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Vasculopathy

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https://www.readbyqxmd.com/read/28429859/aneurysms-in-neurofibromatosis-type-2-evidence-for-vasculopathy
#1
Shazia K Afridi, Suki Thomson, Steve E J Connor, Daniel C Walsh, Rosalie E Ferner
There have been anecdotal reports of vasculopathy associated with Neurofibromatosis Type 2 (NF2). Given the increasing use of bevacizumab, a vascular endothelial growth factor inhibitor which results in an increased risk of bleeding, it is important to ascertain if there is a predisposition to vascular abnormalities in NF2. In our unit NF2 patients undergo annual MRI brain and internal auditory meatus imaging. We noted incidental intracranial aneurysms in some patients and sought to determine the prevalence of intracranial aneurysms in our cohort of NF2 patients...
April 21, 2017: American Journal of Medical Genetics. Part A
https://www.readbyqxmd.com/read/28429615/regulation-of-vascular-large-conductance-calcium-activated-potassium-channels-by-nrf2-signalling
#2
Yong Li, Xiao-Li Wang, Xiaojing Sun, Qiang Chai, Jingchao Li, Benjamin Thompson, Win-Kuang Shen, Tong Lu, Hon-Chi Lee
BK channels are major ionic determinants of vasodilation. BK channel function is impaired in diabetic vessels due to accelerated proteolysis of its beta-1 (BK-β1) subunits in response to increased oxidative stress. The nuclear factor E2-related factor-2 (Nrf2) signalling pathway has emerged as a master regulator of cellular redox status, and we hypothesized that it plays a central role in regulating BK channel function in diabetic vessels. We found that Nrf2 expression was markedly reduced in db/db diabetic mouse aortas, and this was associated with significant downregulation of BK-β1...
April 1, 2017: Diabetes & Vascular Disease Research
https://www.readbyqxmd.com/read/28428893/characteristics-and-racial-variations-of-polypoidal-choroidal-vasculopathy-in-tertiary-centers-in-the-united-states-and-united-kingdom
#3
Tarek Alasil, Nelida Munoz, Pearse A Keane, Adnan Tufail, Patrick A Coady, Eduardo Novais, Talisa E de Carlo, Caroline R Baumal, Nadia K Waheed, Jay S Duker, Ron A Adelman
PURPOSE: To evaluate the characteristics and racial variations amongst patients with polypoidal choroidal vasculopathy (PCV) in the United States and the United Kingdom. METHODS: Fundus photos and indocyanine green angiography images were evaluated in a multicenter retrospective study to establish the diagnosis of PCV. Visual acuity (VA) was recorded in ETDRS letter count. RESULTS: Eighty eyes of 71 PCV patients (average age of 69.4 ± 10...
2017: International Journal of Retina and Vitreous
https://www.readbyqxmd.com/read/28425200/high-dose-calcineurin-inhibitor-free-everolimus-as-a-maintenance-regimen-for-heart-transplantation-may-be-a-risk-factor-for%C3%A2-pneumocystis-pneumonia
#4
Yu-Ning Hu, Nan-Yao Lee, Jun-Neng Roan, Chi-Hsin Hsu, Chwan-Yao Luo
BACKGROUND: Everolimus reduces the incidence of cardiac-allograft vasculopathy (CAV) and is less renally toxic than are calcineurin inhibitors (CNIs). We evaluated the safety of CNI-free everolimus for post-heart transplant (HTx) patients. METHODS: We retrospectively reviewed the records of 36 consecutive patients who had undergone an HTx between January 2006 and December 2013 in National Cheng Kung University Hospital. All patients initially had been treated with the standard tacrolimus regimen...
April 20, 2017: Transplant Infectious Disease: An Official Journal of the Transplantation Society
https://www.readbyqxmd.com/read/28422438/moyamoya-syndrome-in-children-with-neurofibromatosis-type-1-italian-french-experience
#5
Claudia Santoro, Federico Di Rocco, Manoelle Kossorotoff, Michel Zerah, Nathalie Boddaert, Raphael Calmon, Dominique Vidaud, Mario Cirillo, Giuseppe Cinalli, Giuseppe Mirone, Teresa Giugliano, Giulio Piluso, Alessandra D'Amico, Valeria Capra, Marco Pavanello, Armando Cama, Bruno Nobili, Stanislas Lyonnet, Silverio Perrotta
Moyamoya syndrome (MMS) is the most common cerebral vasculopathy among children with neurofibromatosis type 1 (NF1). In this study, we clinically, radiologically, and genetically examined a cohort that was not previously described, comprising European children with NF1 and MMS. The NF1 genotyping had been registered. This study included 18 children. The mean age was 2.93 ± 3.03 years at the NF1 diagnosis and 7.43 ± 4.27 years at the MMS diagnosis. In seven patients, MMS was diagnosed before or at the same time as NF1...
