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https://www.readbyqxmd.com/read/28230898/manifestations-and-medicolegal-significance-of-loeys-dietz-syndrome
#1
Roger W Byard
Loeys-Dietz syndrome is a recently described autosomal dominant disorder with underlying vasculopathy characterized by aortic and other vascular aneurysmal dissection/rupture. A 61-year-old man is reported who died suddenly and unexpectedly and at autopsy was found to have a ruptured abdominal aortic aneurysm. Additional findings included dolichostenomelia, high-arched palate, and pectus excavatum. There was a strong family history of Loeys-Dietz syndrome, although the decedent had never been tested. Death was, therefore, due to a ruptured abdominal aortic aneurysm in a case of probable Loeys-Dietz syndrome...
February 23, 2017: Journal of Forensic Sciences
https://www.readbyqxmd.com/read/28223776/idiopathic-polypoidal-choroidal-vasculopathy-in-thai-patients-with-clinical-and-angiographic-choroidal-neovascularization
#2
Chavakij Bhoomibunchoo, Yosanan Yospaiboon, Somanus Thoongsuwan, Duangnate Rojanaporn, Nawat Watanachai, Pichai Jirarattanasopa, Nattapon Wongcumchang, Atchara Amphornphruet, Sritatath Vongkulsiri, Eakkachai Arayangkoon
OBJECTIVE: This study aimed to study the prevalence and characteristics of idiopathic polypoidal choroidal vasculopathy (IPCV) in Thai patients with clinical and angiographic choroidal neovascularization (CNV). PATIENTS AND METHODS: A consecutive case study of 140 patients presenting with CNV was conducted in nine large referral eye centers throughout Thailand. The demographic data, fundus photographs, fundus fluorescein angiography and indocyanine green angiography of the patients were analyzed...
2017: Clinical Ophthalmology
https://www.readbyqxmd.com/read/28223767/fast-cicatrization-of-extensive-livedoid-vasculopathy-ulcers-under-treatment-with-sildenafil
#3
Lediane Moreira Lopes, Guilherme Gomes Dias Campos, Matheus Augusto Eisenreich, Aline Defaveri do Prado, Markus Bredemeier
No abstract text is available yet for this article.
February 2017: Annals of Dermatology
https://www.readbyqxmd.com/read/28220365/binding-of-11-c-pittsburgh-compound-b-correlated-with-white-matter-injury-in-hypertensive-small-vessel-disease
#4
Tetsuya Hashimoto, Chiaki Yokota, Kazuhiro Koshino, Takashi Temma, Makoto Yamazaki, Satoshi Iguchi, Ryo Shimomura, Toshiyuki Uehara, Naoko Funatsu, Tenyu Hino, Kazuo Minematsu, Hidehiro Iida, Kazunori Toyoda
OBJECTIVE: (11)C-Pittsburgh compound-B ((11)C-PIB) positron emission tomography (PET) is used to visualize and quantify amyloid deposition in the brain cortex in pathological conditions such as Alzheimer's disease (AD). Intense (11)C-PIB retention is also observed in the white matter (WM) of both healthy individuals and AD patients. However, the clinical implications of this retention in brain WM have not been clarified. We investigated the relationship between the extent of white matter lesions (WMLs) and the binding potential of (11)C-PIB (BPND) in the WM in patients with hypertensive small vessel disease...
February 20, 2017: Annals of Nuclear Medicine
https://www.readbyqxmd.com/read/28219580/use-of-anti-thymocyte-globulin-for-induction-therapy-in-cardiac-transplantation-a-review
#5
V Ruan, L S C Czer, M Awad, M Kittleson, J Patel, F Arabia, F Esmailian, D Ramzy, J Chung, M De Robertis, A Trento, J A Kobashigawa
The most common causes of death after heart transplantation (HTx) include acute rejection and multi-organ failure in the early period and malignancy and cardiac allograft vasculopathy (CAV) in the late period. Polyclonal antibody preparations such as rabbit anti-thymocyte globulin (ATG) may reduce early acute rejection and the later occurrence of CAV after HTx. ATG therapy depletes T cells, modulates adhesion and cell-signaling molecules, interferes with dendritic cell function, and induces B-cell apoptosis and regulatory and natural killer T-cell expansion...
