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Vascular malformations

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https://www.readbyqxmd.com/read/28816965/blue-rubber-bleb-nevus-syndrome-our-experience-and-new-endoscopic-management
#1
Wenguo Chen, Hongtan Chen, Guodong Shan, Ming Yang, Fengling Hu, Qi Li, Lihua Chen, Guoqiang Xu
The aim of our study is to enhance the awareness of blue rubber bleb nevus syndrome (BRBNS) through the patients in our hospital and introduced a new measure of endoscopic intervention.A retrospective review of 5 patients, who were diagnosed as BRBNS in our hospital from January 2013 to January 2017, was conducted. Data were collected with regard to demographics, clinical presentation, endoscopic and imaging findings, management, and follow-up data.In total of 5 patients, the mean age was 28.8 years, range 16 to 44 years (male/female, 1/4) with the average initial age of onset 15...
August 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/28816935/perimesencephalic-nonaneurysmal-subarachnoid-hemorrhage-caused-by-transverse-sinus-thrombosis-a-case-report-and-review-of-literature
#2
Fang-Wang Fu, Jie Rao, Yuan-Yuan Zheng, Liang Song, Wei Chen, Qi-Hui Zhou, Jian-Guang Yang, Jiang-Qiong Ke, Guo-Qing Zheng
RATIONALE: Perimesencephalic nonaneurysmal subarachnoid hemorrhage (PNSAH) is characterized by a pattern of extravasated blood restricted to the perimesencephalic cisterns, normal angiographic findings, and an excellent prognosis with an uneventful course and low risks of complication. The precise etiology of bleeding in patients with PNSAH has not yet been established. The most common hypothesis is that PNSAH is venous in origin. Intracranial venous hypertension has been considered as the pivotal factor in the pathogenesis of PNSAH...
August 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/28814672/vipar-a-quantitative-approach-to-3d-histopathology-applied-to-lymphatic-malformations
#3
René Hägerling, Dominik Drees, Aaron Scherzinger, Cathrin Dierkes, Silvia Martin-Almedina, Stefan Butz, Kristiana Gordon, Michael Schäfers, Klaus Hinrichs, Pia Ostergaard, Dietmar Vestweber, Tobias Goerge, Sahar Mansour, Xiaoyi Jiang, Peter S Mortimer, Friedemann Kiefer
BACKGROUND: Lack of investigatory and diagnostic tools has been a major contributing factor to the failure to mechanistically understand lymphedema and other lymphatic disorders in order to develop effective drug and surgical therapies. One difficulty has been understanding the true changes in lymph vessel pathology from standard 2D tissue sections. METHODS: VIPAR (volume information-based histopathological analysis by 3D reconstruction and data extraction), a light-sheet microscopy-based approach for the analysis of tissue biopsies, is based on digital reconstruction and visualization of microscopic image stacks...
August 17, 2017: JCI Insight
https://www.readbyqxmd.com/read/28811547/up-regulation-of-nadph-oxidase-mediated-redox-signaling-contributes-to-the-loss-of-barrier-function-in-krit1-deficient-endothelium
#4
Luca Goitre, Peter V DiStefano, Andrea Moglia, Nicholas Nobiletti, Eva Baldini, Lorenza Trabalzini, Julie Keubel, Eliana Trapani, Vladimir V Shuvaev, Vladimir R Muzykantov, Ingrid H Sarelius, Saverio Francesco Retta, Angela J Glading
The intracellular scaffold KRIT1/CCM1 is an established regulator of vascular barrier function. Loss of KRIT1 leads to decreased microvessel barrier function and to the development of the vascular disorder Cerebral Cavernous Malformation (CCM). However, how loss of KRIT1 causes the subsequent deficit in barrier function remains undefined. Previous studies have shown that loss of KRIT1 increases the production of reactive oxygen species (ROS) and exacerbates vascular permeability triggered by several inflammatory stimuli, but not TNF-α...
August 15, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28808648/traumatic-arteriovenous-malformation-of-the-superficial-temporal-artery
#5
Mahmoud Omrani Fard, Omid Yousofnejad, Mohammadbagher Heydari
Most of the vascular lesions on head and neck soft tissue are congenital, but a rare cause can be trauma. A 23-year-old man came to our clinic with a wide pulsatile tortuous mass in the left temporofrontal area. That mass was appeared since 10 years ago. Ten years before his coming to our clinic, he had a blunt trauma in that area. After that, a small wound appeared there and healed gradually. In clinical examination, mass was large and pulsatile, and a fine murmur was detected from it. In paraclinical examination, computed tomography scan with intravenous contrast and sonography revealed a vascular mass with arteriovenous (AV) fistula in soft tissue only in that area...
