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Talisa E de Carlo, Gregg T Kokame, Kyle N Kaneko, Rebecca Lian, James C Lai, Raymond Wee
PURPOSE: Determine sensitivity and specificity of polypoidal choroidal vasculopathy (PCV) diagnosis with structural en face optical coherence tomography (OCT) and OCT angiography (OCTA). METHODS: Retrospective review of the medical records of eyes diagnosed with PCV by indocyanine green angiography with review of diagnostic testing with structural en face OCT and OCTA by a trained reader. Structural en face OCT, cross-sectional OCT angiograms alone, and OCTA in its entirety were reviewed blinded to the findings of indocyanine green angiography and each other to determine if they could demonstrate the PCV complex...
March 20, 2018: Retina
Marzia Dolcino, Andrea Pelosi, Piera Filomena Fiore, Giuseppe Patuzzo, Elisa Tinazzi, Claudio Lunardi, Antonio Puccetti
Systemic sclerosis (SSc) is a rare connective tissue disease characterized by three pathogenetic hallmarks: vasculopathy, dysregulation of the immune system, and fibrosis. A particular feature of SSc is the increased frequency of some types of malignancies, namely breast, lung, and hematological malignancies. Moreover, SSc may also be a paraneoplastic disease, again indicating a strong link between cancer and scleroderma. The reason of this association is still unknown; therefore, we aimed at investigating whether particular genetic or epigenetic factors may play a role in promoting cancer development in patients with SSc and whether some features are shared by the two conditions...
2018: Frontiers in Immunology
Justine R Smith, Larry L David, Binoy Appukuttan, Phillip A Wilmarth
Diseases that involve retinal or choroidal vascular endothelial cells are leading causes of vision loss: age-related macular degeneration, retinal ischemic vasculopathies and non-infectious posterior uveitis. Proteins differentially expressed by these endothelial cell populations are potential drug targets. We used deep proteomic profiling to define the molecular phenotype of human retinal and choroidal endothelial cells at the protein level. MethodsRetinal and choroidal vascular endothelial cells were separately isolated from five human eye pairs by selection on CD31...
March 17, 2018: American Journal of Ophthalmology
Sven Haller, Meike W Vernooij, Joost P A Kuijer, Elna-Marie Larsson, Hans Rolf Jäger, Frederik Barkhof
Cerebral microbleeds (CMBs), also referred to as microhemorrhages, appear on magnetic resonance (MR) images as hypointense foci notably at T2*-weighted or susceptibility-weighted (SW) imaging. CMBs are detected with increasing frequency because of the more widespread use of high magnetic field strength and of newer dedicated MR imaging techniques such as three-dimensional gradient-echo T2*-weighted and SW imaging. The imaging appearance of CMBs is mainly because of changes in local magnetic susceptibility and reflects the pathologic iron accumulation, most often in perivascular macrophages, because of vasculopathy...
April 2018: Radiology
Heidi J Reich, Jon A Kobashigawa, Tamar Aintablian, Danny Ramzy, Michelle M Kittleson, Fardad Esmailian
Using older donor hearts in cardiac transplantation may lead to inferior outcomes: older donors have more comorbidities that reduce graft quality, including coronary artery disease, hypertension, diabetes mellitus, and dyslipidemia. Shorter cold ischemic times might overcome the detrimental effect of older donor age. We examined the relationship between donor allograft age and cold ischemic time on the long-term outcomes of heart transplant recipients. rom 1994 through 2010, surgeons at our hospital performed 745 heart transplantations...
February 2018: Texas Heart Institute Journal
David E Mandelbaum, Amanda Arsenault, Barbara S Stonestreet, Stefan Kostadinov, Suzanne M de la Monte
A pregnant woman with new-onset type 1 diabetes and ketoacidosis delivered an infant at 28 weeks of gestation who died with multiple organ failure and severe cerebral vasculopathy with extensive hemorrhage, diffuse microgliosis, and edema. This illustrates that antenatal metabolic and inflammatory stressors may be associated with neonatal encephalopathy and cerebral hemorrhage.
March 16, 2018: Journal of Pediatrics
Celeste Sassi, Michael A Nalls, Perry G Ridge, Jesse R Gibbs, Michelle K Lupton, Claire Troakes, Katie Lunnon, Safa Al-Sarraj, Kristelle S Brown, Christopher Medway, Jenny Lord, James Turton, Jose Bras, Sonja Blumenau, Mareike Thielke, Christa Josties, Dorette Freyer, Annette Dietrich, Monia Hammer, Michael Baier, Ulrich Dirnagl, Kevin Morgan, John F Powell, John S Kauwe, Carlos Cruchaga, Alison M Goate, Andrew B Singleton, Rita Guerreiro, Angela Hodges, John Hardy
Mendelian adult-onset leukodystrophies are a spectrum of rare inherited progressive neurodegenerative disorders affecting the white matter of the central nervous system. Among these, cerebral autosomal dominant and recessive arteriopathy with subcortical infarcts and leukoencephalopathy, cerebroretinal vasculopathy, metachromatic leukodystrophy, hereditary diffuse leukoencephalopathy with spheroids, and vanishing white matter disease present with rapidly progressive dementia as dominant feature and are caused by mutations in NOTCH3, HTRA1, TREX1, ARSA, CSF1R, EIF2B1, EIF2B2, EIF2B3, EIF2B4, and EIF2B5, respectively...
