Highly intense NIR emissive Cu4Pt2 bimetallic clusters featuring Pt(i)-Cu4-Pt(i) sandwich kernel.

Metal nanoclusters (NCs) capable of near-infrared (NIR) photoluminescence (PL) are gaining increasing interest for their potential applications in bioimaging, cell labelling, and phototherapy. However, the limited quantum yield (QY) of NIR emission in metal NCs, especially those emitting beyond 800 nm, hinders their widespread applications. Herein, we present a bright NIR luminescence (PLQY up to 36.7%, ∼830 nm) bimetallic Cu4Pt2 NC, [Cu4Pt2(MeO-C6H5-C[triple bond, length as m-dash]C)4(dppy)4]2+ (dppy = diphenyl-2-pyridylphosphine), with a high yield (up to 67%). Furthermore, by modifying the electronic effects of R in RC[triple bond, length as m-dash]C- (R = MeO-C6H5, F-C6H5, CF3-C6H5, Nap, and Biph), we can effectively modulate phosphorescence properties, including the PLQY, emission wavelength, and excited state decay lifetime. Experimental and computational studies both demonstrate that in addition to the electron effects of substituents, ligand modification enhances luminescence intensity by suppressing non-radiation transitions through intramolecular interactions. Simultaneously, it allows the adjustment of emitting wavelengths by tuning the energy gaps and first excited triplet states through intermolecular interactions of ligand substituents. This study provides a foundation for rational design of the atomic-structures of alloy metal NCs to enhance their PLQY and tailor the PL wavelength of NIR emission.

Clinical analysis of developmental and/or epileptic encephalopathy with spike-and-wave activation in sleep: A single tertiary care center experience in China.

PURPOSE: To analyze the electroclinical features of patients with developmental and/or epileptic encephalopathy with spike-and-wave activation in sleep (DEE/EE-SWAS) and study the efficacy of different therapies on seizure control, electroencephalogram (EEG) improvements of electrical status epilepticus during sleep (ESES), and cognition outcomes. METHODS: Patients with DEE/EE-SWAS who underwent at least one follow-up EEG 3 months after therapy were retrospectively enrolled. The demographic and clinical characteristics of the patients were analyzed. Variables that influenced the outcomes were evaluated using logistic regression models. RESULTS: In total, 87 patients (47 males) were included. The median age at ESES recognition was 81.0 months (IQR 64.0, 96.0). Forty-six patients were diagnosed with self-limited focal epilepsies (SeLFEs) before ESES recognition, 24 with developmental and epileptic encephalopathies with spike-and-wave activation in sleep (DEE-SWAS), and 17 with other epilepsies. Steroids, benzodiazepines, and antiseizure medications (ASMs) were the initial treatment options for ESES. Patients with structural etiologies or slow EEG backgrounds at the time of ESES recognition were less likely to respond to treatment than other patients. However, only children with slow EEG backgrounds had lower odds of response in logistic regression models. Children with clinical or EEG response showed improvements in cognition. CONCLUSION: Steroids, benzodiazepines, and ASMs are effective treatments for patients with DEE/EE-SWAS. Children with structural etiologies or slow EEG backgrounds at the time of ESES recognition may have a poor long-term prognosis. The efficacy of seizure reduction and EEG improvement is associated with cognitive improvement.

Determining individual suitability for neoadjuvant systemic therapy in breast cancer patients through deep learning.

BACKGROUND: The survival advantage of neoadjuvant systemic therapy (NST) for breast cancer patients remains controversial, especially when considering the heterogeneous characteristics of individual patients. OBJECTIVE: To discern the variability in responses to breast cancer treatment at the individual level and propose personalized treatment recommendations utilizing deep learning (DL). METHODS: Six models were developed to offer individualized treatment suggestions. Outcomes for patients whose actual treatments aligned with model recommendations were compared to those whose did not. The influence of certain baseline features of patients on NST selection was visualized and quantified by multivariate logistic regression and Poisson regression analyses. RESULTS: Our study included 94,487 female breast cancer patients. The Balanced Individual Treatment Effect for Survival data (BITES) model outperformed other models in performance, showing a statistically significant protective effect with inverse probability treatment weighting (IPTW)-adjusted baseline features [IPTW-adjusted hazard ratio: 0.51, 95% confidence interval (CI), 0.41-0.64; IPTW-adjusted risk difference: 21.46, 95% CI 18.90-24.01; IPTW-adjusted difference in restricted mean survival time: 21.51, 95% CI 19.37-23.80]. Adherence to BITES recommendations is associated with reduced breast cancer mortality and fewer adverse effects. BITES suggests that patients with TNM stage IIB, IIIB, triple-negative subtype, a higher number of positive axillary lymph nodes, and larger tumors are most likely to benefit from NST. CONCLUSIONS: Our results demonstrated the potential of BITES to aid in clinical treatment decisions and offer quantitative treatment insights. In our further research, these models should be validated in clinical settings and additional patient features as well as outcome measures should be studied in depth.

