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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/28196298/human-to-human-transmission-of-brucella-a-systematic-review
#3
REVIEW
Felipe F Tuon, Regina B Gondolfo, Natacha Cerchiari
OBJECTIVE: The most common form of transmitting human brucellosis is through contaminated food or direct contact with infected animals. Human-to-human transmission (HHT) has been described as isolated case reports. The aim of this systematic review was to describe all cases of HHT of human brucellosis reported in the medical literature. METHODS: A literature search was conducted using PubMed, Scopus and Scielo databases using specific search terms published until March 2016...
February 14, 2017: Tropical Medicine & International Health: TM & IH
https://www.readbyqxmd.com/read/28188364/a-survey-of-pulmonary-arteriovenous-malformation-screening-management-and-follow-up-in-hereditary-hemorrhagic-telangiectasia-centers-of-excellence
#4
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/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/28116537/sinonasal-quality-of-life-outcomes-following-laser-treatment-of-epistaxis-related-to-hereditary-hemorrhagic-telangiectasia
#6
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/28114930/quality-of-life-in-patients-with-hereditary-haemorrhagic-telangiectasia-hht
#7
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/28110727/a-new-approach-to-early-diagnosis-of-congestive-heart-failure-disease-by-using-hilbert-huang-transform
#8
Gokhan Altan, Yakup Kutlu, Novruz Allahverdi
Congestive heart failure (CHF) is a degree of cardiac disease occurring as a result of the heart's inability to pump enough blood for the human body. In recent studies, coronary artery disease (CAD) is accepted as the most important cause of CHF. This study focuses on the diagnosis of both the CHF and the CAD. The Hilbert-Huang transform (HHT), which is effective on non-linear and non-stationary signals, is used to extract the features from R-R intervals obtained from the raw electrocardiogram data. The statistical features are extracted from instinct mode functions that are obtained applying the HHT to R-R intervals...
December 2016: Computer Methods and Programs in Biomedicine
https://www.readbyqxmd.com/read/28070641/influence-of-temporary-nasal-occlusion-tno-on-epistaxis-frequency-in-patients-with-hereditary-hemorrhagic-telangiectasia-hht
#9
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
#10
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/28064338/-unknown-title
#11
V J Lund, Y Darby, J Rimmer, M Amin, S Husain
INTRODUCTION: Hereditary haemorrhagic telangiectasia (HHT) is an autosomal dominant vascular disease characterized by recurrent epistaxis, mucocutaneous telangiectasia and visceral arteriovenous malformations. The nosebleeds can be life-threatening and in these circumstances, radical treatment is required. METHODS: Since 1994, closure of the nose has been undertaken to prevent severe nasal bleeding in patients meeting specific selection criteria. Outcome data collected on this cohort pre- and post-operatively is available for analysis...
January 8, 2017: Rhinology
https://www.readbyqxmd.com/read/28061447/the-combination-effect-of-homoharringtonine-and-ibrutinib-on-flt3-itd-mutant-acute-myeloid-leukemia
#12
Xia Li, Xiufeng Yin, Huafeng Wang, Jiansong Huang, Mengxia Yu, Zhixin Ma, Chenying Li, Yile Zhou, Xiao Yan, ShuJuan Huang, Jie Jin
Acute myeloid leukemia (AML) is a highly heterogeneous disease and internal tandem duplication mutation in FMS-like tyrosine-kinase-3 (FLT3-ITD) has a negative impact on outcome. Finding effective treatment regimens is desperately needed. In this study, we explored the inhibitory effect and mechanism of homoharringtonine (HHT) in combination with ibrutinib on FLT3-ITD mutant AML cells. Consequently, we observed a synergistic inhibitory effect when ibrutinib was combined with HHT to inhibit cell proliferation, induce apoptosis and arrest cell cycle at G0/G1 phase in MV4-11 and MOLM-13 leukemia cells...
January 3, 2017: Oncotarget
https://www.readbyqxmd.com/read/28056549/pegylated-long-circulating-liposomes-deliver-homoharringtonine-to-suppress-multiple-myeloma-cancer-stem-cells
#13
Miao Li, Fangfang Shi, Xiong Fei, Songyan Wu, Di Wu, Meng Pan, Shouhua Luo, Ning Gu, Jun Dou
The goal of this investigation was to evaluate the inhibiting effect of high proportion polyethyleneglycol of long-circulating homoharringtonine liposomes on RPMI8226 multiple myeloma cancer stem cells. The CD138(-)CD34(-) multiple myeloma cancer stem cells isolated from RPMI8226 cell line using magnetic activated cell sorting system were, respectively, incubated with the optimized formulation of polyethyleneglycol of long-circulating homoharringtonine liposomes and the homoharringtonine in vitro, and the multiple myeloma cancer stem cell proliferation, colony formation, and cell cycle were analyzed...
