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Worth syndrome

Austen J J Worth, Charlotte J Houldcroft, Claire Booth
Epstein-Barr virus (EBV) infection is ubiquitous in humans, but the majority of infections have an asymptomatic or self-limiting clinical course. Rarely, individuals may develop a pathological EBV infection with a variety of life threatening complications (including haemophagocytosis and malignancy) and others develop asymptomatic chronic EBV viraemia. Although an impaired ability to control EBV infection has long been recognised as a hallmark of severe T-cell immunodeficiency, the advent of next generation sequencing has identified a series of Primary Immunodeficiencies in which EBV-related pathology is the dominant feature...
October 17, 2016: British Journal of Haematology
Asmaa I Owis, Amira M Abo-Youssef, Ahmed H Osman
Cordia boissieri A. DC. (Boraginaceae) is traditionally used as an herbal remedy for diabetes by Hispanic women in Southwestern USA. A recent investigation showed the significant protective effect of ethyl acetate extract against metabolic syndrome (MS). However, the corresponding active principles responsible for this effect and relations between their structure and biological actions remain unclear. Thus, ethyl acetate extract was subjected to column chromatography, which yielded seven compounds identified on the basis of spectroscopic data as rutin, hesperidin, kaempferol-3-O-β-d-glucopyranoside, rosmarinic acid, β-sitosterol-3-O-β-d-glucopyranoside, quercetin, and kaempferol...
October 4, 2016: Zeitschrift Für Naturforschung. C, A Journal of Biosciences
Torunn S Søyseth, May-Brit Lund, Øystein Bjørtuft, Aasta Heldal, Vidar Søyseth, Mary Amanda Dew, Gro Killi Haugstad, Ulrik Fredrik Malt
OBJECTIVE: We sought to investigate type and prevalence of psychiatric disorders and psychological distress in patients being evaluated for lung transplantation. METHODS: One hundred eighteen patients were assessed [74% with chronic obstructive pulmonary disease (COPD)] with the MINI Neuropsychiatric Interview, the General Health Questionnaire (GHQ), and Hospital Anxiety Depression Scale (HADS). Spirometry and the 6-min walk test (6MWT) assessed lung function with data subject to multivariate regression analyses...
September 2016: General Hospital Psychiatry
Jacek Bil, Robert J Gil
Bioresorbable vascular scaffolds (BVS) have emerged as an interesting alternative since the presence of the prosthesis in the coronary artery is transient. This technology enables to restore the normal vasomotor tone and allows positive remodeling, simultaneously reducing the trigger for persistent inflammation and facilitating further interventions by percutaneous or surgical means. Absorb BVS(®) is the first generation everolimus-eluting poly-L-lactide (PLLA) bioresorbable scaffold. In recent meta-analyses Absorb BVS(®) was definitely proved to be safe and effective device in the treatment of symptomatic coronary artery disease...
August 2016: Journal of Thoracic Disease
Mehmet Selcuki, Mesut Mete, Deniz Selcuki
AIM: The Chiari Malformation I (CM I) and tethered cord syndrome (TCS) are both congenital abnormalities whose mechanism are still not fully understood. Associaton of CM I and TCS have been reported only for a few times previously Material and Methods: This retrospective study includes 7 patients who were diagnosed with CM I and TCS managed by cutting of the filum terminale. RESULTS: The mean follow-up period was 21 months and 28 days. Although all patients were undergone untethering surgical procedure by cutting the filum terminale only, all patients reported significant early postsurgical resolution of CM I symptoms and symptoms related to TCS as well...
July 1, 2016: Turkish Neurosurgery
Shawn Haji-Momenian, Jonathan Rischall, Neil Okey, Myles Taffel, Nadia Khati, Robert Zeman
This study aimed to determine the incidence of non-traumatic acute aortic injury (AAI) extending from the chest into the abdomen or pelvis in emergency department (ED) patients with acute aortic syndrome (AAS), to estimate the effective dose of the abdominopelvic portion of these CT exams, and to compare the number needed to screen (NNS) with the collective population radiation dose of imaging those stations. All patients (n = 238) presenting to the ED with AAS between March 2014 and June 2015 who were imaged per CT AAI protocol (noncontrast and contrast-enhanced CT angiography of the chest, abdomen, and pelvis) were retrospectively identified in this IRB-approved HIPAA-compliant study...
August 27, 2016: Emergency Radiology
Renzo Bianchi, Jay Verkuilen, Romain Brisson, Irvin Sam Schonfeld, Eric Laurent
We investigated whether burnout and depression differed in terms of public stigma and help-seeking attitudes and behaviors. Secondarily, we examined the overlap of burnout and depressive symptoms. A total of 1046 French schoolteachers responded to an Internet survey in November-December 2015. The survey included measures of public stigma, help-seeking attitudes and behaviors, burnout and depressive symptoms, self-rated health, neuroticism, extraversion, history of anxiety or depressive disorder, social desirability, and socio-demographic variables...