2017: Advanced Biomedical Research
https://www.readbyqxmd.com/read/28806758/developmental-vascular-remodeling-defects-and-postnatal-kidney-failure-in-mice-lacking-gpr116-adgrf5-and-eltd1-adgrl4
#6
Shun Lu, Shuya Liu, Astrid Wietelmann, Baktybek Kojonazarov, Ann Atzberger, Cong Tang, Ralph Theo Schermuly, Hermann-Josef Gröne, Stefan Offermanns
GPR116 (ADGRF5) and ELTD1 (ADGRL4) belong to different subfamilies of the adhesion G-protein-coupled receptor group but are both expressed in endothelial cells. We therefore analyzed their functions in mice lacking these receptors. While loss of GPR116 or ELTD1 alone had no obvious effect on cardiovascular or kidney function, mice lacking both, GPR116 and ELTD1, showed malformations of the aortic arch arteries and the cardiac outflow tract leading to perinatal lethality in about 50% of the mutants. In addition to cardiovascular malformations, surviving mice developed renal thrombotic microangiopathy as well as hemolysis and splenomegaly, and their lifespan was significantly reduced...
2017: PloS One
https://www.readbyqxmd.com/read/28805772/-diagnosis-and-treatment-of-extracranial-internal-carotid-artery-lesion-combined-with-cerebrovascular-malformation
#7
A I Khripun, A D Pryamikov, A B Mironkov, S A Asratyan, I N Tyurin, V S Suryakhin, M V Abashin, V P Mikhaylenko
AIM: To analyze own experience of diagnosis and treatment of patients with extracranial internal carotid artery lesion and cerebrovascular malformations. MATERIAL AND METHODS: There were 16 patients with combined lesion of extracranial and intracranial internal carotid artery for the period January 2013 - December 2014. Occlusive-stenotic lesion and tortuosity of ICA were observed in 11 and 5 cases respectively. RESULTS: Incidence of combined ICA lesion was 5...
2017: Khirurgiia
https://www.readbyqxmd.com/read/28804779/language-mapping-using-t2-prepared-bold-functional-mri-in-the-presence-of-large-susceptibility-artifacts-initial-results-in-patients-with-brain-tumor-and-epilepsy
#8
Jun Hua, Xinyuan Miao, Shruti Agarwal, Chetan Bettegowda, Alfredo Quiñones-Hinojosa, John Laterra, Peter C M Van Zijl, James J Pekar, Jay J Pillai
At present, presurgical functional mapping is the most prevalent clinical application of functional magnetic resonance imaging (fMRI). Signal dropouts and distortions caused by susceptibility effects in the current standard echo planar imaging (EPI)-based fMRI images are well-known problems and pose a major hurdle for the application of fMRI in several brain regions, many of which are related to language mapping in presurgical planning. Such artifacts are particularly problematic in patients with previous surgical resection cavities, craniotomy hardware, hemorrhage, and vascular malformation...
June 2017: Tomography: a Journal for Imaging Research
https://www.readbyqxmd.com/read/28802071/microrna-137-and-195-inhibit-vasculogenesis-in-brain-arteriovenous-malformations
#9
Jun Huang, Jianping Song, Meijie Qu, Yang Wang, Qinzhu An, Yaying Song, Wei Yan, Bingshun Wang, Xiaojin Wang, Song Zhang, Xi Chen, Bing Zhao, Peixi Liu, Tongyi Xu, Zhijun Zhang, David A Greenberg, Yongting Wang, Pingjin Gao, Wei Zhu, Guo-Yuan Yang
OBJECTIVES: Brain arteriovenous malformations (AVMs) are the most common cause of non-traumatic intracerebral hemorrhage in young adults. The genesis of brain AVM remains enigmatic. We investigated microRNA (miRNA) expression and its contribution to the pathogenesis of brain AVMs. METHODS: We used a large-scale miRNA analysis on 16 samples including AVMs, hemangioblastoma, and controls to identify a distinct AVM miRNA signature. AVM smooth muscle cells (AVMSMCs) were isolated and identified by flow cytometry and immunohistochemistry and candidate miRNAs were then tested in these cells...