March 2017: Transplantation Proceedings
https://www.readbyqxmd.com/read/28217310/the-impact-of-gender-mismatching-on-early-and-late-outcomes-following-heart-transplantation
#6
Yael Peled, Jacob Lavee, Michael Arad, Yedida Shemesh, Moshe Katz, Yigal Kassif, Elad Asher, Dan Elian, Yedael Har-Zahav, Ilan Goldenberg, Dov Freimark
AIMS: The role of donor/recipient gender matching on the long-term rejection process and clinical outcomes following heart transplantation (HT) outcomes is still controversial. We aim to investigate the impact of gender matching on early and long-term outcome HT. METHODS AND RESULTS: The study population comprised 166 patients who underwent HT between 1991 and 2013 and were prospectively followed up in a tertiary referral centre. Early and late outcomes were assessed by the type of donor-recipient gender match (primary analysis: female donor-male recipient [FD-MR, n = 36] vs...
February 2017: ESC Heart Failure
https://www.readbyqxmd.com/read/28216809/healing-ulcers-and-preventing-their-recurrences-in-the-diabetic-foot
#7
S Raja Sabapathy, Madhu Periasamy
Fifteen percent of people with diabetes develop an ulcer in the course of their lifetime. Eighty-five percent of the major amputations in diabetes mellitus are preceded by an ulcer. Management of ulcers and preventing their recurrence is important for the quality of life of the individual and reducing the cost of care of treatment. The main causative factors of ulceration are neuropathy, vasculopathy and limited joint mobility. Altered bio-mechanics due to the deformities secondary to neuropathy and limited joint mobility leads to focal points of increased pressure, which compromises circulation leading to ulcers...
September 2016: Indian Journal of Plastic Surgery: Official Publication of the Association of Plastic Surgeons of India
https://www.readbyqxmd.com/read/28212798/protein-kinase-c-as-regulator-of-vascular-smooth-muscle-function-and-potential-target-in-vascular-disorders
#8
H C Ringvold, R A Khalil
Vascular smooth muscle (VSM) plays an important role in maintaining vascular tone. In addition to Ca(2+)-dependent myosin light chain (MLC) phosphorylation, protein kinase C (PKC) is a major regulator of VSM function. PKC is a family of conventional Ca(2+)-dependent α, β, and γ, novel Ca(2+)-independent δ, ɛ, θ, and η, and atypical ξ, and ι/λ isoforms. Inactive PKC is mainly cytosolic, and upon activation it undergoes phosphorylation, maturation, and translocation to the surface membrane, the nucleus, endoplasmic reticulum, and other cell organelles; a process facilitated by scaffold proteins such as RACKs...
2017: Advances in Pharmacology
https://www.readbyqxmd.com/read/28212149/new-developments-for-the-detection-and-treatment-of-cardiac-vasculopathy
#9
Kevin J Clerkin, Ziad A Ali, Donna M Mancini
PURPOSE OF REVIEW: Cardiac allograft vasculopathy (CAV) is a major limitation to long-term survival after heart transplantation. Innovative new techniques to diagnose CAV have been applied to detect disease. This review will examine the current diagnostic and treatment options available to clinicians for CAV. RECENT FINDINGS: Diagnostic modalities addressing the pathophysiology underlying CAV (arterial wall thickening and decreased coronary blood flow) improve diagnostic sensitivity when compared to traditional (angiography and dobutamine stress echocardiography) techniques...
February 15, 2017: Current Opinion in Cardiology
https://www.readbyqxmd.com/read/28210186/bilateral-retinitis-pigmentosa-with-unilateral-choroidal-nevus-a-hitherto-unreported-association
#10
Rupak Roy, Kumar Saurabh, Debmalya Das, Preeti Sharma, Avirupa Ghose, Dhileesh P Chandrasekharan
Retinitis Pigmentosa (RP) has been reported to be associated with retinal hemangioma, retinal angiomatous proliferation, polypoidal choroidal vasculopathy and vasoproliferative tumours. However there is no previous report of choroidal nevus in an eye with RP. We describe such a case in which the eye with RP had a choroidal nevus at the macula. The coexistence of these two entities in the same eye emphasizes the need of careful examination of patients with RP. The presence of choroidal nevus warrants additional investigations such as B scan ultrasonography and a close follow-up to pick up changes in the nevus size and morphology...
