Purpose
The purpose of this study was to compare clinical outcomes and surgical results after encephaloduroarteriosynangiosis (EDAS) in pediatric patients with Moyamoya disease that manifested as ...either ischemia or epileptic seizures.
Methods
We treated 23 children who underwent EDAS; we divided the patients into either ischemic or epileptic groups according to the individuals’ clinical presentation. Group Ia included those who mainly presented with cerebral ischemia in the form of preoperative transient ischemic attacks (TIA), while Group Ib presented with ischemia in the form of irreversible neurologic deficits or proven cerebral infarcts. Group II included those who presented with epileptic seizures rather than cerebral ischemia. We compared the clinical outcomes and surgical results following EDAS in the three groups.
Results
We grouped the patients into three groups according to their main preoperative clinical symptoms (Group Ia
n
= 10, Group Ib
n
= 6, and Group II
n
= 7). Group II, the epileptic manifestation group, tended to show more favorable clinical outcomes compared to the ischemic manifestation group, especially the severe ischemic group. However, there were no significant differences in postoperative neuroimaging and hemodynamic assessments between the groups.
Conclusions
EDAS is a safe and effective surgical technique that prevents epileptic seizures and shows more favorable clinical outcomes when used in patients with Moyamoya disease presenting with epileptic seizures compared to cerebral ischemia.
Abstract
Background
In a substantial proportion of patients with syncope, the exact cause is not identified because of the difficulty to document electrocardiograms correlated with the events during ...a syncope episode. Thus, an implantable loop recorder (ILR) was introduced for diagnosing hidden arrhythmia; however, its clinical use remains limited. Therefore, we conducted a retrospective analysis to assess the diagnostic value of the ILR in patients with unexplained syncope or palpitations.
Methods
All patients who had undergone ILR implantation from May 2016 to January 2020, were studied retrospectively. We analyzed their electrocardiogram stored in the device.
Results
Among the 70 patients (mean age ± SD; 50.2 ± 20.3 years, 27 men) with unknown causes of syncope or palpitation, during two years follow-up, arrhythmia was detected in 26 patients (37.1%). Nineteen (73.1%) patients underwent permanent pacemaker implantation due to symptomatic bradycardia or atrioventricular block. All arrhythmias were detected within 6 days to 39 months after loop recorder implantation. Thirteen patients (50%) showed sick sinus syndrome (eight long pauses and five tachycardia-bradycardia syndromes). Eleven patients (42.3%) had paroxysmal atrioventricular block. Two patients who underwent permanent pacemaker implantation showed a positive tilt-table test. Three patients underwent radiofrequency catheter ablation for paroxysmal supraventricular tachycardia and atrial fibrillation. The mean duration for the detection of first sign (arrhythmia or palpitations) was 7.5 months, and the time from the detection of arrhythmia to ablation or device implantation was 3.4 months.
Conclusion
ILR monitoring detected a substantial number of significant bradycardias in patients with unexplained syncope and palpitations, suggesting that it is an effective diagnostic method that can shorten the time required to identify the cause of arrhythmias.
Human apurinic/apyrimidinic endonuclease 1 (APE1) functions mainly in DNA repair as an enzyme removing AP sites and in redox signaling as a coactivator of various transcription factors. Based on ...these multifunctions of APE1 within cells, numerous studies have reported that the alteration of APE1 could be a crucial factor in development of human diseases such as cancer and neurodegeneration. In fact, the study on the combination of an individual’s genetic make-up with environmental factors (gene-environment interaction) is of great importance to understand the development of diseases, especially lethal diseases including cancer. Recent reports have suggested that the human carcinogenic risk following exposure to environmental toxicants is affected by APE1 alterations in terms of gene-environment interactions. In this review, we initially outline the critical APE1 functions in the various intracellular mechanisms including DNA repair and redox regulation and its roles in human diseases. Several findings demonstrate that the change in expression and activity as well as genetic variability of APE1 caused by environmental chemical (e.g., heavy metals and cigarette smoke) and physical carcinogens (ultraviolet and ionizing radiation) is likely associated with various cancers. These enable us to ultimately suggest APE1 as a vital marker for the prediction of environmental carcinogenesis risk.
