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https://www.readbyqxmd.com/read/29048420/characterization-of-pulmonary-arteriovenous-malformations-in-acvrl1-versus-eng-mutation-carriers-in-hereditary-hemorrhagic-telangiectasia
#1
Weiyi Mu, Zachary A Cordner, Kevin Yuqi Wang, Kate Reed, Gina Robinson, Sally Mitchell, Doris Lin
PurposePulmonary arteriovenous malformations (pAVMs) are major contributors to morbidity and mortality in hereditary hemorrhagic telangiectasia (HHT). Mutations in ENG and ACVRL1 underlie the vast majority of clinically diagnosed cases. The aims of this study were to characterize and compare the clinical and morphologic features of pAVMs between these two genotype groups.MethodsSixty-six patients with HHT and affected family members were included. Genotype, phenotypic data, and imaging were obtained from medical records...
October 19, 2017: Genetics in Medicine: Official Journal of the American College of Medical Genetics
https://www.readbyqxmd.com/read/29042026/identification-signaling-and-functions-of-ltb4-receptors
#2
REVIEW
Kazuko Saeki, Takehiko Yokomizo
Leukotriene B4 (LTB4), a lipid mediator produced from arachidonic acid, is a chemoattractant for inflammatory leukocytes. We identified two receptors for LTB4, the high-affinity receptor BLT1 and the low-affinity receptor BLT2. BLT1 is expressed in various subsets of leukocytes, and analyses of BLT1-deficient mice revealed that the LTB4/BLT1 axis enhances leukocyte recruitment to infected sites, and is involved in the elimination of pathogens. Hyperactivation of the LTB4/BLT1 axis induces acute and chronic inflammation, resulting in various inflammatory diseases...
October 2017: Seminars in Immunology
https://www.readbyqxmd.com/read/29038497/non-steroidal-anti-inflammatory-drug-delays-corneal-wound-healing-by-reducing-production-of-12-hydroxyheptadecatrienoic-acid-a-ligand-for-leukotriene-b4-receptor-2
#3
Satoshi Iwamoto, Tomoaki Koga, Mai Ohba, Toshiaki Okuno, Masato Koike, Akira Murakami, Akira Matsuda, Takehiko Yokomizo
Non-steroidal anti-inflammatory drugs (NSAIDs) are widely used to reduce inflammation by suppressing cyclooxygenases (COXs). NSAID eye drops are frequently prescribed after ocular surgery to reduce inflammation and pain, but this treatment has clinically significant side effects, including corneal ulcer and perforation. The molecular mechanisms underlying these side effects remain unknown. Recently, the COX product 12(S)-hydroxyheptadeca-5Z,8E,10E-trienoic acid (12-HHT) was identified as an endogenous ligand for leukotriene B4 receptor 2 (BLT2), which is important in maintenance of epithelial homeostasis...
October 16, 2017: Scientific Reports
https://www.readbyqxmd.com/read/29032044/multiplex-quantitative-analysis-of-eicosanoid-mediators-in-human-plasma-and-serum-possible-introduction-into-clinical-testing
#4
Atsushi Yasumoto, Suzumi M Tokuoka, Yoshihiro Kita, Takao Shimizu, Yutaka Yatomi
Eicosanoid mediators play important roles in maintaining the physiological and pathophysiological homeostasis in the body. Their measurements, however, are rarely performed in clinical practice. In the present study, we analyzed 30 varieties of eicosanoid mediators that were detectable in human plasma and serum collected from healthy donors, using liquid chromatography-tandem mass spectrometry from the viewpoint of the clinical application of the multiplex quantitation of eicosanoid mediators. Wider variety of eicosanoid mediators were detected in serum (27 out of 30) than in plasma (14 out of 30), since the serum was thought to contain lipid mediators released from activated platelets...
October 9, 2017: Journal of Chromatography. B, Analytical Technologies in the Biomedical and Life Sciences
https://www.readbyqxmd.com/read/29018941/pulmonary-arteriovenous-malformations-in-hereditary-haemorrhagic-telangiectasia-correlations-between-computed-tomography-findings-and-cerebral-complications
#5
Johan Etievant, Salim Si-Mohamed, Nicolas Vinurel, Sophie Dupuis-Girod, Evelyne Decullier, Delphine Gamondes, Chahera Khouatra, Vincent Cottin, Didier Revel
OBJECTIVES: Computed tomography (CT) is the modality of choice to characterise pulmonary arteriovenous malformations (PAVMs) in patients with hereditary haemorrhagic telangiectasia (HHT). Our objective was to determine if CT findings were associated with frequency of brain abscess and ischaemic stroke. METHODS: This retrospective study included patients with HHT-related PAVMs. CT results, i.e. PAVM presentation (unique, multiple, disseminated or diffuse), the number of PAVMs and the largest feeding artery size, were correlated to prevalence of ischaemic stroke and brain abscess...
