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https://www.readbyqxmd.com/read/28070641/influence-of-temporary-nasal-occlusion-tno-on-epistaxis-frequency-in-patients-with-hereditary-hemorrhagic-telangiectasia-hht
#1
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
#2
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
#3
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
#4
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
#5
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
#6
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
#7
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...
December 29, 2016: 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
#8
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
#9
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
#10
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
#11
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
#12
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/27936056/spatial-temporal-analysis-of-environmental-data-of-north-beijing-district-using-hilbert-huang-transform
#13
Yu Xiang, Xuezhi Wang, Lihua He, Wenyong Wang, William Moran
Temperature, solar radiation and water are major important variables in ecosystem models which are measurable via wireless sensor networks (WSN). Effective data analysis is necessary to extract significant spatial and temporal information. In this work, information regarding the long term variation of seasonal field environment conditions is explored using Hilbert-Huang transform (HHT) based analysis on the wireless sensor network data collection. The data collection network, consisting of 36 wireless nodes, covers an area of 100 square kilometres in Yanqing, the northwest of Beijing CBD, in China and data collection involves environmental parameter observations taken over a period of three months in 2011...
2016: PloS One
https://www.readbyqxmd.com/read/27918414/wearable-sensor-based-human-activity-recognition-method-with-multi-features-extracted-from-hilbert-huang-transform
#14
Huile Xu, Jinyi Liu, Haibo Hu, Yi Zhang
Wearable sensors-based human activity recognition introduces many useful applications and services in health care, rehabilitation training, elderly monitoring and many other areas of human interaction. Existing works in this field mainly focus on recognizing activities by using traditional features extracted from Fourier transform (FT) or wavelet transform (WT). However, these signal processing approaches are suitable for a linear signal but not for a nonlinear signal. In this paper, we investigate the characteristics of the Hilbert-Huang transform (HHT) for dealing with activity data with properties such as nonlinearity and non-stationarity...
December 2, 2016: Sensors
https://www.readbyqxmd.com/read/27895866/liver-findings-in-patients-with-hereditary-hemorrhagic-telangiectasia
#15
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
https://www.readbyqxmd.com/read/27876060/20-year-follow-up-study-of-danish-hht-patients-survival-and-causes-of-death
#16
Anette Kjeldsen, Katrine Saldern Aagaard, Pernille Mathiesen Tørring, Sören Möller, Anders Green
BACKGROUND: Hereditary Haemorrhagic Telangiectasia (HHT) is a dominantly inheritable disorder, with a wide variety of clinical manifestations due to presence of multiple arteriovenous manifestations. The most common mutations are found in HHT1 (ENG) and HHT2 (ACVRL1) patients, causing alterations in the TGF-β pathway which is responsible for angiogenesis. Modulations of angiogenesis may influence cancer rates. The objective of the study was to evaluate 20-year survival according to HHT subtype, as well as to evaluate differences in causes of death comparing HHT patients and controls...
November 22, 2016: Orphanet Journal of Rare Diseases
https://www.readbyqxmd.com/read/27874028/a-mouse-model-of-hereditary-hemorrhagic-telangiectasia-generated-by-transmammary-delivered-immunoblocking-of-bmp9-and-bmp10
#17
Santiago Ruiz, Haitian Zhao, Pallavi Chandakkar, Prodyot K Chatterjee, Julien Papoin, Lionel Blanc, Christine N Metz, Fabien Campagne, Philippe Marambaud
Hereditary hemorrhagic telangiectasia (HHT) is a potentially life-threatening genetic vascular disorder caused by loss-of-function mutations in the genes encoding activin receptor-like kinase 1 (ALK1), endoglin, Smad4, and bone morphogenetic protein 9 (BMP9). Injections of mouse neonates with BMP9/10 blocking antibodies lead to HHT-like vascular defects in the postnatal retinal angiogenesis model. Mothers and their newborns share the same immunity through the transfer of maternal antibodies during lactation...
November 22, 2016: Scientific Reports
https://www.readbyqxmd.com/read/27864873/hereditary-haemorrhagic-telangiectasia-to-transplant-or-not-to-transplant-is-there-a-right-time-for-liver-transplantation
#18
Yannick D Muller, Roland Oppliger, Romain Breguet, Philippe Meyer, Laura Rubbia-Brandt, Pierre-Auguste Petignat, Thomas Harr, Eric Dayer, Jörg D Seebach
BACKGROUND & AIMS: Hereditary haemorrhagic telangiectasia is characterized by arterio-venous malformations (AVM). It frequently involves the liver without clinical symptoms, but may lead to biliary ischaemia, portal hypertension, or fatal high-output heart failure. The indication of liver transplantation is controversial. METHODS: Herein, we report the case of a 65-year-old female patient with a 'double Osler syndrome' consisting of hereditary haemorrhagic telangiectasia (HHT) and type I hereditary angioedema diagnosed at the age of 25 and 22 years respectively...
December 2016: Liver International: Official Journal of the International Association for the Study of the Liver
https://www.readbyqxmd.com/read/27864871/hereditary-haemorrhagic-telangiectasia-to-transplant-or-not-to-transplant
#19
Carlo Sabbà
The Association of hereditary hemorrhagic telangiectasia (HHT) and type I hereditary angioedema is a very rare condition in medicine. The case reported by Muller et al., describes the coexistence of the two diseases and emphasizes the need for listing HHT patients for liver transplantation in case indications occurs, such as the presence of HCC, abnormally increased cardiac output, and gastrointestinal bleeding as reported for this case. The case described by Mueller et al. is anecdotal for the usefulness of liver transplantation in HHT patients and shows that liver transplantation may be the best supportive care and, sometimes, the unique and final therapeutic option for these patients...
December 2016: Liver International: Official Journal of the International Association for the Study of the Liver
https://www.readbyqxmd.com/read/27864870/hereditary-hemorrhagic-telangiectasia-to-transplant-or-not-to-transplant
#20
Sophie Dupuis-Girod, Elisabetta Buscarini
This report comments the case reported by Muller et al. which describes a combination of at least two different indications for orthotopic liver transplant (OLT) in a same patient: hepatocarcinoma and HHT complicated with severe liver involvement and high output cardiac failure. This case report highlighted that the clear time for OLT in HHT can be difficult to determine. In HHT, if intensive medical approach is not efficient, OLT, has to be considered. In the case of Muller et al., the patient was correctly listed for OLT for a single hepatocellular carcinoma, however, he did not receive a sufficient priority so as to avoid worsening of liver vascular malformations complications...
December 2016: Liver International: Official Journal of the International Association for the Study of the Liver
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