January 1, 2017: Experimental Biology and Medicine
https://www.readbyqxmd.com/read/28053185/macrophage-ltb4-drives-efficient-phagocytosis-of-borrelia-burgdorferi-via-blt1-or-blt2
#14
Yan Zhang, Rachel M Olson, Charles R Brown
Unresolved experimental Lyme arthritis in C3H 5-LOX-/- mice is associated with impaired macrophage phag-ocytosis of Borrelia burgdorferi. In the present study, we further investigated the effects of the 5-LOX metabolite, LTB4, on phagocytosis of B. burgdorferi. Bone marrow-derived macrophages (BMDM) from 5-LOX-/- mice were defective in the uptake and killing of B. burgdorferi from the earliest stages of spirochete internali-zation. BMDM from mice deficient for the LTB4 high-affinity receptor (BLT1-/-) were also unable to efficiently phagocytose B...
January 4, 2017: Journal of Lipid Research
https://www.readbyqxmd.com/read/28040200/follow-up-interval-for-small-untreated-pulmonary-arteriovenous-malformations-in-hereditary-haemorrhagic-telangiectasia
#15
D J Ryan, T M O'Connor, M M Murphy, A P Brady
AIM: To investigate the natural history of untreated small (<3 mm) and microscopic pulmonary arteriovenous malformations (PAVMs) in hereditary haemorrhagic telangiectasia (HHT) in order to discern the optimal frequency of follow-up thoracic computed tomography (CT). MATERIALS AND METHODS: A retrospective analysis was performed on the follow-up data for definite and suspected HHT patients with untreated PAVMs. RESULTS: For small PAVMs in definite HHT (n=13), PAVM enlargement was identified in one patient (1/13, 7...
March 2017: Clinical Radiology
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
#16
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/28024482/-effects-of-homoharringtonine-combined-with-imatinib-on-k562-cell-apoptosis-and-bcl6-expression
#17
Qian Wang, Wei Ding, Jing-Jing Wu, Yi-Han Ding, Yu-Feng Li
OBJECTIVE: To explore the effects of homoharringtonine (HHT) alone or combined with imatinib (IM) on K562 cell proliferation and apoptosis, as well as the mRNA and protein expression of BCL6. METHODS: The CCK-8 was used to detect the inhibitory effect of drugs on cell growth, the flow cytometry was used to detect the cell apoptosis. The expression of BCL6 protein was assayed by Western blot, and BCL6 mRNA expression was detected by RT-PCR. RESULTS: HHT alone displayed a proliferation inhibition effect with dose- dependent manner, and induced apotosis; after combination of HHT and IM drugs, both the inhibitory rate and the apoptosis rate were significantly increased compared with the drug alone(P<0...
December 2016: Zhongguo Shi Yan Xue Ye Xue za Zhi
https://www.readbyqxmd.com/read/28024471/-combination-of-homoharringtonine-with-arsenic-trioxide-induces-apoptosis-of-human-acute-myeloid-leukemia-cell-line-u937
#18
Ping Chen, Xi Jiang, Pei-Dong You, Qing Jin, Qin Yuan, Hui-Fang Huang
OBJECTIVE: To investigate the effects and mechanisms of the combination of homoharringtonine (HHT) with arsenic trioxide(As2O3) on human myeloid cell line U937 in vitro. METHODS: MTT method was used to determine the antiproliferating effect of different concentrations of HHT, As2O3 and their combination on U937 cells; the flow cytometry with Annexin-V-FITC/PI double staining was used to determine the apoptosis-induced effect of HHT and As2O3 alone or their combination; Western blot method was used to detect the protein expression of P-Akt(Ser473),P-Akt(Thr308),BCL-XL, BID,MCL-1,P-MCL-1 and so on...
December 2016: Zhongguo Shi Yan Xue Ye Xue za Zhi
https://www.readbyqxmd.com/read/28005445/preparation-and-in-vivo-safety-evaluations-of-antileukemic-homoharringtonine-loaded-pegylated-liposomes
#19
Dong Liu, Jing Xing, Fei Xiong, Fang Yang, Ning Gu
In order to improve the in vivo safety and specific delivery efficiency of the antileukemic homoharringtonine (HHT) at the targets, the long-circulating PEGylated liposomes loaded with HHT (LCLipo-HHT) were prepared. Their physical characteristics, in vitro drug release, in vivo pharmacokinetic properties and elementary toxicity were evaluated. The mean diameter of the prepared LCLipo-HHT is 75.6 ± 3.2 nm and the zeta potential is -16.9 ± 2.5 mV. The entrapment efficiency (EE) of HHT in the liposomes is 69...
December 22, 2016: Drug Development and Industrial Pharmacy
https://www.readbyqxmd.com/read/27997001/-a-rare-cardiomegaly
#20
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
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