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Background This study aimed to evaluate the efficacy and retention rate of a ketogenic diet (KD) and assess factors that influence the efficacy of KD therapy in children with refractory epilepsy (RE). Methods We retrospectively studied the efficacy and retention rate of 56 RE children who accepted KD therapy from January 2013 to December 2019. Patients who had a ≥50% reduction in seizure frequency were defined as responders. The retention rate was calculated as the proportion of children who continued KD/the total number of children who were followed up at the time of enrollment. We also analyzed the effects of different factors (such as gender, KD initial age, KD duration, the type of epilepsy syndrome, and others) on the efficacy of the KD. Results (1) The efficacy rates for the KD at 3, 6, 12, and 18 months were 51.8, 53.6, 39.2, and 23.2%, respectively. (2) The retention rates for the KD at 3, 6, 12 and 18 months were 100, 69.6, 41.1, and 23.2%, respectively. (3) There was no correlation between efficacy and gender, epilepsy onset age, the type of epilepsy syndrome, electroencephalogram improvement, or the number of antiseizure medications, while cranial magnetic resonance imaging (MRI) abnormalities, KD duration, and KD initial age affected its efficacy at 3 months. Conclusion (1) KD therapy for refractory childhood epilepsy was effective and produced a high retention rate. (2) MRI abnormalities and the initial age and duration of KD influenced its short-term efficacy in RE children.

The high-pathogenicity island (HPI), carried by Yersinia pestis, is an essential pathogenic component of the virulence distributed in Escherichia coli (E. coli). As an NF-κB pathway activator, Ubiquitin (Ub) is crucial in the inflammatory response. However, the molecular mechanismsof Ub in the TLR4/Myd88/NF-κB signaling pathway induced by HPI in E. coliis still unclear. In our experiment, Ub was overexpressed usingsmall intestinal epithelial cells (in vitro), and BALB/c mouse models (in vivo) infected with E. coli HPI and analysed by using qPCR, ELISA, immunofluorescence, immunohistochemistry, and H&E staining assays. We demonstrated that E. coli HPI can promote the expression of TLR4, NF-κB, and Ub in. IPEC-J2 cells. Immunofluorescence and Immunohistochemistry analysis revealed that TLR4 and NF-κB key factor in the E. coli HPI group were expressed, and the NF-κB p65 protein translocated to the nucleus. Further research showed that the mRNA expression levels of TLR4, MyD88, NF-κB, IL-1β, and TNF-α were significantly increased in the Ub overexpression group and BALB/c mice group. H&E staining assay showed that Ub overexpression with IPEC-J2 cells have thecharacteristics of elongated, rounded, fragmented, duodenal villi diminished, and the surface was covered with many sloughed necrotic cells, indicating that exacerbating cell damage and duodenitis. Taken together, this study suggested that E. coliHPI can activate NF-κB via increasing the expression of Ub, andUb can exacerbate E. coli HPI-induced duodenitis through the TLR4/MyD88/NF-κB signaling pathway.

An experimental investigation of the stereocamera's systematic error is carried out to optimize three-dimensional (3-D) dust observation on the HL-2A tokamak. It is found that a larger 3-D region occupied by all calibration points is able to reduce the 3-D reconstruction systematic error of the stereocamera. In addition, the 3-D reconstruction is the most accurate around the region where the calibration points are located. Based on these experimental results, the design of the stereocamera on the HL-2A tokamak is presented, and a set of practical procedures to optimize the 3-D reconstruction accuracy of the stereocamera are proposed.

Objective To determine the diagnostic accuracy of pneumococcal antigen detection in diagnosis of pneumococcal meningitis in children. Methods Purulent meningitis was diagnosed according to European Society for Clinical Microbiology and Infectious Diseases (ESCMID) guideline between July 2014 and June 2016. Along with a cerebrospinal fluid (CSF) culture, pneumococcal antigen detection in cerebrospinal fluid (CSF) was performed, and further identification of pathogens was done with 16S rDNA-PCR and high-throughput sequencing. Results CSF samples collected from 184 children (median age of 1.92 mo). CSF culture was used as the gold standard. 46 (25%) had positive results for culture and 10 (5.4%) were pneumococci; 34 (18.5%) were pneumococcal antigen positive. The sensitivity and specificity of pneumococcal antigen detection were 100% (95% CI: 89.4%-100%) and 86.2% (95% CI: 96.4%-99.9%), respectively. 92.3% (12/13) were confirmed by nucleic acid detection to be pneumococci. Conclusions Pneumococcal antigen detection in CSF has adequate sensitivity and specificity in diagnosing pneumococcal meningitis.