November 30, 2016: Psychiatry Research
Gustavo Savino, Edoardo Abed, Maria Teresa Rebecchi, Maria Spreca, Costanza Tredici, Anna Dickmann
OBJECTIVE: To assess and compare sensory-motor status and clinical features of type I and type II acute acquired concomitant esotropia (group A) and decompensated monofixation syndrome (group B). METHODS: In a retrospective, comparative study, 26 patients with a confirmed postoperative diagnosis of type I and II acute acquired concomitant esotropia and monofixation syndrome were enrolled. The 2-tailed unpaired t test and 2-tailed χ(2) test were performed to compare angle of deviation and sensory-motor status under the environmental conditions and after prismatic adaptation test and progressive prism test of 2 groups...
August 2016: Canadian Journal of Ophthalmology. Journal Canadien D'ophtalmologie
Kai Zhang, Yawei Guo, Zhenzhen Ge, Zhihui Zhang, Yurong Da, Wen Li, Zimu Zhang, Zhenyi Xue, Yan Li, Yinghui Ren, Long Jia, Koon-Ho Chan, Fengrui Yang, Jun Yan, Zhi Yao, Aimin Xu, Rongxin Zhang
T helper 17 (Th17) cells are vital components of the adaptive immune system involved in the pathogenesis of most autoimmune and inflammatory syndromes, and adiponectin(ADN) is correlated with inflammatory diseases such as multiple sclerosis (MS) and type II diabetes. However, the regulatory effects of adiponectin on pathogenic Th17 cell and Th17-mediated autoimmune central nervous system (CNS) inflammation are not fully understood. In this study, we demonstrated that ADN could inhibit Th1 and Th17 but not Th2 cells differentiation in vitro...
August 11, 2016: Molecular Neurobiology
Camille Lefèvre, Nathalie Derache, Frédérique Grandhomme, Sabine Fradin, Stéphane Allouche
Despite the revisions of the Mac Donald criteria of multiple sclerosis (MS) in 2010, the cerebrospinal fluid (CSF) analysis by isoelectrofocusing (IEF) remains useful for atypical presentations of MS. The IEF is considered as positive when at least two or more additional bands are detected in the CSF by comparison with the patient's serum but sometimes, the IEF interpretation is more difficult. The goal of our study was to determine the significance when a single band in the CSF is detected by IEF. We conducted a retrospective study on 990 patients who underwent a lumbar puncture followed by a CSF analysis by IEF...
August 1, 2016: Annales de Biologie Clinique
Kouki Gondo, Amane Ike, Masahiro Ogawa, Kazuyuki Shirai, Makoto Sugihara, Hiroaki Nishikawa, Atsushi Iwata, Akira Kawamura, Ken Mori, Bo Zhang, Shin'ichiro Miura, Shin'ichiro Yasunaga, Keijiro Saku
BACKGROUND: Percutaneous coronary intervention (PCI) with a drug-eluting stent (DES) is increasingly being used for the treatment of coronary artery diseases. However, the role and effectiveness of PCI with a bare metal stent (BMS) have not yet been established. METHODS: Among the 2197 patients (2653 lesions) treated with PCI from 2003 to 2012 at three institutions in Fukuoka, 859 patients (1032 lesions) without acute coronary syndrome and in whom we were able to perform follow-up coronary angiography after stent placement and collect detailed data were selected for this study...
August 2, 2016: Journal of Cardiology
Pia Svedberg, Lennart Hallsten, Jurgita Narusyte, Lennart Bodin, Victoria Blom
In the self-worth model, burnout is considered to be a syndrome of performance-based self-esteem (PBSE) and experiences of exhaustion. Studies have shown that PBSE and burnout indices such as Pines' Burnout Measure (BM) are associated. Whether these variables have overlapping etiologies has however not been studied before. Genetic and environmental components of covariation between PBSE and exhaustion measured with Pines' BM were examined in a bivariate Cholesky model using data from 14,875 monozygotic and dizygotic Swedish twins...
October 2016: Scandinavian Journal of Psychology
Bernhard J Steinhoff, Matthias Bacher, Christoph Kurth, Anke M Staack, Reinhold Kornmeier
Perampanel (PER) is the first-in-class selective, noncompetitive α-amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid (AMPA) receptor antagonist that has been licensed and marketed as antiepileptic drug (AED) indicated for patients with partial-onset and primary generalized tonic-clonic seizures. A positive effect was reported in some patients with epileptic myoclonic jerks in idiopathic generalized epilepsy and in progressive myoclonic epilepsy. We treated a male patient with posthypoxic nonepileptic myoclonus (Lance-Adams syndrome) with add-on PER and achieved an almost complete cessation of jerks...