July 2016: Saudi Journal of Ophthalmology: Official Journal of the Saudi Ophthalmological Society
https://www.readbyqxmd.com/read/28205373/pulmonary-hypertension-in-connective-tissue-diseases-an-update
#11
REVIEW
Ramya Aithala, Anoop G Alex, Debashish Danda
Pulmonary hypertension (PH) is a relatively commoner complication of systemic sclerosis (SSc) with estimated prevalence ranging between 8% and 12% as compared to much lower figures in other connective tissue diseases (CTD). It is a major cause of morbidity and mortality in CTDs. PH is classified into five major groups. CTD-associated PH belongs to group 1 PH, also known as pulmonary arterial hypertension (PAH). Around 30% of scleroderma-related deaths are due to PAH. Underlying pathogenesis is related to pulmonary vasculopathy involving small vessels...
February 16, 2017: International Journal of Rheumatic Diseases
https://www.readbyqxmd.com/read/28203138/lupus-gastrointestinal-tract-vasculopathy-lupus-enteritis-involving-the-entire-gastrointestinal-tract-from-esophagus-to-rectum
#12
Joseph Bert, Elie Gertner
Gastrointestinal symptoms are very common in systemic lupus erythematosus (SLE). Lupus "enteritis" is very responsive to treatment but can have devastating consequences if not detected. Most descriptions of enteritis involve the small and large bowel. This is the first report of lupus "enteritis" involving the entire gastrointestinal tract from the esophagus and stomach to the rectum. Lupus "enteritis" is another cause of upper gastrointestinal involvement in SLE (involving even the esophagus and stomach) in addition to involvement of the lower intestinal tract...
January 2017: Case Reports in Gastroenterology
https://www.readbyqxmd.com/read/28196060/angiographic-subtypes-of-polypoidal-choroidal-vasculopathy-in-taiwan-a-prospective-multicenter-study
#13
Ling Yeung, Chien-Neng Kuo, An-Ning Chao, Kuan-Jen Chen, Wei-Chi Wu, Chien-Hsiung Lai, Nan-Kai Wang, Yih-Shiou Hwang, Ching-Lung Chen, Chi-Chun Lai
PURPOSE: To determine the incidence and clinical characteristics of angiographic subtypes of polypoidal choroidal vasculopathy (PCV). METHODS: It is a prospective, multicenter, cross-sectional study. Patients with newly diagnosed exudative macular degeneration are classified into PCV, age-related macular degeneration (AMD), and retinal angiomatous proliferation. Polypoidal choroidal vasculopathy is further classified into two subtypes depending on the presence (Type 1: polypoidal choroidal neovascularization) or absence (Type 2: typical PCV) of feeder vessels on indocyanine green angiography...
February 10, 2017: Retina
https://www.readbyqxmd.com/read/28195615/quantitative-analysis-of-en-face-spectral-domain-optical-coherence-tomography-imaging-in-polypoidal-choroidal-vasculopathy
#14
Joseph M Simonett, Errol W Chan, Jonathan Chou, Dimitra Skondra, Daniel Colon, Caroline K Chee, Gopal Lingam, Amani A Fawzi
BACKGROUND AND OBJECTIVE: Spectral-domain optical coherence tomography (SD-OCT) imaging can be used to visualize polypoidal choroidal vasculopathy (PCV) lesions in the en face plane. Here, the authors describe a novel lesion quantification technique and compare PCV lesion area measurements and morphology before and after anti-vascular endothelial growth factor (VEGF) treatment. PATIENTS AND METHODS: Volumetric SD-OCT scans in eyes with PCV before and after induction anti-VEGF therapy were retrospectively analyzed...
February 1, 2017: Ophthalmic Surgery, Lasers & Imaging Retina
https://www.readbyqxmd.com/read/28195603/classification-of-exudative-age-related-macular-degeneration-with-pachyvessels-on-en-face-swept-source-optical-coherence-tomography
#15
Danny Siu-Chun Ng, Malini Bakthavatsalam, Frank Hiu-Ping Lai, Carol Yim-Lui Cheung, Gemmy Chu-Ming Cheung, Fang Yao Tang, Chi Wai Tsang, Timothy Yuk-Yau Lai, Tien Yin Wong, Mårten Erik Brelén
Purpose: The purpose of this study was to classify exudative maculopathy by the presence of pachyvessels on en face swept-source optical coherence tomography (SSOCT). Methods: Consecutive patients with signs of exudative maculopathy underwent SSOCT, fluorescein and indocyanine green angiography (ICGA), ultra-widefield fundus color photography, and autofluorescence examinations. Images were analyzed in a masked fashion by two sets of four examiners in different sessions: (1) the presence of pachyvessels in en face OCT and (2) features of exudative maculopathy in conventional imaging modalities...