Electrochemical and spectroscopic properties of Tb(III) in molten LiCl–KCl eutectic at high temperature were investigated by cyclic voltammetry and time-resolved laser-induced fluorescence ...spectroscopy (TRLFS). The diffusion coefficient of Tb(III) and the formal standard potential of Tb(III)/Tb
0 were determined to be 2.06
±
0.4
×
10
−
5
cm
2 s
−
1
and −
2.83
±
0.03
V vs. Cl
2/Cl
− at 887
K, respectively. Additionally, visible fluorescence of Tb(III) due to the electronic transitions from
5D
3 and
5D
4 to
7F
J was observed and measured by TRLFS for the first time. These results provide the first fluorescence spectroscopic evidence for a direct
in situ quantification of Tb(III) in the high temperature molten salt system.
In this study, it has been investigated the disinfection of Korean cultural artifacts by gamma irradiation, simulating the absorbed dose distribution on the object with the Monte Carlo methodology. ...Fungal contamination was identified on two traditional Korean agricultural tools, Hongdukkae and Holtae, which had been stored in a museum. Nine primary species were identified from these items: Bjerkandera adusta, Dothideomycetes sp., Penicillium sp., Cladosporium tenuissimum, Aspergillus versicolor, Penicillium sp., Entrophospora sp., Aspergillus sydowii, and Corynascus sepedonium. However, these fungi were completely inactivated by gamma irradiation at an absorbed dose of 20kGy on the front side. Monte Carlo N Particle Transport Code was used to simulate the doses applied to these cultural artifacts, and the measured dose distributions were well predicted by the simulations. These results show that irradiation is effective for the disinfection of cultural artifacts and that dose distribution can be predicted with Monte Carlo simulations, allowing the optimization of the radiation treatment.
► Radiation was applied for the disinfection of Korean cultural artifacts. ► Fungi on the artifacts were completely inactivated by the irradiation. ► Monte Carlo N Particle Transport Code was used to predict the dose distribution. ► This study is applicable for the preservation of cultural artifacts by irradiation.
In this study, a self-emulsifying drug delivery system (SEDDS) was employed to prepare novel squalene oil-based emulsion adjuvants. Deionized water, 0.01% and 0.02% (
/
) carbomer solutions of C-971P ...NF and C-940 grades were used to prepare emulsions containing 3%, 5% and 10% of squalene oil. Altogether 15 candidate emulsions were prepared and used as adjuvants for the delivery of a combination vaccine containing a porcine circovirus type 2 (PCV2) antigen and inactivated
(J101 strain) antigen. Most of the emulsions showed droplet sizes in the submicron range and maintained zeta potential values between -40 mV to 0 mV for six months, indicating good physical stability as a vaccine adjuvant. Emulsion-based candidate adjuvants prepared with SEDDS technology stimulated IgG, IgG1 and IgG2a like a currently commercially available adjuvant, Montanide ISA
201, and they were safe and their
-specific antibody titers were considered as comparable with that of Montanide ISA
201.