February 2, 2018: Neurobiology of Aging
Carina Mihai, Vanessa Smith, Rucsandra Dobrota, Ana Maria Gheorghiu, Maurizio Cutolo, Oliver Distler
Systemic sclerosis (SSc) is a connective tissue disease with high morbidity and mortality, characterized by autoimmunity, obliterative vasculopathy involving mainly the microvasculature, and fibrosis. SSc-specific nailfold capillaroscopic changes have been defined, and nailfold capillaroscopy (NFC) is now unequivocally accepted to be a cornerstone for the early diagnosis of SSc. However, the use of NFC in patients already diagnosed with SSc is still not standardized. Several studies have shown that NFC abnormalities correlate with disease activity and severity and are predictive for disease worsening, such as occurrence of new digital ulcers...
March 12, 2018: Microvascular Research
Beth M Kline-Fath, Arnold C Merrow, Maria A Calvo-Garcia, Usha D Nagaraj, Howard M Saal
Fetal ventriculomegaly is a common referral for prenatal MRI, with possible etiologies being hydrocephalus and hydranencephaly. The underlying cause of hydranencephaly is unknown, but many have suggested that the characteristic supratentorial injury is related to idiopathic bilateral occlusions of the internal carotid arteries from an acquired or destructive event. Fowler syndrome is a rare genetic disorder that causes fetal akinesia and a proliferative vasculopathy that can result in an apparent hydranencephaly-hydrocephaly complex...
March 14, 2018: Pediatric Radiology
Heather Reagin, Etan Marks, Stephen Weis, Joseph Susa
We report a case in which a 43-year-old African American male with medical history of sickle cell disease (SCD) presented with a nonhealing ulcer. Biopsy revealed features of livedoid vasculopathy. Previously, livedoid vasculopathy had only been described in a patient with sickle cell trait, but never in a patient with SCD. Livedoid vasculopathy most commonly affects the distal lower extremities and is characterized by irregular, punched-out, painful ulcers that heal with stellate white scars of atrophie blanche...
February 28, 2018: American Journal of Dermatopathology
Neslihan Arzu Akgün, Orçun Çiftci, Kerem Can Yılmaz, Emir Karaçağlar, Alp Aydınalp, Atilla Sezgin, I Haldun Müderrisoğlu, Mehmet Haberal
OBJECTIVES: Coronary vasospasm in heart transplant recipients occurs through various mechanisms. It has been linked to allograft rejection and coronary vasculopathy, which can result in mortality during follow-up. Here, we investigated the prevalence of coronary vasospasm among heart transplant recipients undergoing surveillance coronary angiography procedures. MATERIALS AND METHODS: This study was prospectively performed at Başkent University Faculty of Medicine by retrospectively analyzing medical information of patients who underwent bicaval heart transplant between 2003 and 2016 and subsequently had coronary angiography to rule out allograft vasculopathy...
March 2018: Experimental and Clinical Transplantation
B Handan Özdemir, F Nurhan Özdemir, Pelin Börcek, Çiğdem Sercan, Gökçe Özdemir, Ebru H Ayvazoğlu Soy, Mehmet Haberal
OBJECTIVES: Transplant vasculopathy is a significant predictor of poor outcome. We investigated whether age or pretransplant renal arterial vasculopathy of grafted kidneys affected allograft survival. MATERIALS AND METHODS: This study included 148 recipients and their donors. All donors underwent pretransplant renal arterial biopsy, with renal artery vascular score determined for each artery. Chronic rejection and graft loss were noted for all patients. RESULTS: Variable grades of pretransplant renal arterial lesions were noted in 103 donors (69...
March 2018: Experimental and Clinical Transplantation
Pradeep Venkatesh, Brijesh Takkar, Shreyas Temkar
Current imaging advancements have led to emergence of pachychoroid as an association of important vision threatening diseases like chronic serous chorioretinopathy and polypoidal choroidal vasculopathy. While the precise relation between thick choroid and such disorder is being investigated, the etiology behind pachychoroid remains elusive. We hypothesize pachychoroid to be a resultant of impeded vascular outflow due to thick sclera and increased scleral rigidity. We discuss our hypothesis in the perspective of other choroidal manifestations of anomalously thick scleral structure...