April 19, 2017: American Journal of Medical Genetics. Part A
https://www.readbyqxmd.com/read/28422002/treatment-of-psoriasis-with-ustekinumab-improved-skin-tightening-in-systemic-sclerosis
#6
Asako Ichihara, Masatoshi Jinnin, Hironobu Ihn
Systemic sclerosis (SSc) is a chronic autoimmune disease characterised by fibrosis, inflammation and vasculopathy in the skin and internal organs. Recently, several articles described that Th17 cells, IL-23 and IL-17 levels were significantly elevated in the peripheral blood or fibrotic sites of SSc. We report a case of SSc and psoriasis administered ustekinumab, IL-12/IL-23 inhibitor. In this case, the skin tightening was successfully improved and ustekinumab was more effective, even though oral prednisolone (9-12 mg/day) had some effect on skin tightening and arthralgia...
April 19, 2017: Clinical and Experimental Rheumatology
https://www.readbyqxmd.com/read/28416296/hiv-infection-and-aortic-stiffness
#7
REVIEW
Luisa Helena Maia Leite, Ariel Cohen, Franck Boccara
People living with human immunodeficiency virus (HIV) infection and receiving antiretroviral therapy now have the same life expectancy as the general population. However, they have a higher risk of atherosclerotic cardiovascular events because of a complex and polyfactorial vasculopathy, combining the effects of antiretroviral therapy, the HIV virus itself, immune activation, chronic inflammation and metabolic disturbances. Whether people living with HIV infection experience increased vascular aging compared with the general population remains controversial...
April 14, 2017: Archives of Cardiovascular Diseases
https://www.readbyqxmd.com/read/28413189/heart-transplantation-in-asia
#8
Hae-Young Lee, Byung-Hee Oh
Heart transplantation (HTx) is the effective way to improve quality of life as well as survival in terminal heart failure (HF) patients. Since the first heart transplant in 1968 in Japan and in earnest in 1987 at Taiwan, HTx has been continuously increasing in Asia. Although the current percentage of heart transplants from Asia comprises only 5.7% of cases in the International Society of Heart and Lung Transplantation (ISHLT) registry, the values were under-reported and soon will be greatly increased. HTx in Asia shows comparable with or even better results compared with ISHLT registry data...
April 11, 2017: Circulation Journal: Official Journal of the Japanese Circulation Society
https://www.readbyqxmd.com/read/28413064/systemic-sclerosis
#9
REVIEW
Christopher P Denton, Dinesh Khanna
Systemic sclerosis, also called scleroderma, is an immune-mediated rheumatic disease that is characterised by fibrosis of the skin and internal organs and vasculopathy. Although systemic sclerosis is uncommon, it has a high morbidity and mortality. Improved understanding of systemic sclerosis has allowed better management of the disease, including improved classification and more systematic assessment and follow-up. Additionally, treatments for specific complications have emerged and a growing evidence base supports the use of immune suppression for the treatment of skin and lung fibrosis...
April 13, 2017: Lancet
https://www.readbyqxmd.com/read/28412596/is-lenticulostriated-vasculopathy-an-unfavorable-prognostic-finding-in-infants-with-congenital-cytomegalovirus-infection
#10
Antonietta Giannattasio, Pasquale Di Costanzo, Paola Milite, Daniela De Martino, Eleonora Capone, Antonia Romano, Carmela Bravaccio, Letizia Capasso, Francesco Raimondi
BACKGROUND: Lenticulostriated vasculopathy (LSV) detected in head ultrasound (HUS) has been related to neurological and hearing sequelae in infants with congenital cytomegalovirus (cCMV) infection. OBJECTIVE: To assess the role of LSV in predicting neurodevelopmental and hearing outcomes in infants with cCMV infection. STUDY DESIGN: We enrolled consecutive infants who were affected by cCMV infection and underwent HUS within the first month of life...