October 10, 2017: European Radiology
https://www.readbyqxmd.com/read/28989145/-clinical-and-genetic-diagnosis-in-a-hereditary-hemorrhagic-telangiectasia-family
#6
Yan Yi, Xiaofeng Liu, Boda Wu, Jun Liu, Shenglei Ge, Xiaoliu Shi
To investigate the clinical features and feasibility genetic diagnosis in a hereditary hemorrhagic telangiectasia (HHT) family, and to explore the application of gene mutation testing in HHT diagnosis.
 Methods: Medical histories and clinical features of a family were analyzed to diagnose HHT patients and suspected individuals according to the clinical diagnostic criteria. Sequence analysis of endoglin (ENG) and activin A receptor like type 1 (ACVRL1) gene in the proband was performed with PCR and Sanger sequencing technology...
September 28, 2017: Zhong Nan da Xue Xue Bao. Yi Xue Ban, Journal of Central South University. Medical Sciences
https://www.readbyqxmd.com/read/28981519/pulmonary-hypertension-subtypes-associated-with-hereditary-haemorrhagic-telangiectasia-haemodynamic-profiles-and-survival-probability
#7
Sabine Revuz, Evelyne Decullier, Isabelle Ginon, Nicolas Lamblin, Pierre-Yves Hatron, Pierre Kaminsky, Marie-France Carette, Pascal Lacombe, Anne-Claire Simon, Sophie Rivière, Jean-Robert Harlé, Alain Fraisse, Christian Lavigne, Vanessa Leguy-Seguin, Ari Chaouat, Chahera Khouatra, Sophie Dupuis-Girod, Eric Hachulla
BACKGROUND: Different pulmonary hypertension (PH) mechanisms are associated with hereditary haemorrhagic telangiectasia (HHT). METHODS AND RESULTS: We conducted a retrospective study of all suspected cases of PH (echocardiographically estimated systolic pulmonary artery pressure [sPAP] ≥ 40 mmHg) in patients with definite HHT recorded in the French National Reference Centre for HHT database. When right heart catheterization (RHC) was performed, PH cases were confirmed and classified among the PH groups according to the European guidelines...
2017: PloS One
https://www.readbyqxmd.com/read/28978360/topical-propranolol-improves-epistaxis-in-patients-with-hereditary-hemorrhagic-telangiectasia-a-preliminary-report
#8
Meir Mei-Zahav, Hannah Blau, Elchanan Bruckheimer, Eyal Zur, Neta Goldschmidt
BACKGROUND: Severe epistaxis is often difficult to control in patients with hereditary hemorrhagic telangiectasia (HHT). Propranolol has been shown to have antiangiogenic properties in vitro and in vivo and is commonly used to treat hemangiomas. We present our experience with topical nasal propranolol for the treatment of moderate to severe epistaxis in patients with HHT. METHODS: Retrospective case series. Six patients with HHT were treated with 0.5 cm(3) of 1...
October 4, 2017: Journal of Otolaryngology—Head & Neck Surgery
https://www.readbyqxmd.com/read/28973643/tacrolimus-rescues-the-signaling-and-gene-expression-signature-of-endothelial-alk1-loss-of-function-and-improves-hht-vascular-pathology
#9
Santiago Ruiz, Pallavi Chandakkar, Haitian Zhao, Julien Papoin, Prodyot K Chatterjee, Erica Christen, Christine N Metz, Lionel Blanc, Fabien Campagne, Philippe Marambaud
Hereditary hemorrhagic telangiectasia (HHT) is a highly debilitating and life-threatening genetic vascular disorder arising from endothelial cell (EC) proliferation and hypervascularization, for which no cure exists. Because HHT is caused by loss-of-function mutations in BMP9-ALK1-Smad1/5/8 signaling, interventions aimed at activating this pathway are of therapeutic value. We interrogated the whole-transcriptome in human umbilical vein ECs (HUVECs) and found that ALK1 signaling inhibition was associated with a specific pro-angiogenic gene expression signature, which included a significant elevation of DLL4 expression...
