequency-analysis
#15
Jeng-Cheng Liu, Yuang-Tung Cheng, Hsien-Sen Hung
Direction-of-arrival (DOA) and range estimation is an important issue of sonar signal processing. In this paper, a novel approach using Hilbert-Huang transform (HHT) is proposed for joint bearing and range estimation of multiple targets based on a uniform linear array (ULA) of hydrophones. The structure of this ULA based on micro-electro-mechanical systems (MEMS) technology, and thus has attractive features of small size, high sensitivity and low cost, and is suitable for Autonomous Underwater Vehicle (AUV) operations...
January 19, 2018: Sensors
https://www.readbyqxmd.com/read/29349377/emergency-thoracic-endovascular-aortic-repair-with-celiac-artery-coverage-in-hereditary-hemorrhagic-telangiectasia
#16
Yuji Kawano, Yuji Kanaoka, Nobuhiko Hiraiwa, Daisuke Nakatsuka, Minoru Tabata
Celiac artery (CA) coverage during thoracic endovascular aortic repair has been demonstrated to be a feasible and effective strategy for selected cases. However, there is a potential risk of ischemic complications due to CA coverage in patients with certain types of hereditary hemorrhagic telangiectasia (HHT). Herein, we report a case of thoracoabdominal aortic rupture in a patient with HHT that was successfully treated with emergency thoracic endovascular aortic repair covering the CA preceded by hepatic artery bypass...
June 2017: Journal of Vascular Surgery Cases and Innovative Techniques
https://www.readbyqxmd.com/read/29340222/direct-molecular-fishing-of-new-protein-partners-for-human-thromboxane-synthase
#17
A V Svirid, P V Ershov, E O Yablokov, L A Kaluzhskiy, Yu V Mezentsev, A V Florinskaya, T A Sushko, N V Strushkevich, A A Gilep, S A Usanov, A E Medvedev, A S Ivanov
Thromboxane synthase (TBXAS1) catalyzes the isomerization reaction of prostaglandin H2 producing thromboxane A2, the autocrine and paracrine factor in many cell types. A high activity and metastability by these arachidonic acid derivatives suggests the existence of supramolecular structures that are involved in the regulation of the biosynthesis and directed translocation of thromboxane to the receptor. The objective of this study was to identify TBXAS1 protein partners from human liver tissue lysate using a complex approach based on the direct molecular fishing technique, LC-MS/MS protein identification, and protein-protein interaction validation by surface plasmon resonance (SPR)...
October 2017: Acta Naturae
https://www.readbyqxmd.com/read/29339534/inactivating-mutations-in-drosha-mediate-vascular-abnormalities-similar-to-hereditary-hemorrhagic-telangiectasia
#18
Xuan Jiang, Whitney L Wooderchak-Donahue, Jamie McDonald, Prajakta Ghatpande, Mai Baalbaki, Melissa Sandoval, Daniel Hart, Hilary Clay, Shaun Coughlin, Giorgio Lagna, Pinar Bayrak-Toydemir, Akiko Hata
The transforming growth factor-β (TGF-β) and bone morphogenetic protein (BMP) family of cytokines critically regulates vascular morphogenesis and homeostasis. Impairment of TGF-β or BMP signaling leads to heritable vascular disorders, including hereditary hemorrhagic telangiectasia (HHT). Drosha, a key enzyme for microRNA (miRNA) biogenesis, also regulates the TGF-β and BMP pathway through interaction with Smads and their joint control of gene expression through miRNAs. We report that mice lacking Drosha in the vascular endothelium developed a vascular phenotype resembling HHT that included dilated and disorganized vasculature, arteriovenous fistulae, and hemorrhages...
January 16, 2018: Science Signaling
https://www.readbyqxmd.com/read/29334312/targeting-of-phospho-eif4e-by-homoharringtonine-eradicates-a-distinct-subset-of-human-acute-myeloid-leukemia
#19
Hong Zhou, Rong Zhen Xu, Ying Gu, Peng Fei Shi, Shenxian Qian
More than half of the patients with acute myeloid leukemia (AML) fail to achieve long-term disease-free survival with current therapies and novel therapeutic strategies are urgently needed. The effects of homoharringtonine (HHT) on the growth of AML cell lines and primary leukemia cells were examined using MTT, colony formation assay. The effects of HHT on both eukaryotic translation initiation factor 4E (eIF4E) and phospho-eIF4E(p-eIF4E) were examined through western blot and immunofluorescence staining. HHT selectively reduced levels of p-eIF4E and its downstream oncoprotein Mcl-1, and potently inhibited in vitro and in vivo the growth of a distinct subset of AML cells and primary leukemia cells expressing high level of p-eIF4E through apoptosis...
January 15, 2018: Leukemia & Lymphoma
https://www.readbyqxmd.com/read/29323699/leukotriene-b4-receptor-2-regulates-the-proliferation-migration-and-barrier-integrity-of-bronchial-epithelial-cells
#20
Min Liu, Juan Shen, Huimin Yuan, Fengling Chen, Huaidong Song, Hui Qin, Yanqin Li, Jiabo Xu, Qing Ye, Shenxian Li, Kazuko Saeki, Takehiko Yokomizo
The airway epithelium plays a crucial role in the pathogenesis of asthma. The functions of leukotriene B4 receptor 2 (BLT2) on the airway epithelial cells remains unknown. In our study, BLT2 expression in 16HBE bronchial epithelial cells were manipulated by transfection with BLT2 overexpression plasmid or BLT2 small interference RNA. 16HBE cells were then exposed to BLT2 antagonist (LY255283) or BLT2 agonist (12(S)-hydroxyheptadeca-5Z,8E,10E-trienoic acid [12-HHT] or CAY10583). The results showed that BLT2 overexpression, 12-HHT stimulation or CAY10583 treatment resulted in the enhanced proliferation and migration of 16HBE cells...
January 11, 2018: Journal of Cellular Physiology
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