Deep learning-guided adjuvant chemotherapy selection for elderly patients with breast cancer.

PURPOSE: The efficacy of adjuvant chemotherapy in elderly breast cancer patients is currently controversial. This study aims to provide personalized adjuvant chemotherapy recommendations using deep learning (DL). METHODS: Six models with various causal inference approaches were trained to make individualized chemotherapy recommendations. Patients who received actual treatment recommended by DL models were compared with those who did not. Inverse probability treatment weighting (IPTW) was used to reduce bias. Linear regression, IPTW-adjusted risk difference (RD), and SurvSHAP(t) were used to interpret the best model. RESULTS: A total of 5352 elderly breast cancer patients were included. The median (interquartile range) follow-up time was 52 (30-80) months. Among all models, the balanced individual treatment effect for survival data (BITES) performed best. Treatment according to following BITES recommendations was associated with survival benefit, with a multivariate hazard ratio (HR) of 0.78 (95% confidence interval (CI): 0.64-0.94), IPTW-adjusted HR of 0.74 (95% CI: 0.59-0.93), RD of 12.40% (95% CI: 8.01-16.90%), IPTW-adjusted RD of 11.50% (95% CI: 7.16-15.80%), difference in restricted mean survival time (dRMST) of 12.44 (95% CI: 8.28-16.60) months, IPTW-adjusted dRMST of 7.81 (95% CI: 2.93-11.93) months, and p value of the IPTW-adjusted Log-rank test of 0.033. By interpreting BITES, the debiased impact of patient characteristics on adjuvant chemotherapy was quantified, which mainly included breast cancer subtype, tumor size, number of positive lymph nodes, TNM stages, histological grades, and surgical type. CONCLUSION: Our results emphasize the potential of DL models in guiding adjuvant chemotherapy decisions for elderly breast cancer patients.

Etiologies and clinical characteristics of acute ataxia in a single national children's medical center.

OBJECTIVE: To analyze etiologic factors of pediatric acute ataxia and to identify the severity of its underlying causes for urgent medical intervention. METHODS: Clinical data of children diagnosed with acute ataxia between December 2015 and December 2021 from one national medical center were analyzed retrospectively. RESULTS: A total of 99 children (59 boys, 40 girls), median age at disease onset 55 (range: 12-168) months, were enrolled. The median follow period was 46 (range 6-78) months. Eighty-six (86.9 %) children were diagnosed with immune-associated acute ataxia, among which acute post-infectious cerebellar ataxia (APCA) was the most common diagnosis (50.5 %), followed by demyelinating diseases of the central nervous system (18.2 %) and Guillain-Barré syndrome (9.1 %). On cerebrospinal fluid (CSF) examination, 35/73 (47.9 %) patients had pleocytosis (>5 cells/mm3), and 18/73 (24.7 %) had elevated protein levels. Thirty-one patients (31.3 %) had an abnormal cerebral MRI. Children with other immune-associated acute cerebellar ataxia had more extracerebellar symptoms, intracranial MRI lesions, abnormal CSF results, longer hospital stay, higher recurrence rates and incidence of neurological sequelae than children with APCA. CONCLUSION: Immune-associated acute ataxia is the main cause of pediatric acute ataxia, among which APCA is the most common phenotype. However, some immune-associated diseases, especially autoantibody-mediated disease, which has a higher recurrence rate and neurological sequelae account for an increasing proportion of pediatric acute ataxia. When children present with extracerebellar symptoms, abnormal cranial MRI or CSF results, and without prodromal infection, prudent differential diagnosis is recommended.

