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https://www.readbyqxmd.com/read/28332851/empathy-and-facial-expression-recognition-in-children-with-and-without-attention-deficit-hyperactivity-disorder-effects-of-stimulant-medication-on-empathic-skills-in-children-with-attention-deficit-hyperactivity-disorder
#1
Funda Gumustas, Ibrahim Yilmaz, Yasemin Yulaf, Sebla Gokce, Osman Sabuncuoglu
OBJECTIVE: The aim of this study was to compare children and adolescents with attention-deficit/hyperactivity disorder (ADHD) to healthy children and adolescents in terms of state and trait empathy and emotion expression recognition skills. The goal was also to determine whether there are changes in emotion recognition and empathy measures in children with ADHD after methylphenidate (MPH) treatment. METHODS: The research sample consisted of outpatient drug-naive children and adolescents between the age of 8 and 14 years (n = 65) with ADHD according to the Diagnostic and Statistical Manual of Mental Disorders, 4th ed...
March 23, 2017: Journal of Child and Adolescent Psychopharmacology
https://www.readbyqxmd.com/read/28319420/modelling-long-term-evolution-of-chitotriosidase-in-non-neuronopathic-gaucher-disease
#2
Cristina Drugan, Tudor C Drugan, Paula Grigorescu-Sido, Ioana Naşcu
Chitotriosidase, an enzyme secreted by activated macrophages, is widely used as a biomarker for therapeutic monitoring and patient follow-up in Gaucher disease (GD), a lysosomal disorder caused by an inherited deficiency of glucocerebrosidase. We analyzed the long-term evolution of chitotriosidase aiming to establish an accurate model that describes the influence of enzyme replacement therapy (ERT) and the impact of several covariates. A total of 55 patients with non-neuronopathic (type 1) GD were followed for almost 17 years (during a maximum of 7...
March 20, 2017: Scandinavian Journal of Clinical and Laboratory Investigation
https://www.readbyqxmd.com/read/28316567/distinct-functional-connectivities-predict-clinical-response-with-emotion-regulation-therapy
#3
REVIEW
David M Fresco, Amy K Roy, Samantha Adelsberg, Saren Seeley, Emmanuel García-Lesy, Conor Liston, Douglas S Mennin
Despite the success of available medical and psychosocial treatments, a sizable subgroup of individuals with commonly co-occurring disorders, generalized anxiety disorder (GAD) and major depressive disorder (MDD), fail to make sufficient treatment gains thereby prolonging their deficits in life functioning and satisfaction. Clinically, these patients often display temperamental features reflecting heightened sensitivity to underlying motivational systems related to threat/safety and reward/loss (e.g., somatic anxiety) as well as inordinate negative self-referential processing (e...
2017: Frontiers in Human Neuroscience
https://www.readbyqxmd.com/read/28300554/amelioration-of-serum-8-ohdg-level-by-enzyme-replacement-therapy-in-patients-with-fabry-cardiomyopathy
#4
Kuan-Hsuan Chen, Yueh-Ching Chou, Chen-Yuan Hsiao, Yueh Chien, Kang-Ling Wang, Ying-Hsiu Lai, Yuh-Lih Chang, Dau-Ming Niu, Wen-Chung Yu
OBJECTIVES: The level of 8-hydroxy-2-deoxyguanosise (8-OHdG) is a marker of oxidative stress. The objective of this study was to evaluate the effect of enzyme replacement therapy (ERT) on the level of 8-OHdG in patients with Fabry cardiomyopathy and the clinical evolution of Fabry cardiomyopathy. METHODS: We measured the serum levels of 8-OHdG in 20 healthy control and 22 patients with Fabry cardiomyopathy before and after ERT. RESULTS: The mean lysoGb3 and 8-OHdG levels was significantly increased in patients with Fabry cardiomyopathy compared with that of control subjects (lysoGb3, 3...
March 11, 2017: Biochemical and Biophysical Research Communications
https://www.readbyqxmd.com/read/28296917/enzyme-replacement-therapy-for-anderson-fabry-disease-a-complementary-overview-of-a-cochrane-publication-through-a-linear-regression-and-a-pooled-analysis-of-proportions-from-cohort-studies
#5
Regina El Dib, Huda Gomaa, Alberto Ortiz, Juan Politei, Anil Kapoor, Fellype Barreto
BACKGROUND: Anderson-Fabry disease (AFD) is an X-linked recessive inborn error of glycosphingolipid metabolism caused by a deficiency of alpha-galactosidase A. Renal failure, heart and cerebrovascular involvement reduce survival. A Cochrane review provided little evidence on the use of enzyme replacement therapy (ERT). We now complement this review through a linear regression and a pooled analysis of proportions from cohort studies. OBJECTIVES: To evaluate the efficacy and safety of ERT for AFD...