2016: Epilepsy & Behavior Case Reports
Marisa A P Baptista, Marton Keszei, Mariana Oliveira, Karen K S Sunahara, John Andersson, Carin I M Dahlberg, Austen J Worth, Agne Liedén, I-Chun Kuo, Robert P A Wallin, Scott B Snapper, Liv Eidsmo, Annika Scheynius, Mikael C I Karlsson, Gerben Bouma, Siobhan O Burns, Mattias N E Forsell, Adrian J Thrasher, Susanne Nylén, Lisa S Westerberg
Wiskott-Aldrich syndrome (WAS) is caused by loss-of-function mutations in the WASp gene. Decreased cellular responses in WASp-deficient cells have been interpreted to mean that WASp directly regulates these responses in WASp-sufficient cells. Here, we identify an exception to this concept and show that WASp-deficient dendritic cells have increased activation of Rac2 that support cross-presentation to CD8(+) T cells. Using two different skin pathology models, WASp-deficient mice show an accumulation of dendritic cells in the skin and increased expansion of IFNγ-producing CD8(+) T cells in the draining lymph node and spleen...
2016: Nature Communications
Mio Kitano, Gail W Davidson, Lawrence A Shirley, Carl R Schmidt, Gregory E Guy, Hooman Khabiri, Joshua D Dowell, Manisha H Shah, Mark Bloomston
BACKGROUND: Neuroendocrine tumors (NETs) have a propensity to metastasize to the liver, often resulting in massive tumor burden and hepatic dysfunction. While transarterial chemoembolization (TACE) is effective in treating patients with NET metastatic to the liver, there are limited data on its utility and benefit in patients with large hepatic involvement. The aim of our study was to determine the clinical benefit and complication rate of TACE in patients with massive hepatic tumor burden...
July 8, 2016: Annals of Surgical Oncology
Rohit Sharma, Chiyyarath Gopalan Muralidharan, Indranil Deb Roy, Lalit Janjani
Acquired Brown's syndrome is a rare entity. Delay in treatment can cause fibrosis or scarring with questionable prognosis of vertical diplopia. To the best of the knowledge of the authors the present case of 22-year-old male is the first in existing literature where delayed release of superior oblique muscle entrapment in orbital roof fracture was found to be an effective technique.
July 2016: Journal of Craniofacial Surgery
Gheath Alatrash, Peter F Thall, Benigno C Valdez, Patricia S Fox, Jing Ning, Haven R Garber, Selma Janbey, Laura L Worth, Uday Popat, Chitra Hosing, Amin M Alousi, Partow Kebriaei, Elizabeth J Shpall, Roy B Jones, Marcos de Lima, Gabriela Rondon, Julianne Chen, Richard E Champlin, Borje S Andersson
Pretransplant conditioning regimens critically determine outcomes in the setting of allogeneic stem cell transplantation (allo-SCT). The use of nucleoside analogs such as fludarabine (Flu) in combination with i.v. busulfan (Bu) has been shown to be highly effective as a pretransplant conditioning regimen in acute myeloid leukemia (AML), chronic myeloid leukemia (CML), and myelodysplastic syndrome (MDS). Because leukemia relapse remains the leading cause of death after allo-SCT, we studied whether clofarabine (Clo), a nucleoside analog with potent antileukemia activity, can be used to complement Flu...
October 2016: Biology of Blood and Marrow Transplantation
Ozgur Haki Yuksel, Ahmet Urkmez, Ayhan Verit
Types of prostatitis can be defined as groups of syndromes in adult men associated with infectious and noninfectious causes characterized frequently by lower abdominal and perineal signs and diverse clinical symptoms and complications. Etiopathogenesis of chronic prostatitis is not well defined. Moreover, its treatment outcomes are not satisfactory. Presence of c-kit positive interstitial cells in human prostate is already known. It has been demonstrated that these cells can be pacemaker cells which trigger spontaneous slow-wave electrical activity in the prostate and can be responsible for the transport of glandular secretion from acinar cells into major and minor prostatic ducts and finally into urethra...
2016: Archivio Italiano di Urologia, Andrologia
Masato Yoshihara, Michinori Mayama, Mayu Ukai, Sho Tano, Yasuyuki Kishigami, Hidenori Oguchi
Hepatic infarction is an extremely rare and fatal complication associated with hemolysis, elevated liver enzymes, and low platelets (HELLP) syndrome. It can develop into fulminant liver failure, which increases both maternal and neonatal mortality rates. A 34-year-old woman with no remarkable past medical history developed eclampsia after delivery at 40 weeks of gestation. Imaging indicated massive hepatic infarction and rupture followed by cardiac arrest and fulminant liver failure. Despite liver replacement therapy with plasma exchange and continuous hemodiafiltration, the patient gradually deteriorated with persistent bacterial infection until death at 98 days after delivery...
June 29, 2016: Journal of Obstetrics and Gynaecology Research
Chenjie Xia, Bradford C Dickerson
We have arrived at an exciting juncture in dementia research: the second major pathological hallmark of Alzheimer's disease (AD)-tau-can now be seen for the first time in the living human brain. The major proteinopathies in AD include amyloid-β plaques and neurofibrillary tangles (NFTs) made of hyperphosphorylated paired helical filament (PHF) tau. Since its advent more than a decade ago, amyloid PET imaging has revolutionized the field of dementia research, enabling more confident diagnosis of the likely pathology in patients with a variety of clinical dementia syndromes, paving the way for the identification of people with preclinical or prodromal AD pathology, and serving as a minimally invasive molecular readout in clinical trials of putative disease-modifying interventions...
September 2016: International Psychogeriatrics
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