Simultaneous Determination of Thirteen Iridoid Glycosides in Crude and Processed Fructus Corni from Different Areas by UPLC-MS/MS Method.

Fructus Corni (F. Corni) is the dried mature pulp of Cornus officinalis Sieb. et Zucc.(Cornaceae), which is rich in iridoids. In this study, a simple, sensitive and rapid UPLC-MS/MS method was developed for the simultaneous determination of 13 iridoid glycosides of F. Corni from different areas. Specifically, we included five new compounds (cornusdiridoid C, cornusdiridoid E, cornusdiridoid F, 3'',5''-dehydroxycornuside and 2'-O-p-coumaroyl-kingiside) and isomers (2'-O-p-E-coumaroylloganin and 2'-O-p-Z-coumaroylloganin) for the first time in the quality markers of F. Corni. A total of 13 compounds and two pairs of isomers were well isolated and tested within just 14 min. All calibration curves showed good linear regression (r2 ≥ 0.99) within the tested concentration ranges. The limit of detection and limit of quantification were in the range of 0.19-1.90 and 0.38-3.76 ng/mL, respectively. The intra-day and inter-day precision were <3.21% and 12.49%, the RSD values of repeatability did not exceed 6.81% and the average recoveries were 90.95-113.59% for the analytes. All iridoid glycosides stabilized within 12 h (RSD < 10.99%). This method has been successfully applied to the quality evaluation of crude and processed F. Corni from different areas. The determination of characteristic iridoid glycosides and isomers will provide a more reliable and comprehensive method for the evaluation of F. Corni.

Long-term efficacy and safety of tacrolimus in young children with myasthenia gravis.

OBJECTIVE: This study was performed to evaluate the efficacy and long-term safety of tacrolimus for young children with myasthenia gravis (MG). METHODS: Children with corticosteroids (CSs)-ineffective, CSs-dependent or CSs-intolerable MG treated with tacrolimus for at least one year were recruited. The Myasthenia Gravis Foundation of America (MGFA) clinical classification and MGFA post-intervention status (MGFA-PIS) were used to evaluate before tacrolimus administration and at the last visit, respectively. MG Activities of Daily Living (MG-ADL) score and the dose of prednisone were recorded. Patients were divided into responders and poor responders based on changes in MG-ADL score to investigate the factors that affected tacrolimus efficacy. Unfavorable events were recorded. RESULTS: Twenty-one patients with MG were enrolled. The median age of starting tacrolimus was 8.7 (range 2.2-15.1) years old. At the last visit, 15 patients (71.4%) achieved minimal manifestation (MM) or better status. The symptoms evaluated by MG-ADL improved significantly one month after initiating tacrolimus (p＜0.05) and the dose of prednisone decreased significantly three months later (p＜0.05), and it continued to improve throughout the study. Thirteen patients (61.9%) were ultimately weaned off prednisone. Compared with 16 responders, 5 poor responders had lower MG-ADL scores. MG-ADL score was the only clinical factor of tacrolimus efficacy. Intraocular pressure and transient urine microprotein were present in one patient. CONCLUSION: A course of tacrolimus of more than one year was effective and well-tolerated in young children with MG, and tacrolimus improved MG symptoms and reduced the dose and adverse events of oral prednisone.

Pharmacological activities and therapeutic potential of galangin, a promising natural flavone, in age-related diseases.