Anxiety is a common emotional disorder in children. To understand its underlying mechanisms, chronic unpredictable stress (CUS) has been established as a stress model in zebrafish. By using the tall tank test, the stress response reliability could be improved in adult fish which has not been confirmed in larvae. In addition, the increasing evidences have shown that cerebellum plays important roles in anxiety. Whether CUS will affect cerebellar neuronal activity remains unknown. We found that CUS exposure to larvae (from 10 to 17 days post fertilization) induced anxiety-like behaviors and social cohesion impairments within 1-2 d after CUS, including a prolonged freezing time, an increased time spent at the bottom of tank, an increased thigmotaxis index, and an increased interindividual distance. Our results showed that the four behavioral tests were homogeneous, especially the tall tank test either anxiety-like behaviors or the basal locomotion. Furthermore, we found that CUS enhanced the excitability of cerebellar neurons, as the amplitude, frequency, time to peak and half-width of spontaneous firing significantly decreased, as well as the amplitude of excitatory post-synaptic current when compared with the control group. CUS also activated hyperpolarization-activated cyclic nucleotide-gated and potassium channels of cerebellar neurons. Multiple linear regression analysis showed that the total distance in bottom (tall tank test) was correlated positively with outward Na

Rett syndrome (RTT) is a neurodevelopmental disease in children that is mainly caused by mutations in the MeCP2 gene, which codes for a transcriptional regulator. The expression of insulin-like growth factor-1 (IGF-1) is reduced in RTT patients and animal models, and IGF-1 treatment is a promising therapeutic strategy for RTT. However, the mechanism underlying the effects of IGF-1 remains to be further explored. FXYD1 is an auxiliary subunit of Na, K-ATPase. Overexpression of FXYD1 is involved in the pathogenesis of RTT. However, whether IGF-1 exerts its effect through normalizing FXYD1 is completely unknown. To this end, we evaluated the effect of IGF-1 on FXYD1 expression and posttranslational modification in a mouse model of RTT (MeCP2308) using both in vitro and in vivo experiments. The results show that FXYD1 mRNA and phosphorylated protein (p-FXYD1) were significantly elevated in the frontal cortex in RTT mice, compared to wild type. In RTT mice, IGF-1 treatment significantly reduced levels of FXYD1 mRNA and p-FXYD1, in parallel with improvements in behavior, motor coordination, and cognitive function. For mechanistic insight into the effect of IGF-1 on p-FXYD1, we found the decreased phosphorylated forms of PI3K-AKT-mTOR signaling pathway components in the frontal cortex of RTT mice and the normalizing effect of IGF-1 on the phosphorylated forms of these components. Interestingly, blocking the PI3K/AKT pathway by PI3K inhibitor could abolish the effect of IGF-1 on p-FXYD1 level, in addition to the effect of IGF-1 on the phosphorylation of other components in the PI3K/AKT pathway. Thus, our study has provided new insights into the mechanism of IGF-1 treatment for RTT, which appears to involve FXYD1.

Background: Tic disorders (TD) are common neuropsychiatric disorders among children and adolescents. It is controversial that trace elements may participate in the pathogenesis of TD. Our study aimed to investigate the trace elements status of zinc (Zn), copper (Cu), iron (Fe), and magnesium (Mg) in children with TD, in comparison to healthy controls.Methods: The medical records of eligible TD children and normal healthy children from January 1 to December 31, 2018 in the outpatient clinic were retrospectively reviewed. The clinical information of all subjects were collected including age, gender, diagnosis, previous health records, and serum trace elements level (Cu, Zn, Fe, Mg) at the time of diagnosis before initiating treatment.Results: In total, 1204 TD children (7.63 ± 2.45 years) and 1,220 healthy children (7.27 ± 3.15 years) who were divided into two gender and three age groups (2–4years, 5–9years, ≥10 years) were reviewed in our study. Our study showed that TD children generally had lower whole blood levels of Zn, Cu, Fe than the normal controls (P < 0.01). No significant difference was observed in whole blood levels of Mg. After adjusting for gender, the trends still remained. Further analysis was performed according to age, the trends still remained in Zn and Fe in all age groups (P < 0.05). However, we observed an almost significantly (P = 0.055) lower level of Cu in TD of 2–4 years group while significant differences in other two groups (P < 0.01). Further multiple linear regression and point biserial correlation showed that the lower blood levels of Zn, Cu, and Fe were correlated with the incidence of TD.Conclusion: The present results indicated that lower blood levels of zinc, iron, copper were associated with TD. Trace elements may be used as an auxiliary treatment for TD and need to be further explored.