April 6, 2017: Journal of Clinical Virology: the Official Publication of the Pan American Society for Clinical Virology
https://www.readbyqxmd.com/read/28411233/vasculopathy-in-the-setting-of-cardiorenal-syndrome-the-roles-of-protein-bound-uremic-toxins
#11
Jingbin Guo, Lu Lu, Yue Hua, Kevin Huang, Ian Wang, Li Huang, Qiang Fu, Aihua Chen, Paul Chan, Huimin Fan, Zhong-Min Liu, Bing Hui Wang
Chronic kidney disease (CKD) often leads to and accelerates the progression of cardiovascular disease (CVD), whilst CVD also causes kidney dysfunction. This bidirectional interaction leads to the development of a complex syndrome known as cardiorenal syndrome (CRS). CRS not only involves both the heart and the kidney, but also the vascular system through a vast array of contributing factors. In addition to hemodynamic, neuro-hormonal, mechanical and biochemical factors, the non-dialyzable protein-bound uremic toxins (PBUTs) are also key contributing factors that have been demonstrated through in vitro, in vivo and clinical observations...
April 14, 2017: American Journal of Physiology. Heart and Circulatory Physiology
https://www.readbyqxmd.com/read/28408138/diabetic-retinopathy-is-a-neurodegenerative-disorder
#12
Stephanie K Lynch, Michael D Abràmoff
Since 1875, controversy has ensued over whether ocular diabetic complications are primarily vasculopathic or neuropathic in nature. Here, we discuss the historical context by which diabetic retinopathy (DR) came to be considered a primary vasculopathy, in contrast to more recent data suggesting the importance of diabetic retinal neurodegeneration (DRN) as the primary manifestation of ocular diabetic damage. Unsurprisingly, DRN parallels other diabetic complications related to neuropathy. In general, there are three possible relationships between microvascular DR and DRN: i) microvasculopathy causes neurodegeneration; ii) neurodegeneration causes microvasculopathy or iii) they are mutually independent...
April 10, 2017: Vision Research
https://www.readbyqxmd.com/read/28403691/novel-nlrc4-mutation-causes-a-syndrome-of-perinatal-autoinflammation-with-hemophagocytic-lymphohistiocytosis-hepatosplenomegaly-fetal-thrombotic-vasculopathy-and-congenital-anemia-and-ascites
#13
Jiancong Liang, Danielle N Alfano, James E Squires, Melissa M Riley, W Tony Parks, Julia Kofler, Areeg El-Gharbawy, Suneeta Madan-Kheterpal, Roxanne Acquaro, Jennifer Picarsic
Autoinflammatory diseases are caused by pathologic activation of the innate immune system. Primary hemophagocytic lymphohistiocytosis (HLH) is an aggressive syndrome of excessive immune activation caused by monogenic mutations resulting in cytotoxic cell defects and subsequent failure to eliminate activated macrophages. Secondary HLH is often diagnosed in cases without a known Mendelian inheritance. However, some cases of "secondary" HLH have been shown to harbor mutations with partial dysfunction of the cytotoxic system...
January 1, 2017: Pediatric and Developmental Pathology
https://www.readbyqxmd.com/read/28403037/characteristics-of-submacular-hemorrhages-in-age-related-macular-degeneration
#14
Jae Hui Kim, Young Suk Chang, Jong Woo Kim, Chul Gu Kim
PURPOSE: The aims of this research are to report the incidence and characteristics of submacular hemorrhage secondary to neovascular age-related macular degeneration (AMD) and to compare the detailed morphologic features of hemorrhages between typical neovascular AMD and polypoidal choroidal vasculopathy (PCV). METHODS: This retrospective observational study included 791 eyes of 791 patients who had newly diagnosed neovascular AMD at a single institution. The incidence and extent of submacular hemorrhage of one disc area or greater were estimated and compared between typical neovascular AMD and PCV...
May 2017: Optometry and Vision Science: Official Publication of the American Academy of Optometry
https://www.readbyqxmd.com/read/28397288/extensive-levamisole-induced-vasculitis
#15
C Desvignes, C Becquart, D Launay, L Terriou, P Patenotre, S Deheul, G Peytavin, N Dupin, E Delaporte, D Staumont-Sallé
Levamisole (an increasingly frequent contaminant of cocaine) can cause antineutrophil cytoplasmic antibody-associated vasculitis. Dermatologists should consider a diagnosis of cocaine/levamisole-associated cutaneous vasculopathy syndrome in cases of purpura of the ears and/or extensive retiform purpura in drug users. We report a case of particularly severe levamisole-induced necrotic purpura and immunological abnormalities in a 40-year-old woman.