BACKGROUND: The extension of average life expectancy and the aggravation of population aging have become the inevitable trend of human development. In an aging society, various problems related to medical care for the elderly have become increasingly prominent. However, most of the age-related diseases have the characteristics of multiple diseases at the same time, prone to complications, and atypical clinical manifestations, which bring great difficulties to its treatment. Galangin (3,5,7-trihydroxyflavone) is a natural active compound extracted from the root of Alpinia officinarum Hance (Zingiberaceae). Recently, many studies have shown that galangin has potential advantages in the treatment of neurodegenerative diseases and cardiovascular and cerebrovascular diseases, which are common in the elderly. In addition, it also showed that galangin had prospective activities in the treatment of tumor, diabetes, liver injury, asthma and arthritis. PURPOSE: This review aims to systematically summarize and discuss the effects and the underlying mechanism of galangin in the treatment of age-related diseases. METHODS: We searched PubMed, SciFinder, Web of Science and CNKI literature database resources, combined with the keywords "galangin", "neurodegenerative disease", "tumor", "diabetes", "pharmacological activity", "drug combination", "pharmacokinetics", "drug delivery system" and "safety", and comprehensively reviewed the pharmacological activities and mechanism of galangin in treating age-related diseases. RESULTS: According to the previous studies on galangin, the anti-neurodegenerative activity, cardiovascular and cerebrovascular protective activity, anti-tumor activity, anti-diabetes activity, anti-arthritis activity, hepatoprotective activity and antiasthmatic activity of galangin were discussed, and the related mechanisms were classified and summarized in detail. In addition, the drug combination, pharmacokinetics, drug delivery system and safety of galangin were furtherly discussed. CONCLUSIONS: This review will provide reference for galangin in the treatment of age-related diseases. Meanwhile, further experimental research and long-term clinical trials are needed to determine the therapeutic safety and efficacy of galangin.

The role of PDIA3 in oral squamous cell carcinoma and its value as A diagnostic and prognostic biomarker.

Background: This study aimed to investigate the role of protein disulfide isomerase A3 (PDIA3) in oral squamous cell carcinoma (OSCC) and evaluate its significance as a diagnostic and prognostic biomarker. Methods: Comprehensive bioinformatics analysis of the OSCC dataset from The Cancer Genome Atlas (TCGA) was performed. PDIA3 was depleted in CAL27 and SCC25 OSCC cells by transfection with PDIA3-specific siRNA oligos. The effects of PDIA3 downregulation on cell viability, apoptosis, and cell migration were evaluated using CCK8, ELISA, and wound healing assays, respectively. Results: The mRNA and protein expression of PDIA3 was significantly up-regulated in OSCC tissues compared to adjacent normal tissues. Knockdown of PDIA3 led to significantly decreased cell viability, increased apoptosis, and suppressed migratory ability in OSCC cells. The Kaplan-Meier survival curve showed that patients with higher PDIA3 expression levels had shorter survival than those with low PDIA3 levels. The receiver operating characteristic (ROC) curve indicated that PDIA3 had high sensitivity and accuracy for detecting OSCC (area under the curve (AUC): 0.917, CI: 0.879-0.955). Univariate and multivariate Cox regression analyses identified PDIA3 as an independent prognostic factor of OSCC. Furthermore, the depletion of PDIA3 inhibited AKT activity in OSCC cells. Gene set enrichment analysis (GSEA) indicated that PDIA3 is involved in various important biological functions and signaling pathways closely related to cancer development. Conclusion: PDIA3 plays an oncogenic role in OSCC and represents a good candidate as a diagnostic and prognostic biomarker for OSCC.

X-ray photochromism of chalcogenide frameworks linked with metal-amine chelates.

Reported here are two X-ray photochromic metal chalcogenide frameworks, which consist of tetrahedral clusters that are linked by transition-metal amine chelates. They have similar structures, but with different organic amine species, and they exhibit different coloration behavior. The photoinduced electron transfer from the metal chalcogenide clusters to the zinc amine chelates is a key point in accounting for their photochromism. Interestingly, a high-contrast (up to 12.4 times) enhancement of the optoelectronic response is obtained for the title compounds after they are treated by X-ray irradiation.

[Effects of addition of organic carbon with different chemical structure on the fate and accumulation of exogenous carbon in red and sandy soils]. / çº¢å£¤å’Œé£Žæ²™åœŸæ·»åŠ ä¸åŒåŒ–å­¦ç»“æž„æœ‰æœºç¢³å¯¹å¤–æºç¢³åŽ»å‘åŠç´¯ç§¯è´¡çŒ®çš„å½±å“.