September 14, 2017: Human Molecular Genetics
https://www.readbyqxmd.com/read/28973426/surgical-treatment-vs-nonsurgical-treatment-for-brain-arteriovenous-malformations-in-patients-with-hereditary-hemorrhagic-telangiectasia-a-retrospective-multicenter-consortium-study
#10
Ali Tayebi Meybodi, Helen Kim, Jeffrey Nelson, Steven W Hetts, Timo Krings, Karel G terBrugge, Marie E Faughnan, Michael T Lawton
BACKGROUND: Cerebral arteriovenous malformations (AVMs) are common in patients with hereditary hemorrhagic telangiectasia (HHT). However, due to the rarity of HHT and little published evidence of outcomes from management of brain AVMs in this disease, current international HHT guidelines recommend an individualized approach. Specifically, the outcomes for surgical vs nonsurgical management of these lesions have not been reported to date. OBJECTIVE: To report long-term outcomes of surgical resection of brain AVMs in HHT patients compared to outcomes in nonsurgically treated patients...
July 4, 2017: Neurosurgery
https://www.readbyqxmd.com/read/28963191/deregulated-tgf-%C3%AE-bmp-signaling-in-vascular-malformations
#11
REVIEW
Sara I Cunha, Peetra U Magnusson, Elisabetta Dejana, Maria Grazia Lampugnani
Correct organization of the vascular tree requires the balanced activities of several signaling pathways that regulate tubulogenesis and vascular branching, elongation, and pruning. When this balance is lost, the vessels can be malformed and fragile, and they can lose arteriovenous differentiation. In this review, we concentrate on the transforming growth factor (TGF)-β/bone morphogenetic protein (BMP) pathway, which is one of the most important and complex signaling systems in vascular development. Inactivation of these pathways can lead to altered vascular organization in the embryo...
September 29, 2017: Circulation Research
https://www.readbyqxmd.com/read/28942104/homoharringtonine-prevents-surgery-induced-epidural-fibrosis-through-endoplasmic-reticulum-stress-signaling-pathway
#12
Xiaolei Li, Shuguang Wang, Jihang Dai, Lianqi Yan, Shuai Zhao, Jingcheng Wang, Yu Sun
Surgery-induced epidural fibrosis after laminectomy often results in poor clinical outcomes. Fibroblasts proliferation is considered to be one of the major causes of epidural fibrosis formation. Previously, there was no research about the effect of Homoharringtonine(HHT) on inhibiting fibroblast proliferation and reducing epidural fibrosis. Here, we performed in vitro and in vivo experiments to explore the effect of HHT on inhibiting fibroblast proliferation, inducing fibroblast apoptosis and preventing epidural fibrosis formation...
September 20, 2017: European Journal of Pharmacology
https://www.readbyqxmd.com/read/28936439/a-case-of-pulmonary-arteriovenous-malformation-role-of-interventional-radiology-in-diagnosis-and-treatment
#13
Pranav Sharma, Puneet Kochar, Salil Sharma, Nishant Gupta, Shuo Li, Kusum Hooda, Yogesh Kumar
Pulmonary arterio-venous malformations (PAVMs) are abnormal pulmonary arteries and pulmonary veins communicating directly without interposition of a capillary bed and about 80-90% of patients with PAVMs eventually may present with hereditary hemorrhagic telangiectasia (HHT), remaining ones are sporadic cases. On the other hand, about 15-35% of HHT patients may present with PAVMs. The PAVMs have a tendency to grow and increase in size over time and various factors like puberty, pregnancy and pulmonary arterial hypertension (PAH) affect growth...
September 2017: Annals of Translational Medicine
https://www.readbyqxmd.com/read/28931195/hereditary-hemorrhagic-telangiectasia-laser-treatment-of-epistaxis
#14
Gorazd Poje, Marcel Marjanović Kavanagh
Hereditary hemorrhagic telangiectasia (HHT) is a rare, autosomal dominant disorder characterized by recurrent epistaxis, telangiectasias, and multiorgan vascular dysplasia. Various modalities exist for the treatment of HHT-related chronic epistaxis, although no method is preferred over another. The aim of this study was to review the effectiveness of diode laser photocoagulation in the treatment of epistaxis in patients with HHT. The study included 17 patients (7 men, 10 women) treated with diode laser photocoagulation from year 2008 to 2012...
September 2017: Ear, Nose, & Throat Journal
https://www.readbyqxmd.com/read/28890303/a-simulation-framework-for-humeral-head-translations
#15
Ehsan Sarshari, Alain Farron, Alexandre Terrier, Dominique Pioletti, Philippe Mullhaupt
Humeral head translations (HHT) play a crucial role in the glenohumeral (GH) joint function. The available shoulder musculoskeletal models developed based on inverse dynamics however fall short of predicting the HHT. This study aims at developing a simulation framework that allows forward-dynamics simulation of a shoulder musculoskeletal model with a 6 degrees of freedom (DOF) GH joint. It provides a straightforward solution to the HHT prediction problem. We show that even within a forward-dynamics simulation addressing the HHT requires further information about the contact...