2017: PloS One
https://www.readbyqxmd.com/read/28287048/lycium-barbarum-polysaccharide-protects-human-keratinocytes-against-uvb-induced-photo-damage
#6
Huaping Li, Zhenjie Li, Liqian Peng, Na Jiang, Qing Liu, Erting Zhang, Bihua Liang, Runxiang Li, Huilan Zhu
Ultraviolet B (UVB) irradiation plays a key role in skin damage, which induces oxidative and inflammatory damages, thereby causing photoaging or photocarcinogenesis. Lycium barbarum polysaccharide (LBP), the most biologically active fraction of wolfberry, possesses significant antioxidative and anti-inflammatory effects on multiple tissues. In the present study, the photoprotective effects and potential underlying molecular mechanisms of LBP against UVB-induced photo-damage were investigated in immortalized human keratinocytes (HaCaT cells)...
February 2017: Free Radical Research
https://www.readbyqxmd.com/read/28282631/does-goal-relevant-episodic-future-thinking-amplify-the-effect-on-delay-discounting
#7
Sara O'Donnell, Tinuke Oluyomi Daniel, Leonard H Epstein
Delay discounting (DD) is the preference for smaller immediate rewards over larger delayed rewards. Research shows episodic future thinking (EFT), or mentally simulating future experiences, reframes the choice between small immediate and larger delayed rewards, and can reduce DD. Only general EFT has been studied, whereby people reframe decisions in terms of non-goal related future events. Since future thinking is often goal-oriented and leads to greater activation of brain regions involved in prospection, goal-oriented EFT may be associated with greater reductions in DD than general goal-unrelated EFT...
March 7, 2017: Consciousness and Cognition
https://www.readbyqxmd.com/read/28279069/brain-and-organ-uptake-in-the-rhesus-monkey-in-vivo-of-recombinant-iduronidase-compared-to-an-insulin-receptor-antibody-iduronidase-fusion-protein
#8
Ruben J Boado, William M Pardridge
Mucopolysaccharidosis type I (MPSI) is caused by mutations in the gene encoding the lysosomal enzyme, α-l-iduronidase (IDUA), and patients with MPSI are currently treated with IDUA enzyme replacement therapy (ERT). However, the majority of MPSI patients have severe CNS involvement, and conventional ERT does not treat the brain. The failure of ERT to treat the brain is believed to be due to the lack of IDUA transport through the blood-brain barrier (BBB). However, BBB transport of IDUA has not been directly measured, to date...
March 16, 2017: Molecular Pharmaceutics
https://www.readbyqxmd.com/read/28277716/from-anomalies-to-forecasts-toward-a-descriptive-model-of-decisions-under-risk-under-ambiguity-and-from-experience
#9
Ido Erev, Eyal Ert, Ori Plonsky, Doron Cohen, Oded Cohen
Experimental studies of choice behavior document distinct, and sometimes contradictory, deviations from maximization. For example, people tend to overweight rare events in 1-shot decisions under risk, and to exhibit the opposite bias when they rely on past experience. The common explanations of these results assume that the contradicting anomalies reflect situation-specific processes that involve the weighting of subjective values and the use of simple heuristics. The current article analyzes 14 choice anomalies that have been described by different models, including the Allais, St...
March 9, 2017: Psychological Review
https://www.readbyqxmd.com/read/28275553/enzyme-replacement-therapy-for-farber-disease-proof-of-concept-studies-in-cells-and-mice
#10
Xingxuan He, Shaalee Dworski, Changzhi Zhu, Victor DeAngelis, Alex Solyom, Jeffrey A Medin, Calogera M Simonaro, Edward H Schuchman
A series of studies were carried out in Farber disease (OMIM #228000) cells and mice to evaluate the feasibility of enzyme replacement therapy (ERT) for this disorder. Media from Chinese hamster ovary (CHO) cells overexpressing human recombinant acid ceramidase (rhAC) was used to treat fibroblasts from a Farber disease patient, leading to significantly reduced ceramide. We also found that chondrocytes from Farber disease mice had a markedly abnormal chondrogenic phenotype, and this was corrected by rhAC as well...