Plant litter input has important influences on soil CO2 emission and soil organic carbon (SOC) formation in terrestrial ecosystem. However, it is not well known for the fate of carbon when exogenous organic matters with different chemical structures are added to soil with different textures. In this study, we added the uniformly 13C-labelled substrates of glucose, starch, and cellulose to red soil and sandy soil, and compared the net 13C accumulation and recovery and its proportions in soil releasing CO2, SOC, dissolved organic carbon (DOC) and microbial biomass carbon (MBC) pools. The results showed that δ13C values increased after exogenous substrate additions in CO2, SOC, DOC, and MBC, and that the peaks of δ13C in CO2 pool appeared delay with increasing chemical structure complexity. The fate of exogenous C and its contributions of different C pools were significantly influenced by exogenous C types, soil types, and incubation times. In sandy soil, the added exogenous C was more mineralized as CO2, with the net accumulation and recovery of 13C in CO2 pool decreasing in the order of glucose>starch>cellulose. In red soil, more exogenous C was transferred to SOC pool, with the net accumulation and recovery of 13C in SOC pool decreasing in the order of glucose>starch>cellulose. Our results implied that the chemical structure of exogenous substrates and soil texture together controlled the fate and accumulation of exogenous organic carbon.

[Effects of addition of leaf litter with different chemical properties on soil organic carbon mineralization and priming effect]. / ä¸åŒåŒ–å­¦æ€§è´¨å¶å‡‹è½ç‰©æ·»åŠ å¯¹åœŸå£¤æœ‰æœºç¢³çŸ¿åŒ–åŠæ¿€å‘æ•ˆåº”çš„å½±å“.

Litter inputs can affect the mineralization of soil organic carbon (SOC). However, it is yet unknown how the input of leaf litter with different chemical properties drives SOC mineralization and priming effect. In this study, 13C-labeled leaf litter of six tree species were added to soil cores (10 cm depth) collected from a natural secondary forest in subtropical region. We examined the effects of different leaf litters on total soil CO2, litter-derived and soil-derived CO2 emission rates and accumulation and priming effect. We further examined the relationships between litter chemical properties and CO2 accumulation and priming effect. Our results showed that leaf litter addition increased total soil CO2 and soil-derived CO2 emission rates and accumulations, and that there were positive priming effects ranging from 68% to 128%. Soil organic carbon mineralization and priming effects varied among tree species. The Pearson correlation and stepwise multiple linear regression analysis showed that the litter-derived CO2 accumulation had negative correlation with leaf litter C, P and cellulose concentrations, whereas the soil-derived CO2 accumulation were positively correlated to litter C:N and lignin:N. The results implied that tree species could influence SOC mineralization and litter-induced priming effect. Thus it could mitigate soil C loss when we afforested plantation with high quality leaf litter in subtropical region.

Ultrastable Anti-Acid "Shield" in Layered Silver Coordination Polymers.

Surface passivation technology provides noble-metal materials with limited chemical stability, especially under highly acidic condition. To design effective strategy to enhance stability of noble-metal particles, an understanding of their surface anticorrosion mechanism at the atomic level is desirable by using two-dimensional (2D) noble-metal coordination polymer (CP) as an ideal model for their interfacial region. With the protection of 2-thiobenzimidazole (TBI), we isolated two Ag-based 2D CPs, {Ag14 (TBI)12 X2 }n (S-X, where S denotes sheet and X=Cl or Br). These compounds exhibited excellent chemical stability upon immersion in various common solvents, boiling water, boiling ethanol, 10 % hydrogen peroxide, concentrated acid (12 M HCl), and concentrated alkali (19 M NaOH). Systematic characterization and DFT analyses demonstrate that the superior stability of S-X was attributed to the hydrophobic organic shell and dynamic proton buffer layer acting as a double protective "shield".

Clinical characteristics, disease course, and outcomes of paediatric patients with myelin oligodendrocyte glycoprotein-Ab associated disease: A retrospective clinical study.