Secondary prevention via earlier detection would afford the greatest chance for a cure in premalignant lesions. We investigated the exomic profiles of non-malignant and malignant changes in head and ...neck squamous cell carcinoma (HNSCC) and the genomic blueprint of human papillomavirus (HPV)-driven carcinogenesis in oropharyngeal squamous cell carcinoma (OPSCC). Whole-exome (WES) and whole-genome (WGS) sequencing were performed on peripheral blood and adjacent non-tumor and tumor specimens obtained from eight Korean HNSCC patients from 2013 to 2015. Next-generation sequencing yielded an average coverage of 94.3× for WES and 35.3× for WGS. In comparative genomic analysis of non-tumor and tumor tissue pairs, we were unable to identify common cancer-associated early mutations and copy number alterations (CNA) except in one pair. Interestingly, in this case, we observed that non-tumor tonsillar crypts adjacent to HPV-positive OPSCC appeared normal under a microscope; however, this tissue also showed weak p16 expression. WGS revealed the infection and integration of high-risk type HPV16 in this tissue as well as in the matched tumor. Furthermore, WES identified shared and tumor-specific genomic alterations for this pair. Clonal analysis enabled us to infer the process by which this transitional crypt epithelium (TrCE) evolved into a tumor; this evolution was accompanied by the subsequent accumulation of genomic alterations, including an ERBB3 mutation and large-scale CNAs, such as 3q27-qter amplification and 9p deletion. We suggest that HPV16-driven OPSCC carcinogenesis is a stepwise evolutionary process that is consistent with a multistep carcinogenesis model. Our results highlight the carcinogenic changes driven by HPV16 infection and provide a basis for the secondary prevention of OPSCC. BMB Reports 2018; 51(11): 584-589
Behçet's disease (BD) is a chronic systemic inflammatory disorder characterized by four major manifestations: recurrent uveitis, oral and genital ulcers and skin lesions. To identify some pathogenic ...variants associated with severe Behçet's uveitis, we used targeted and massively parallel sequencing methods to explore the genetic diversity of target regions. A solution-based target enrichment kit was designed to capture whole-exonic regions of 132 candidate genes. Using a multiplexing strategy, 32 samples from patients with a severe type of Behçet's uveitis were sequenced with a Genome Analyzer IIx. We compared the frequency of each variant with that of 59 normal Korean controls, and selected five rare and eight common single-nucleotide variants as the candidates for a replication study. The selected variants were genotyped in 61 cases and 320 controls and, as a result, two rare and seven common variants showed significant associations with severe Behçet's uveitis (P<0.05). Some of these, including rs199955684 in KIR3DL3, rs1801133 in MTHFR, rs1051790 in MICA and rs1051456 in KIR2DL4, were predicted to be damaging by either the PolyPhen-2 or SIFT prediction program. Variants on FCGR3A (rs396991) and ICAM1 (rs5498) have been previously reported as susceptibility loci of this disease, and those on IFNAR1, MTFHR and MICA also replicated the previous reports at the gene level. The KIR3DL3 and KIR2DL4 genes are novel susceptibility genes that have not been reported in association with BD. In conclusion, this study showed that target enrichment and next-generation sequencing technologies can provide valuable information on the genetic predisposition for Behçet's uveitis.
Cadmium and nickel have been classified as carcinogenic to humans by the World Health Organization's International Agency for Research on Cancer. Given their prevalence in the environment, the fact ...that cadmium and nickel may cause diseases including cancer even at low doses is a cause for concern. However, the exact mechanisms underlying the toxicological effects induced by low-dose exposure to cadmium and nickel remain to be elucidated. Furthermore, it has recently been recognized that integrative analysis of DNA, mRNA and proteins is required to discover biomarkers and signaling networks relevant to human toxicant exposure. In the present study, we examined the deleterious effects of chronic low-dose exposure of either cadmium or nickel on global profiling of DNA copy number variation, mRNA and proteins. Array comparative genomic hybridization, gene expression microarray and functional proteomics were conducted, and a bioinformatics tool, which predicted signaling pathways, was applied to integrate data for each heavy metal separately and together. We found distinctive signaling networks associated with subchronic low-dose exposure to cadmium and nickel, and identified pathways common to both. ACTB, HSP90AA1, HSPA5 and HSPA8, which are key mediators of pathways related to apoptosis, proliferation and neoplastic processes, were key mediators of the same pathways in low-dose nickel and cadmium exposure in particular. CASP-associated signaling pathways involving CASP3, CASP7 and CASP9 were observed in cadmium-exposed cells. We found that HSP90AA1, one of the main modulators, interacted with HIF1A, AR and BCL2 in nickel-exposed cells. Interestingly, we found that HSP90AA1 was involved in the BCL2-associated apoptotic pathway in the nickel-only data, whereas this gene interacted with several genes functioning in CASP-associated apoptotic signaling in the cadmium-only data. Additionally, JUN and FASN were main modulators in nickel-responsive signaling pathways. Our results provide valuable biomarkers and distinctive signaling networks that responded to subchronic low-dose exposure to cadmium and nickel.
PDF