June 2017: BBA Clinical
https://www.readbyqxmd.com/read/28266734/cardiac-response-to-enzyme-replacement-therapy-in-infantile-pompe-disease-with-severe-hypertrophic-cardiomyopathy
#11
Sravani Avula, Thuylinh M Nguyen, Michael Marble, Christian Lilje
Classic infantile-onset Pompe disease (IOPD), characterized by predominantly cardiac involvement, used to be considered uniformly lethal within months. The availability of enzyme replacement therapy (ERT) has transformed the course of the disease. Decrease in ventricular hypertrophy and improvement in ventricular function have been suggested as proof for efficacy. We report the cardiac response to ERT of a child with IOPD and severe hypertrophic cardiomyopathy. The myocardial hypertrophy resolved. Change in ejection fraction, however, was slow...
March 7, 2017: Echocardiography
https://www.readbyqxmd.com/read/28263001/assessment-of-bone-health-in-patients-with-type-1-gaucher-disease-using-impact-microindentation
#12
Sabina Herrera, Jordi Pérez-López, Marc Moltó-Abad, Roberto Güerri-Fernández, Elena Cabezudo, Silvana Novelli, Jordi Esteve, Albert Hernández, Inmaculada Roig, Xavier Solanich, Daniel Prieto-Alhambra, Xavier Nogués, Adolfo Díez-Pérez
BACKGROUND: Gaucher disease (GD), one of the commonest lysosomal disorders (a global population incidence of 1:50,000), is characterized by beta-glucocerebrosidase deficiency. Some studies have demonstrated bone infiltration in up to 80% of patients, even if asymptomatic. Bone disorder remains the main cause of morbidity in these patients, along with osteoporosis, avascular necrosis, and bone infarcts. Enzyme replacement therapy (ERT) has been shown to improve these symptoms. METHODS: This cross-sectional study included patients with type 1 Gaucher disease (GD1) selected from the Catalan Study Group on GD...
March 6, 2017: Journal of Bone and Mineral Research: the Official Journal of the American Society for Bone and Mineral Research
https://www.readbyqxmd.com/read/28253518/disease-progression-modeling-to-evaluate-the-effects-of-enzyme-replacement-therapy-on-kidney-function-in-adult-patients-with-the-classic-phenotype-of-fabry-disease
#13
Albina Nowak, Gilbert Koch, Uyen Huynh-Do, Martin Siegenthaler, Hans-Peter Marti, Marc Pfister
BACKGROUND/AIMS: Fabry disease (FD) is a rare inherited lysosomal storage disease with common and serious kidney complications. Enzyme replacement therapies (ERT) with agalsidase-α and -β were investigated to characterize their therapeutic effect on kidney function in FD patients with Classic phenotype. METHODS: The prospective FD cohort consisted of 98 genetically confirmed patients (females, n = 61, males, n = 37). The median [interquartile range] follow-up time (time difference from first to last visit) was 9 [6, 12] years...
February 28, 2017: Kidney & Blood Pressure Research
https://www.readbyqxmd.com/read/28245874/timing-of-thoracic-radiotherapy-in-the-treatment-of-extensive-stage-small-cell-lung-cancer-important-or-not
#14
Jing Luo, Liming Xu, Lujun Zhao, Yuanjie Cao, Qingsong Pang, Jun Wang, Zhiyong Yuan, Ping Wang
BACKGROUND: This study evaluated the prognosis of patients with extensive-stage small-cell lung cancer (ES-SCLC) that may be associated with timing of thoracic radiotherapy (TRT). METHODS: ES-SCLC patients (n = 232) without progression were retrospectively analyzed after first-line induction chemotherapy. Patients in the TRT group were stratified as early-TRT (ERT; ≤3 cycles of induction chemotherapy received prior to TRT, n = 65) or late-TRT (LRT; >3 cycles, n = 122)...
February 28, 2017: Radiation Oncology
https://www.readbyqxmd.com/read/28220089/emotion-regulation-therapy-a-mechanism-targeted-treatment-for-disorders-of-distress
#15
Megan E Renna, Jean M Quintero, David M Fresco, Douglas S Mennin
"Distress disorders," which include generalized anxiety disorder and major depression are often highly comorbid with each other and appear to be characterized by common temperamental features that reflect heightened sensitivity to underlying motivational systems related to threat/safety and reward/loss. Further, individuals with distress disorders tend to utilize self-referential processes (e.g., worry, rumination, self-criticism) in a maladaptive attempt to respond to motivationally relevant distress, often resulting in suboptimal contextual learning...