OBJECTIVE: To delineate the outcomes of paediatric patients with myelin oligodendrocyte glycoprotein antibody disease (MOGAD). METHODS: We retrospectively analyzed the clinical characteristics, treatment, and outcomes of 34 paediatric patients with MOGAD from July 2015 to January 2020. RESULTS: The median age at disease onset was 75.5 months (range: 19-170 months). The female-to-male ratio was 1:1.1. The median follow-up duration was 34.5 months (range: 14-63 months). Acute disseminated encephalomyelitis (ADEM) was the most common initial phenotype (52.9%), followed by optic neuritis (ON) (20.6%). Children with ADEM were younger than those with ON (P = 0.045). Twenty-eight (82.4%) and 18 (56.3%) children had abnormal brain and spinal magnetic resonance imaging, respectively, during the first acute attack. MOG-abs titers in children with ON were statistically higher than those in children with ADEM (P = 0.04). Thirty-two children accepted glucocorticoid treatment, while 33 (97%) children demonstrated clinical improvement within 1 week, 21 children (61.8%) achieved clinical recovery within 1 month. Eight children (23.5%) suffered a relapse, the median interval between the initial attack and recurrence was 13 (range: 3-36) months. We detected neurological sequelae in seven (20.6%) children, with visual dysfunction being the most common sequela (85.7%). CONCLUSION: ADEM was the most common phenotype in both monophasic and relapsed paediatric MOGAD, followed by ON. Majority of pediatric MOGAD patients were highly responsive to glucocorticoid. Despite a benign prognosis in most patients, some patients endure neurological sequelae, mainly visual impairment. Patients with initial visual impairment should be carefully evaluated and administered individualized immunotherapy.

Immunotherapies for Anti-N-M-methyl-D-aspartate Receptor Encephalitis: Multicenter Retrospective Pediatric Cohort Study in China.

Background: Anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis has been discovered for more than a decade, but the establishment of standardized immunotherapy protocol for pediatric patients still needs more clinical evidence. Methods: A multicenter, retrospective study was conducted on pediatric patients diagnosed with anti-NMDAR encephalitis between November 2011 and December 2018. The clinical records including clinical manifestations, immunotherapy strategies, and outcomes were collected and analyzed. Results: A total of 386 patients were included in our study and the median onset age was 8.00 (IQR 4.83-10.90) years. All patients received first-line immunotherapy and the majority (341, 88.3%) used the standard combination of methylprednisolone pulses (MEP) and intravenous immunoglobulins (IVIG), but 211 patients did not show satisfactory improvement (mRS ≥ 3). Mainly three treatment strategies were applied after first-line immunotherapy: second-line immunotherapy, repetitive first-line immunotherapy, and maintaining oral prednisolone. For patients with mRS ≥ 4 after first-line immunotherapy, the incidence of poor outcome (mRS ≥ 3) in oral prednisolone group was higher than that in other treatment groups (p = 0.039). No difference in complete recovery rate (mRS = 0) was found between patients receiving second-line and repetitive first-line immunotherapy, or patients using long-term and short-term prednisolone. Out of 149 patients who received anti-myelin oligodendrocyte glycoprotein antibody (MOG-Ab) test, 27 (18.12%) were positive. Patients with concomitantly positive MOG-Ab showed milder conditions compared to patients with typical anti-NMDAR encephalitis and were more inclined to relapses. We also identified female, MOG-Ab positive, and not receiving second-line and/or repetitive first-line immunotherapy were risk factors for relapses. Conclusions: For patients with mRS ≥ 4 after first-line immunotherapy and patients with concomitantly positive MOG-Ab, second-line immunotherapy is recommended. When second-line immunotherapy is not applicable, repetitive first-line immunotherapy can be considered as an option. Both second-line and repetitive first-line immunotherapy are beneficial to reduce relapse rate. The duration of sequential oral prednisolone can be shortened after fully evaluating patients' conditions.

Correlation between epilepsy and genotype: A large retrospective tuberous sclerosis complex cohort.