Introduction: Duchenne muscular dystrophy (DMD) is an X-linked autosomal recessive genetic disorder caused by mutations in DMD gene. Approximately 70% of the mutations are caused by deletions or duplications of DMD exons, while the remaining were minor mutations. Case report: We present a 5-year-old boy with typical clinical features of DMD. A novel mutation was identified as a c.9358_9359insA of DMD gene by next-generation sequencing. This mutation which was origined from mother, generated a frameshift mutation and resulted in abnormal synthesis of protein polypeptide chains. Conclusion: We demonstrated a novel mutation of DMD gene and expanded the spectrum of mutations causing DMD.

Mass customization (MC) under the context ofthe Internet of Things (IoT) is expected to reform traditionalmass manufacturing. To contribute to MC from information communication and user interaction ...

Due to uneven lighting conditions and differences between camera lenses, in actual engineering applications, there is a difference in brightness of the image collected by the camera, which will cause obvious gaps in video stitching and poor stitching effect of panoramic pictures. This paper designs and implements an algorithm based on the gray statistics of overlapping areas to achieve global correction, and combined with the fade-in and fade-out image fusion algorithm to make the splicing transition natural. The experimental results show that in the Visual Studio 2015 platform environment, real-time stitching of 8-channel cameras has a fast algorithm speed and can achieve a stitching speed of 25 frames per second. The video is smooth and the picture quality is good without obvious color difference.

Wilson’s disease (WD) is an autosomal recessive genetic disorder of copper metabolism, caused by mutations in the ATP7B gene, resulting in copper accumulation in the liver, brain, kidney, and cornea and leading to significant disability or death if untreated. Early diagnosis and proper therapy usually predict a good prognosis, especially in pre-symptomatic WD. Genetic testing is the most accurate and effective diagnostic method for early diagnosis. The clinical and biochemical features of three unrelated Han Chinese families with pre-symptomatic WD were reported. The molecular defects in these families were investigated by polymerase chain reaction and DNA sequencing. Hundred healthy children with the same ethnic background served as controls. Bioinformatic tools (polymorphism phenotyping-2, sorting intolerant from tolerant, protein analysis through evolutionary relationships, and predictor of human deleterious single nucleotide polymorphisms) were combined and used to predict the functional effects of mutations. We identified 2 novel ATP7B mutations (p.Leu692Pro and p.Asn728Ser) and 3 known mutations (p.Met769fs, p.Arg778Leu and p.Val1216Met) in these Chinese WD families. These mutations were not observed in the 100 normal controls. The bioinformatic method showed that p.Leu692Pro and p.Asn728Ser mutations are pathogenic. Our research enriches the mutation spectrum of the ATP7B gene worldwide and provides valuable information for studying the mutation types and mode of inheritance of ATP7B in the Chinese population. Liver function analysis and genetic testing in young children with WD are necessary to shorten the time to the initiation of therapy, reduce damage to the liver and brain, and improve prognosis.

This work proposes a new power management scheme for wirelessly-powered UHF RFID tags with flexible inkjet-printed Electrochromic (EC) display for human-to-device interaction. EC display on polyimide substrate is integrated at tag side to provide an ambient and direct human-to-device display interface. An aggressive duty-cycling power management scheme with dual supplies is designed to drive the EC display under the tag power budget in microwatt level through RF energy harvesting. In this scheme, energy for display refreshing is accumulated over multiple power management cycles. A single-pixel addressing scheme with minimal pixel size is proposed to further reduce display power and improve tag sensitivity by exploiting EC display bi-stability. The experimental results show that the EC display can be refreshed with the tag sensitivity of −10.5 dBm at 11.7 sec/cm2 update rate.