April 10, 2017: Clinical and Experimental Dermatology
https://www.readbyqxmd.com/read/28397126/protein-tyrosine-phosphatases-in-systemic-sclerosis-potential-pathogenic-players-and-therapeutic-targets
#16
REVIEW
Cristiano Sacchetti, Nunzio Bottini
PURPOSE OF REVIEW: The pathogenesis of systemic sclerosis depends on a complex interplay between autoimmunity, vasculopathy, and fibrosis. Reversible phosphorylation on tyrosine residues, in response to growth factors and other stimuli, critically regulates each one of these three key pathogenic processes. Protein tyrosine kinases, the enzymes that catalyze addition of phosphate to tyrosine residues, are known players in systemic sclerosis, and tyrosine kinase inhibitors are undergoing clinical trials for treatment of this disease...
May 2017: Current Rheumatology Reports
https://www.readbyqxmd.com/read/28394814/percutaneous-mechanical-circulatory-support-using-impella%C3%A2-devices-for-decompensated-cardiogenic-shock-a-pediatric-heart-center-experience
#17
Dhaval Parekh, Aamir Jeewa, Sebastian C Tume, William J Dreyer, Ricardo Pignatelli, David Horne, Henri Justino, Athar M Qureshi
Cardiogenic shock remains a significant cause of mortality and morbidity in children with heart failure. Percutaneous mechanical circulatory support may be an additional tool to augment left heart support and decompression in addition to conventional therapies. This report aims to review the clinical and hemodynamic outcomes of the Impella® device at a pediatric center. A retrospective review of all implants between October 2014 and November 2016 was conducted. Clinical outcomes, device implant techniques, complications, and hemodynamic data were collected...
April 6, 2017: ASAIO Journal: a Peer-reviewed Journal of the American Society for Artificial Internal Organs
https://www.readbyqxmd.com/read/28393035/retinal-ganglion-cell-complex-changes-using-spectral-domain-optical-coherence-tomography-in-diabetic-patients-without-retinopathy
#18
Ahmed I Hegazy, Rasha H Zedan, Tamer A Macky, Soheir M Esmat
AIM: To assess the ganglion cell complex (GCC) thickness in diabetic eyes without retinopathy. METHODS: Two groups included 45 diabetic eyes without retinopathy and 21 non diabetic eyes. All subjects underwent full medical and ophthalmological history, full ophthalmological examination, measuring GCC thickness and central foveal thickness (CFT) using the RTVue(®) spectral domain-optical coherence tomography (SD-OCT), and HbA1C level. RESULTS: GCC focal loss volume (FLV%) was significantly more in diabetic eyes (22...
2017: International Journal of Ophthalmology
https://www.readbyqxmd.com/read/28393033/combined-photodynamic-therapy-and-ranibizumab-for-polypoidal-choroidal-vasculopathy-a-2-year-result-and-systematic-review
#19
Meng Zhao, Hai-Ying Zhou, Jun Xu, Feng Zhang, Wen-Bin Wei, Ning-Pu Liu
AIM: To report a cohort of patients with polypoidal choroidal vasculopathy (PCV) treated with photodynamic therapy (PDT) followed by intravitreal ranibizumab injection 24-48h later, and to compare the results between eyes with PCV treated by PDT followed by intravitreal anti-vascular endothelial growth factor (VEGF) injection and intravitreal anti-VEGF injection followed by PDT by Meta-analysis. METHODS: Retrospective study and systematic literature review. Medical records of patients with PCV who were initially treated using PDT followed by intravitreal ranibizumab injection 24-48h after PDT and had completed at least 2y follow-up were reviewed and analyzed...
2017: International Journal of Ophthalmology
https://www.readbyqxmd.com/read/28392041/arterial-stiffness-is-an-independent-predictor-for-albuminuria-progression-among-asians-with-type-2-diabetes-a-prospective-cohort-study
#20
Xiao Zhang, Serena Low, Chee Fang Sum, Subramaniam Tavintharan, Yeoh Lee Ying, Jianjun Liu, Na Li, Keven Anga, Simon Bm Lee, Wern Ee Tang, Su Chi Lim
AIM: Albuminuria progression has been associated with renal deterioration in type 2 diabetes (T2DM). Central arterial stiffness can aggravate systemic vasculopathy by propagating elevated systolic and pulse pressures forward, thereby accentuating global vascular injury. We aim to investigate whether central arterial stiffness is an independent predictor for albuminuria progression in a multi-ethnic T2DM Asian cohort in Singapore. METHODS: In a prospective cohort, 1012 T2DM patients were assessed at baseline and after a median follow-up of 3...
February 14, 2017: Journal of Diabetes and its Complications
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