September 7, 2017: Medical Engineering & Physics
https://www.readbyqxmd.com/read/28874282/smad4-gene-mutation-increases-the-risk-of-aortic-dilation-in-patients-with-hereditary-haemorrhagic-telangiectasia
#16
V M M Vorselaars, A Diederik, V Prabhudesai, S Velthuis, J-A Vos, R J Snijder, C J J Westermann, B J Mulder, J K Ploos van Amstel, J J Mager, M E Faughnan, M C Post
BACKGROUND: Mutations in the genes ENG, ACVRL1 and SMAD4 that are part of the transforming growth factor-beta signalling pathway cause hereditary haemorrhagic telangiectasia (HHT). Mutations in non-HHT genes within this same pathway have been found to associate with aortic dilation. Therefore, we investigated the presence of aortic dilation in a large cohort of HHT patients as compared to non-HHT controls. METHODS: Chest computed tomography of consecutive HHT patients (ENG, ACVRL1 and SMAD4 mutation carriers) and non-HHT controls were reviewed...
October 15, 2017: International Journal of Cardiology
https://www.readbyqxmd.com/read/28871312/alk1-signaling-in-development-and-disease-new-paradigms
#17
REVIEW
Beth L Roman, Andrew P Hinck
Activin A receptor like type 1 (ALK1) is a transmembrane serine/threonine receptor kinase in the transforming growth factor-beta receptor family that is expressed on endothelial cells. Defects in ALK1 signaling cause the autosomal dominant vascular disorder, hereditary hemorrhagic telangiectasia (HHT), which is characterized by development of direct connections between arteries and veins, or arteriovenous malformations (AVMs). Although previous studies have implicated ALK1 in various aspects of sprouting angiogenesis, including tip/stalk cell selection, migration, and proliferation, recent work suggests an intriguing role for ALK1 in transducing a flow-based signal that governs directed endothelial cell migration within patent, perfused vessels...
September 4, 2017: Cellular and Molecular Life Sciences: CMLS
https://www.readbyqxmd.com/read/28863586/the-energy-based-characteristics-of-sperm-whale-clicks-using-the-hilbert-huang-transform-analysis-method
#18
Chin-Feng Lin, Yao-Ching Chung, Jin-De Zhu, Shun-Hsyung Chang, Chan-Chuan Wen, Ivan A Parinov, S N Shevtsov
In this paper, a unique analysis method for sperm whale clicks based on Hilbert-Huang transform (HHT) is proposed. Four sperm whale click samples with durations of 10 ms (defined as click I), and four sperm whale click samples with durations of 5 ms (defined as click II) were illustrated. These click samples were recorded in the Mediterranean Sea by Centro Interdisciplinare di Bioacusticae Ricerche Ambientali, Università degli Studi di Pavia. The empirical mode decomposition method was used to decompose click I samples into seven intrinsic mode functions (IMFs) and one residue function (RF), and click II samples were decomposed into six IMFs and one RF...
August 2017: Journal of the Acoustical Society of America
https://www.readbyqxmd.com/read/28839431/hwangryunhaedok-tang-induces-the-depolarization-of-pacemaker-potentials-through-5-ht3-and-5-ht4-receptors-in-cultured-murine-small-intestine-interstitial-cells-of-cajal
#19
Hyun Jung Kim, Guem San Lee, Hyungwoo Kim, Byung Joo Kim
AIM: To investigate the effects of a water extract of Hwangryunhaedok-tang (HHTE) on the pacemaker potentials of mouse interstitial cells of Cajal (ICCs). METHODS: We dissociated ICCs from small intestines and cultured. ICCs were immunologically identified using an anti-c-kit antibody. We used the whole-cell patch-clamp configuration to record the pacemaker potentials generated by cultured ICCs under the current clamp mode (I = 0). All experiments were performed at 30 °C-32 °C...
August 7, 2017: World Journal of Gastroenterology: WJG
https://www.readbyqxmd.com/read/28838429/cephalotaxus-alkaloids
#20
Joëlle Pérard-Viret, Laith Quteishat, Rana Alsalim, Jacques Royer, Françoise Dumas
Cephalotaxus alkaloids represent a family of plant secondary metabolites known for 60 years. Significant activity against leukemia in mice was demonstrated for extracts of Cephalotaxus. Cephalotaxine (CET) (1), the major alkaloid of this series was isolated from Cephalotaxus drupacea species by Paudler in 1963. The subsequent discovery of promising antitumor activity among new Cephalotaxus derivatives reported by Chinese, Japanese, and American teams triggered extensive structure elucidation and biological studies in this family...
2017: Alkaloids. Chemistry and Biology
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