February 1, 2017: Investigative Ophthalmology & Visual Science
https://www.readbyqxmd.com/read/28192815/lenticulostriate-vasculopathy-and-neurodevelopmental-outcomes-in-preterm-infants-a-systematic-review
#16
Abby Robinson, John Flibotte, Summer L Kaplan, Sara B DeMauro
Objective The objective of this study was to perform a systematic review of all studies that report neurodevelopmental outcomes at 12 months' corrected age or later for preterm infants (< 32 weeks) who are diagnosed with lenticulostriate vasculopathy (LSV) on cranial ultrasound. Study Design This is a systematic review. Results We identified 501 unique publications of which 3 met all prespecified eligibility criteria. Neurodevelopmental outcome data were available for 44 preterm infants with LSV. There were mixed results from the studies: the first reported impairments of mental development; the second reported impairments of motor development only; and finally, the third reported that three of three preterm infants with LSV had mild or moderate developmental delay...
February 13, 2017: American Journal of Perinatology
https://www.readbyqxmd.com/read/28192798/identification-of-angpt2-as-a-new-gene-for-neovascular-age-related-macular-degeneration-and-polypoidal-choroidal-vasculopathy-in-the-chinese-and-japanese-populations
#17
Li Ma, Marten E Brelen, Motokazu Tsujikawa, Haoyu Chen, Wai Kit Chu, Timothy Y Y Lai, Danny S C Ng, Kaori Sayanagi, Chicako Hara, Noriyasu Hashida, Vesta C K Chan, Pancy O S Tam, Alvin L Young, Weiqi Chen, Kohji Nishida, Chi Pui Pang, Li Jia Chen
Purpose: We determine the angiopoietin 2 (ANGPT2) gene as a new susceptibility gene for neovascular age-related macular degeneration (nAMD) and polypoidal choroidal vasculopathy (PCV). Methods: A total of 34 haplotype-tagging single-nucleotide polymorphisms (SNPs) were first genotyped in an exploratory Hong Kong Chinese cohort. Suggestive SNPs were replicated in a Shantou Chinese cohort and an Osaka Japanese cohort, with a total of 2343 subjects. The SNP rs800292 in the complement factor H (CFH) gene was genotyped in all the subjects...
February 1, 2017: Investigative Ophthalmology & Visual Science
https://www.readbyqxmd.com/read/28191323/autonomic-imbalance-captures-maternal-and-fetal-circulatory-response-to-pre-eclampsia
#18
Igor Lakhno
BACKGROUND: Pre-eclampsia (PE) is a gestational disease featured by hypertension, arterial systemic vasculopathy, multiple organ failure and fetal compromise. The aim of the investigation was to determine the role of maternal respiratory sinus arrhythmia (RSA) in regulation of the fetal circulatory system in case of healthy pregnancy and in PE. METHODS: The investigation of maternal and fetal HRV and fetal CTG variables in 106 patients at 34-40 weeks of gestation was performed...
2017: Clinical Hypertension
https://www.readbyqxmd.com/read/28191005/significance-of-anti-hla-antibodies-on-adult-and-pediatric-heart-allograft-outcomes
#19
REVIEW
Massimo Mangiola, Marilyn Marrari, Brian Feingold, Adriana Zeevi
As methods for human leukocyte antigens (HLA) antibody detection have evolved and newer solid phase assays are much more sensitive, the last 15 years has seen a renewed focus on the importance of HLA antibodies in solid organ transplant rejection. However, there is still much controversy regarding the clinical significance of antibody level as depicted by the mean fluorescence intensity of a patient's neat serum. Emerging techniques, including those that identify antibody level and function, show promise for the detection of individuals at risk of allograft rejection, determination of the effectiveness of desensitization prior to transplant, and for monitoring treatment of rejection...
2017: Frontiers in Immunology
https://www.readbyqxmd.com/read/28186689/enhanced-mrna-expression-of-plasminogen-activator-inhibitor-1-in-livedoid-vasculopathy-lesions
#20
Mehmet Agirbasli, Fatih Göktay, Irem Peker, Pembegul Gunes, Fugen Vardar Aker, Mustafa Akkiprik
AIM: Thrombosis and inflammation play an important role in pathophysiology of livedoid vasculopathy (LV). Plasminogen activator inhibitor-1 (PAI-1) is the main physiological inhibitor of fibrinolysis and is a pivotal modulator in a broad range of biological processes. METHOD: The study specimens were retrospectively selected from archives of pathology department. We investigated PAI-1 mRNA expression in the paraffin blocks of patients with biopsy proven LV and controls...
February 10, 2017: Cardiovascular Therapeutics
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