August 12, 2017: Annals of Neurology
https://www.readbyqxmd.com/read/28796572/endoglin-and-alk1-as-therapeutic-targets-for-hereditary-hemorrhagic-telangiectasia
#10
Lidia Ruiz-Llorente, Eunate Gallardo-Vara, Elisa Rossi, David M Smadja, Luisa M Botella, Carmelo Bernabeu
Hereditary Haemorrhagic Telangiectasia (HHT) is as an autosomal dominant trait characterized by frequent nose bleeds, mucocutaneous telangiectases, arteriovenous malformations (AVMs) of the lung, liver and brain, and gastrointestinal bleedings due to telangiectases. HHT is originated by mutations in genes whose encoded proteins are involved in the transforming growth factor β (TGF-β) family signalling of vascular endothelial cells. In spite of the great advances in the diagnosis as well as in the molecular, cellular and animal models of HHT, the current treatments remain just at the palliative level...
August 10, 2017: Expert Opinion on Therapeutic Targets
https://www.readbyqxmd.com/read/28791278/venous-thromboembolism-in-pediatric-vascular-anomalies
#11
REVIEW
Taizo A Nakano, Chadi Zeinati
The presence of a vascular anomaly suggests that capillaries, veins, arteries, and/or lymphatic vessels have demonstrated abnormal development and growth. Often dilated and misshaped, these vessels augment normal flow of blood and lymphatic fluids that increases the overall risk to develop intralesional thrombosis. Abnormal endothelial and lymphoendothelial cells activate hemostasis and hyperfibrinolytic pathways through poorly understood mechanisms, which contribute to the development of localized intravascular coagulopathy...
2017: Frontiers in Pediatrics
https://www.readbyqxmd.com/read/28780588/spontaneous-resolution-of-low-flow-spinal-arteriovenous-fistulas
#12
Joe Kang, Lydia Gregg, Philippe Gailloud
PURPOSE: Spinal vascular anomalies with arteriovenous blood shunting include spinal arteriovenous malformations (SAVMs) and spinal arteriovenous fistulas (SAVFs), which are distinguished by the presence or absence of an interposed nidus. SAVFs can be further characterized based on their location (perimedullary, dural, or extradural) and flow pattern (high-flow versus low-flow shunts). The spontaneous resolution of a spinal vascular malformation, i.e., the complete disappearance-in the absence of therapeutic measures-of a lesion previously identified by angiography, seems to represent an exceptional phenomenon...
August 5, 2017: Neuroradiology
https://www.readbyqxmd.com/read/28777021/masson-s-tumor-of-the-pineal-region-case-report
#13
Lefko T Charalambous, Anirudh Penumaka, Jordan M Komisarow, Amanda C Hemmerich, Thomas J Cummings, Patrick J Codd, Allan H Friedman
Intracranial intravascular papillary endothelial hyperplasia (IPEH), also referred to as Masson's tumor, is a condition that rarely occurs in the nervous system. IPEH most frequently occurs extracranially in the skin of the face, skull, neck, and trunk and can easily be mistaken clinically, radiologically, and histologically for angiosarcoma, organizing hematoma, or other vascular malformations. IPEH accounts for roughly 2% of all vascular tumors and is extremely rare intracranially, with only 23 reported cases compared with more than 300 cases of IPEH occurring in the skin and subcutaneous tissue...
August 4, 2017: Journal of Neurosurgery
https://www.readbyqxmd.com/read/28775059/basal-ganglia-t1-hyperintensity-in-hereditary-hemorrhagic-telangiectasia
#14
A Parvinian, V N Iyer, B S Pannu, D R Apala, C P Wood, W Brinjikji
BACKGROUND AND PURPOSE: The implications of basal ganglia T1 hyperintensity remain unclear in patients with hereditary hemorrhagic telangiectasia. This study was performed to assess the prevalence of this imaging finding in a large cohort of patients with hereditary hemorrhagic telangiectasia and to identify any association between this phenomenon and other disease manifestations. MATERIALS AND METHODS: In this retrospective study, we identified all patients at our institution diagnosed with definite hereditary hemorrhagic telangiectasia from 2001 to 2017...