Background Pediatric cerebral sparganosis has been seldom reported. In the current study, we retrospectively reviewed the clinicopathologic records of 9 consecutive pediatric cases of cerebral sparganosis and analyzed their epidemiologic characteristics and clinical outcomes. Methods Our cases included 6 boys and 3 girls, all from rural areas, and their median age at diagnosis was 9.4 (range, 5.8–12.9) years. The median duration of symptoms from onset to definite diagnosis was 21 months (range, 1 week to 3.7 years). Results Enzyme-linked immunosorbent assay revealed that serum anti-sparganosis antibody was positive in 9 of 9 patients and cerebrospinal fluid anti-sparganosis antibody was positive in 4 of 6 patients. Eight patients underwent craniotomy the removal of worms. The patients also received oral praziquantel. They were followed up for 2.2 years to 4.4 years. One patient died, and 8 patients survived. Three cases had poor outcomes whereas the outcome of the remaining 5 cases was satisfactory. Conclusions Children are more at risk for sparganosis and cerebral sparganosis may be missed because of unclear epidemiologic history and nonspecific manifestations. Cerebrospinal fluid eosinophil counts and enzyme-linked immunosorbent assay for anti-sparganosis antibody and computed tomography/magnetic resonance imaging scans may be relied on for an early and accurate diagnosis before surgery.

Background Reversible bilateral striatal necrosis associated with Mycoplasma pneumoniae (M. pneumoniae) infection is a rare neurological disease. The exact pathogenic mechanism remains unknown. Patient We report reversible bilateral striatal lesions with a favorable outcome secondary to M. pneumoniae infection in an 8-year-old Chinese girl. Cranial MRI showed abnormal signals in bilateral striatum, which disappeared 8 months later. To better understand the pathogenesis of this encephalopathy, we examined cytokines levels in serum and cerebrospinal fluid from this patient. The results revealed the concentrations of interleukin-6 and interleukin-8 increased significantly in serum (26 pg/mL and 66 pg/mL, respectively) and cerebrospinal fluid (122 pg/mL and 325 pg/mL, respectively), and were reduced markedly after the therapy. Intrathecal production of interleukin-6 and interleukin-8 is probably related to the pathogenesis of striatal lesions caused by M. pneumoniae . These cytokines may cause local vascular injury, and finally leading to local vascular occlusion. Conclusion Our results suggest that interleukin-6 and interleukin-8 may play important roles in the pathogenesis of this disease. This is the first report to describe the role of cytokines in this condition and relevant literature is reviewed. Our findings may lead to better understanding of the pathogenesis of M. pneumoniae -associated striatal lesions.

This paper proposes an asymmetric ultra-wideband - radio frequency identification (UWB-RFID) tag with electrocardiogram (ECG)-sensing capability for patients remote-monitoring in hospital environment. A UWB-RFID communication protocol is suggested for real-time transmission of undistorted ECG by interleaving ADC sampling and burst-mode UWB transmission. The proposed system shows a maximum accessing capability of 400 tags/second at 1.5 KHz ECG sampling rate with 10 Mbps UWB pulse rate. The tag consists of UHF-RFID receiver, UWB transmitter, ECG analog front-end, multi-input ADC and baseband circuitry integrated on two silicon dies. It was implemented by 6 mm2 -sized 180 nm CMOS technology. Electrodes for ECG-sensing are manufactured by inkjet-printing on polyimide substrate. Experiment results show that the tag transmits UWB pulses at 1 Mbps rate with 18 µW power. The printed electrodes conduct ECG waveform comparable to commercial electrodes.

This paper presents a phase noise improvement method for fine tuning of type-I ADPLL by exploiting its nonlinear quantization effects. When quantization step approaches the same orders of magnitude of standard deviation of input noise, quantization effects become nonlinear, and additive noise modeling of quantization effects is no longer applicable. By proper offsetting the input signals in this case, the feedback loop of ADPLL can be very sensitive to frequency phase deviation. It results in a larger loop bandwidth, and in turn smaller in-band phase noise than in linear quantization case. A theoretic s-domain noise model is proposed to quantify the phase noise in nonlinear case. Results are verified in Modelsim simulations. Proposed method offers possibility of achieving lower phase noise by lower quantization resolution and less circuit design efforts. QC 20150601