OBJECTIVE: To describe the first large population (n = 297) with tuberous sclerosis complex (TSC) in China and to examine the relationship between variants (type and location) and epilepsy. METHODS: All exons and intron-exon boundaries of TSC1/TSC2 were sequenced with next-generation sequencing, and the distribution of several variants and associations between variant types and epilepsy were investigated. RESULTS: Epilepsy occurred in 83.5% (248/297) of the individuals. The TSC1/TSC2 gene variant detection rate was 89.6% (266/297). The rate of epilepsy was significantly higher in the TSC2 group than in the TSC1 (p = 0.02) and no mutation identified (NMI) groups (p = 0.0005). TSC2 individuals are more likely to have spasms than TSC1 individuals (p =0.03). The age at epilepsy onset of individuals in the TSC2 group was younger than that of individuals in the TSC1 group (p = 0.008) and NMI group (p = 0.01). The age at epilepsy onset with truncated variants in the TSC1 group was significantly younger than that of individuals with nontruncated variants (p = 0.0001). The rate of epilepsy was significantly higher if variants occurred in the functional domain than in the nonfunctional domain in TSC2 individuals (p = 0.02). CONCLUSION: This was the first large cohort study of the Chinese TSC population in which a comparative analysis of genotype and epilepsy was conducted. Individuals with TSC2 variants appeared to have more severe epileptic phenotypes, such as younger age at epilepsy onset, than those with TSC1 variants and NMI, and individuals with variants that occurred in TSC2 functional domains were more prone to epilepsy and had a younger age at epilepsy onset.

A Direction-of-Arrival Estimation Algorithm Based on Compressed Sensing and Density-Based Spatial Clustering and Its Application in Signal Processing of MEMS Vector Hydrophone.

Direction of arrival (DOA) estimation has always been a hot topic for researchers. The complex and changeable environment makes it very challenging to estimate the DOA in a small snapshot and strong noise environment. The direction-of-arrival estimation method based on compressed sensing (CS) is a new method proposed in recent years. It has received widespread attention because it can realize the direction-of-arrival estimation under small snapshots. However, this method will cause serious distortion in a strong noise environment. To solve this problem, this paper proposes a DOA estimation algorithm based on the principle of CS and density-based spatial clustering (DBSCAN). First of all, in order to make the estimation accuracy higher, this paper selects a signal reconstruction strategy based on the basis pursuit de-noising (BPDN). In response to the challenge of the selection of regularization parameters in this strategy, the power spectrum entropy is proposed to characterize the noise intensity of the signal, so as to provide reasonable suggestions for the selection of regularization parameters; Then, this paper finds out that the DOA estimation based on the principle of CS will get a denser estimation near the real angle under the condition of small snapshots through analysis, so it is proposed to use a DBSCAN method to process the above data to obtain the final DOA estimate; Finally, calculate the cluster center value of each cluster, the number of clusters is the number of signal sources, and the cluster center value is the final DOA estimate. The proposed method is applied to the simulation experiment and the micro electro mechanical system (MEMS) vector hydrophone lake test experiment, and they are proved that the proposed method can obtain good results of DOA estimation under the conditions of small snapshots and low signal-to-noise ratio (SNR).

Quality of life in children with tuberous sclerosis complex: A pediatric cohort study.

AIMS: To evaluate the quality-of-life (QOL) impairment and identify the possible risk factors in patients with tuberous sclerosis complex (TSC) in China. METHODS: The parent proxy-report PedsQL 4.0 Generic Core Scales were administered to 124 caregivers of children with TSC (aged 2-18 years). For comparison, the survey was also conducted in a demographically group-matched sample of healthy controls (HCs) (aged 2-18 years). RESULTS: A total of 124 children with TSC and 206 HCs were recruited. The mean parent proxy-report total scale score, physical health summary score, and psychosocial health summary score for children with TSC were 65.0 (SD 19.7), 77.6 (SD 22.9), and 58.0 (SD 21.3), respectively, compared with the HC values of 83.6 (SD 14.3), 87.2 (SD 16.9), and 82.8 (SD 15.9). There were statistically significant differences between the two groups (P < .0001). TSC2 mutation (P = .033), epilepsy (P = .011), seizure before 2 years old (P = .001), course of epilepsy (more than 2 years) (P = .001), high reported seizure frequency (more than once a month) (HRSF) (P = .007), multiple antiepileptic drugs (≥2) (P = .002), intellectual disability (ID) (mild and moderate ID, P < .0001, and severe and profound ID, P < .0001), and TANDs (P < .0001) (ADHD, P = .004; agoraphobia, P = .007; and social anxiety disorder, P < .0001) were closely related to lower QOL scores. CONCLUSION: This study is the first large cohort study on QOL in children with TSC in China. The results of the PedsQL 4.0 indicated that the QOL of children with TSC is significantly lower than that of HCs. TSC2 mutation, epilepsy, early onset, long disease course and HRSF, ID, and TANDs are risk factors for poor QOL.