2017: Frontiers in Psychology
https://www.readbyqxmd.com/read/28218669/a-review-of-gaucher-disease-pathophysiology-clinical-presentation-and-treatments
#16
REVIEW
Jérôme Stirnemann, Nadia Belmatoug, Fabrice Camou, Christine Serratrice, Roseline Froissart, Catherine Caillaud, Thierry Levade, Leonardo Astudillo, Jacques Serratrice, Anaïs Brassier, Christian Rose, Thierry Billette de Villemeur, Marc G Berger
Gaucher disease (GD, ORPHA355) is a rare, autosomal recessive genetic disorder. It is caused by a deficiency of the lysosomal enzyme, glucocerebrosidase, which leads to an accumulation of its substrate, glucosylceramide, in macrophages. In the general population, its incidence is approximately 1/40,000 to 1/60,000 births, rising to 1/800 in Ashkenazi Jews. The main cause of the cytopenia, splenomegaly, hepatomegaly, and bone lesions associated with the disease is considered to be the infiltration of the bone marrow, spleen, and liver by Gaucher cells...
February 17, 2017: International Journal of Molecular Sciences
https://www.readbyqxmd.com/read/28217933/cytology-of-the-neovagina-in-transgender-women-and-individuals-with-congenital-or-acquired-absence-of-a-natural-vagina
#17
A Grosse, C Grosse, D Lenggenhager, B Bode, U Camenisch, P Bode
OBJECTIVE: The primary objective of this study was to describe the cytological findings of bowel and (penile) skin-lined neovaginas in patients with gender dysphoria (GD) and individuals with a congenital or acquired absence of a natural vagina. The secondary objective was to correlate the cytological findings with clinical characteristics such as oestrogen replacement therapy (ERT). METHODS: A retrospective review of an institutional pathology archive over a 15-year-period was performed to identify cytological samples of neovaginal vaults...
February 20, 2017: Cytopathology: Official Journal of the British Society for Clinical Cytology
https://www.readbyqxmd.com/read/28216133/effects-of-copper-oxide-nanomaterials-cuonms-are-life-stage-dependent-full-life-cycle-in-enchytraeus-crypticus
#18
Rita C Bicho, Fátima C F Santos, Janeck J Scott-Fordsmand, Mónica J B Amorim
Copper oxide nanomaterials (CuONMs) have various applications in industry and enter the terrestrial environment, e.g. via sewage sludge. The effects of CuONMs and copper chloride (CuCl2) were studied comparing the standard enchytraeid reproduction test (ERT) and the full life cycle test (FLCt) with Enchytraeus crypticus. CuONMs mainly affected growth or juveniles' development, whereas CuCl2 mainly affected embryo development and/or hatching success and adults survival. Compared to the ERT, the FLCt allowed discrimination of effects between life stages and provided indication of the underlying mechanisms; further, the FLCt showed increased sensitivity, e...
February 16, 2017: Environmental Pollution
https://www.readbyqxmd.com/read/28215668/potential-of-surfactant-coated-nanoparticles-to-improve-brain-delivery-of-arylsulfatase-a
#19
Tilman Schuster, Astrid Mühlstein, Claudia Yaghootfam, Olga Maksimenko, Elena Shipulo, Svetlana Gelperina, Jörg Kreuter, Volkmar Gieselmann, Ulrich Matzner
The lysosomal storage disorder (LSD) metachromatic leukodystrophy (MLD) is caused by a deficiency of the soluble, lysosomal hydrolase arylsulfatase A (ASA). The disease is characterized by accumulation of 3-O-sulfogalactosylceramide (sulfatide), progressive demyelination of the nervous system and premature death. Enzyme replacement therapy (ERT), based on regular intravenous injections of recombinant functional enzyme, is in clinical use for several LSDs. For MLD and other LSDs with central nervous system (CNS) involvement, however, ERT is limited by the blood-brain barrier (BBB) restricting transport of therapeutic enzymes from the blood to the brain...
February 16, 2017: Journal of Controlled Release: Official Journal of the Controlled Release Society
https://www.readbyqxmd.com/read/28211716/direct-identification-of-dilute-surface-spins-on-al_-2-o_-3-origin-of-flux-noise-in-quantum-circuits
#20
S E de Graaf, A A Adamyan, T Lindström, D Erts, S E Kubatkin, A Ya Tzalenchuk, A V Danilov
An on-chip electron spin resonance technique is applied to reveal the nature and origin of surface spins on Al_{2}O_{3}. We measure a spin density of 2.2×10^{17}  spins/m^{2}, attributed to physisorbed atomic hydrogen and S=1/2 electron spin states on the surface. This is direct evidence for the nature of spins responsible for flux noise in quantum circuits, which has been an issue of interest for several decades. Our findings open up a new approach to the identification and controlled reduction of paramagnetic sources of noise and decoherence in superconducting quantum devices...
February 3, 2017: Physical Review Letters
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