April 2018: Medical Hypotheses
Alexander Pschibul, Wibke G Janzarik, Peter Franck, Markus Hufnagel, Christopher Beck, Rudolf Korinthenberg
Despite the availability of modern antibiotics, pneumococcal meningitis in both children and adults remains a severe disease-one known to frequently cause grave complications and residual disability. Although the appearance of arterial vasospasms in bacterial meningitis systematically has been investigated and reported on for adult patients, such research is lacking when it comes to infants. We report on a 4-week-old infant who, 6 days after onset of pneumococcal meningitis, suffered severe neurological deterioration with treatment-resistant seizures and coma...
March 9, 2018: Neuropediatrics
Francesco Saverio Sorrentino, Silvia Matteini, Claudio Bonifazzi, Adolfo Sebastiani, Francesco Parmeggiani
In the face of the global epidemic of diabetes, it is critical that we update our knowledge about the pathogenesis of diabetes and the related micro alterations on the vascular network in the body. This may ultimately lead to early diagnosis and novel treatment options for delaying the progression of diabetic complications. Research has recently revealed the pivotal role of endothelin in the pathogenesis of diabetic complications, particularly in the regulation of the capillary flow, which is affected in the course of retinopathy...
March 9, 2018: Eye
Nobuo Yamashiro, Takamura Nagasaka, Naoki Ooishi, Mai Tsuchiya, Ryusuke Takaki, Fumikazu Kobayashi, Kazumasa Shindo, Yoshihisa Takiyama
We report here the clinical presentation and subsequent autopsy of a 90-year-old man who developed small papules with pain and swelling in his right ear. On admission, he exhibited right facial nerve paralysis, neck stiffness and Kernig's sign. The cell count was elevated and the varicella-zoster virus-PCR was positive in the CSF. Brain magnetic resonance imaging showed hyperintense lesions in the left pons and left temporal lobe, in FLAIR images. We diagnosed the patient with Ramsay Hunt syndrome and meningoencephalitis due to varicella-zoster virus...
March 2018: Brain and Nerve, Shinkei Kenkyū No Shinpo
Alfia Khaibullina, Elena A Adjei, Nowah Afangbedji, Andrey Ivanov, Namita Kumari, Luis E F Almeida, Zenaide M N Quezado, Sergei Nekhai, Marina Jerebtsova
Sickle cell disease patients are at increased risk of developing a chronic kidney disease. Endothelial dysfunction and inflammation associated with hemolysis lead to vasculopathy and contribute to the development of renal disease. Here we used a Townes sickle cell disease mouse model to examine renal endothelial injury. Renal disease in Townes mice was associated with glomerular hypertrophy, capillary dilation and congestion, and significant endothelial injury. We also detected substantial renal macrophage infiltration, and accumulation of macrophage stimulating protein 1 in glomerular capillary...
March 8, 2018: Haematologica
Z X Yu, L Q Zhong, H M Song, C Y Wang, W Wang, J Li, M S Ma
Objective: To summarize the clinical characteristics and treatment efficacy of the first reported case of a Chinese boy with stimulator of interferon genes (STING) associated vasculopathy with onset in infancy (SAVI). Methods: Sanger sequencing of the gene TMEM173 was performed based on systemic evaluation and clinical analysis of a highly suspected SAVI child admitted to Peking Union Medical College Hospital. A literature search (search terms included 'STING''SAVI''autoinflammatory diseases' and 'interferonopathy') was conducted using Chinese literature database, EMBASE and PubMed to include recently published SAVI studies (searched from January 2010 to December 2017)...
March 2, 2018: Zhonghua Er Ke za Zhi. Chinese Journal of Pediatrics
Jiang Hua, Shen Gang, Jiang Yizhou, Zhang Jing
Purpose: To evaluate the effectiveness and complications of intra-arterial chemotherapy (IAC) for treating advanced refractory retinoblastoma (RB) in a large single-center cohort. Patients and Methods: Eighty-four eyes of 62 consecutive patients with advanced refractory RB who received IAC were included in the study during January 2013 and April 2015. These patients failed to respond adequately to a standard systemic chemotherapy (i.e., carboplatin, vincristine, and etoposide) with or without local therapy...
January 2018: Journal of Cancer Research and Therapeutics
Pan Hu, Zhi Mao, Chao Liu, Xin Hu, Hongjun Kang, Feihu Zhou
Malignant atrophic papulosis (MAP) is a rare type of obliterating vasculopathy that can present as pure cutaneous lesions or a systemic entity affecting multiple organs. Systemic disease, such as gastrointestinal or central nervous system involvement, may predispose the patients to poorer or even fatal outcomes. We present a 30-year-old female patient with systemic manifestation of MAP 10 days after delivery of a full-term pregnancy who subsequently developed motor aphasia and intestinal perforation. The patient was administrated empirical treatment with an antiplatelet, anticoagulant, methylprednisolone sodium succinate and alprostadil...
March 8, 2018: Journal of Dermatology
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