August 3, 2017: AJNR. American Journal of Neuroradiology
https://www.readbyqxmd.com/read/28764614/quantitative-evaluation-of-arteriovenous-malformation-hemodynamic-changes-after-endovascular-treatment-using-parametric-color-coding-a-case-series-study
#15
Rodrigo Rivera, Juan G Sordo, Daniel Echeverria, Lautaro Badilla, Camila Pinto, Catalina Merino-Osorio
Background Brain arteriovenous malformations (AVMs) are complex vascular lesions. Endovascular treatment results are usually measured by calculating the volume reduction of the lesions. Nevertheless, vascular flow quantification seems a more physiologically accurate way of measuring endovascular results. We evaluated the use of parametric color coding (PCC) with digital subtraction angiography (DSA), in order to determine the feasibility of PCC to detect and measure the impact of AVM endovascular treatment-induced changes using real-time hemodynamic parameters...
January 1, 2017: Interventional Neuroradiology
https://www.readbyqxmd.com/read/28764210/kimura-s-disease-without-peripheral-eosinophilia-an-unusual-and-challenging-case-simulating-venous-malformation-on-imaging-studies-case-report-and-review-of-literature
#16
REVIEW
Vivek Dokania, Digvijay Patil, Ketan Agarwal, Prajakta Thakur, Piyush Prajapati
Kimura's Disease (KD) is a rare chronic inflammatory disorder presenting as multiple painless solitary subcutaneous nodules, predominantly in the head and neck region and frequently associated with regional lymphadenopathy and/or salivary gland involvement. Because of painless nature and indolent course, there is usually a delay in the patient's presentation. KD may radiologically mimic other chronic inflammatory conditions like tuberculosis, vascular malformations and neoplasms. Clinical correlation and histological evaluation along with elevated peripheral eosinophil and serum IgE level are considered important for confirmatory diagnosis...
June 2017: Journal of Clinical and Diagnostic Research: JCDR
https://www.readbyqxmd.com/read/28763068/experience-in-the-corrective-treatment-of-patients-with-atrioventricular-septum
#17
Yuriria Olivares-Fernández, Áurea Zetina-Solórzano
INTRODUCTION: Atrioventricular septal defects are a wide spectrum of cardiac malformations, from partial until complete with one unique atrioventricular valve, atrioventricular valve communication, and leaky left heart valve. Its fast evolution to pulmonary vascular disease calls for early surgical management. Corrective treatment has a high percentage of re-operations and 8.6% mortality. OBJECTIVES: To describe the results of corrective treatments of atrioventricular septum defects in our institution's patients...
May 2017: Gaceta Médica de México
https://www.readbyqxmd.com/read/28761851/immunohistochemical-expression-of-wt-1-helps-to-differentiate-cutaneous-vascular-tumors-from-vascular-malformations
#18
Manisha Choudhary, Ira Sharma, Manveen Kaur, Varsha Dalal, Avninder Singh
No abstract text is available yet for this article.
July 2017: Indian Dermatology Online Journal
https://www.readbyqxmd.com/read/28761841/multiple-verrucous-hemangiomas-a-case-report-with-new-therapeutic-insight
#19
Jasmeet Singh, Preeti Sharma, Sidharth Tandon, Surabhi Sinha
Verrucous hemangioma is an uncommon congenital vascular malformation, which may clinically masquerade angiokeratoma, lymphangioma circumscriptum, or malignant melanoma. Differentiation is essential owing to varied therapeutic and prognostic implications. We present a rare case of multiple verrucous hemangiomas in a teenage girl who presented with multiple warty lesions over the dorsal aspect of the left foot since birth. Magnetic resonance imaging (MRI) scan was suggestive of a vascular malformation, and skin biopsy showed ectatic blood vessels extending from the papillary dermis into the subcutaneous tissue, diagnostic of verrucous hemangioma...
July 2017: Indian Dermatology Online Journal
https://www.readbyqxmd.com/read/28761454/meticulous-observations-essential-before-and-after-coil-embolization-of-pulmonary-arteriovenous-malformation-lessons-learned-from-two-case
#20
Ramin Heidari, Mohaddeseh Behjati, Abbas Balouchi
BACKGROUND: Endovascular coil embolization is an approved treatment for pulmonary arteriovenous malformation (AVM) but it brings high rate of thromboembolic complications with subsequent morbidity and mortality. Hereby, we report two cases of AVM coil embolization and management of their compilations. CASE REPORT: The first case was a 57-year-old male with five implanted coils in the lower lobe of right lung in which two of them were migrated soon after implantation...
January 2017: ARYA Atherosclerosis
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