Expert Consensus on Nutritional Support for Children with Congenital Heart Disease(2023 Edition)

The second edition of the expert consensus on pediatric nutrition was formed based on a global update of pedia-tric nutrition guidelines or consensus worldwide,the management of congenital heart disease,and the results of multi-center clinical nutrition research for congenital heart disease following thefirst Chinese consensus edition of 2016.The consensus was also shaped by the results of three discussion sessions and two questionnaires con-ducted by the 13-member collaboration group.This process was informed by both clinical guidelines and expert consensus.The quality of literature,both in English and Chinese,and the level of recommendations were evaluated using the Grading of Recommendations Assessment,Development,and Evaluations(GRADE)system.

Tunnel boring machine vibration-based deep learning for the ground identification of working faces

Tunnel boring machine(TBM) vibration induced by cutting complex ground contains essential information that can help engineers evaluate the interaction between a cutterhead and the ground itself.In this study,deep recurrent neural networks(RNNs) and convolutional neural networks(CNNs) were used for vibration-based working face ground identification.First,field monitoring was conducted to obtain the TBM vibration data when tunneling in changing geological conditions,including mixed-face,homogeneous,and transmission ground.Next,RNNs and CNNs were utilized to develop vibration-based prediction models,which were then validated using the testing dataset.The accuracy of the long short-term memory(LSTM) and bidirectional LSTM(Bi-LSTM) models was approximately 70% with raw data;however,with instantaneous frequency transmission,the accuracy increased to approximately 80%.Two types of deep CNNs,GoogLeNet and ResNet,were trained and tested with time-frequency scalar diagrams from continuous wavelet transformation.The CNN models,with an accuracy greater than 96%,performed significantly better than the RNN models.The ResNet-18,with an accuracy of 98.28%,performed the best.When the sample length was set as the cutterhead rotation period,the deep CNN and RNN models achieved the highest accuracy while the proposed deep CNN model simultaneously achieved high prediction accuracy and feedback efficiency.The proposed model could promptly identify the ground conditions at the working face without stopping the normal tunneling process,and the TBM working parameters could be adjusted and optimized in a timely manner based on the predicted results.

A novel mutation of glycogen synthase kinase-3β leads to a reduced level of GSK3βprotein in a patient with dilated cardiomyopathy

Dilated cardiomyopathy(DCM)is a frequently inherited heart condition.A familial transmission rate of 20%-35%has been documented,and most of these cases are autosomal dominant.This condition has a high rate of morbidity and mortality,which can lead to cardiac arrest and persistent heart failure.

Identification of a novel MYO1D variant associated with laterality defects,congenital heart diseases,and sperm defects in humans

The establishment of left–right asymmetry is a fundamental process in animal development.Interference with this process leads to a range of disorders collectively known as laterality defects,which manifest as abnormal arrangements of visceral organs.Among patients with laterality defects,congenital heart diseases(CHD)are prevalent.Through multiple model organisms,extant research has established that myosin-Id(MYO1D)deficiency causes laterality defects.This study investigated over a hundred cases and identified a novel biallelic variant of MYO1D(NM_015194:c.1531G>A;p.D511N)in a consanguineous family with complex CHD and laterality defects.Further examination of the proband revealed asthenoteratozoospermia and shortened sperm.Afterward,the effects of the D511N variant and another known MYO1D variant(NM_015194:c.2293C>T;p.P765S)were assessed.The assessment showed that both enhance the interaction withβ-actin and SPAG6.Overall,this study revealed the genetic heterogeneity of this rare disease and found that MYO1D variants are correlated with laterality defects and CHD in humans.Furthermore,this research established a connection between sperm defects and MYO1D variants.It offers guidance for exploring infertility and reproductive health concerns.The findings provide a critical basis for advancing personalized medicine and genetic counseling.

Hybridization-mediated quasiparticle and phonon dynamics in single crystal cerium films

We report the ultrafast optical pump-probe spectroscopy measurements on the single-crystal cerium films.Our experimental results of temperature-dependent quasiparticle dynamics reveal development of the hybridization between localized f moments and conduction electrons,i.e.,evolving from fluctuating hybridization to collective hybridization.Exotic phonon renormalization is discovered to appear at the emerging temperature(T^(+))of fluctuating hybridization apart from its known presence at the coherent temperature(T^(*)),and can hardly be explained by the mean-field theory.The quasiparticle relaxation at high temperatures indicates coexistence of nonthermal electron-electron scattering and inelastic Kondo scattering,while its behavior at low temperatures suggests onset ofαphase in theγ-phase dominated film.We also extract the indirect gap below T^(*),representing the appearance of collective hybridization.Our findings provide novel information about the hybridization and phase evolution in the heavy fermion systems.

Association study of the single nucleotide polymorphisms in adiponectin-associated genes with type 2 diabetes in Han Chinese

Single-nucleotide polymorphisms （SNPs） of ADIPOQ, ADIPOR1, and ADIPOR2 have been associated with type 2 diabetes mellitus （T2DM）, but there are many conflicting results especially in Chinese populations. To investigate the contribution of the adiponectin genes and their receptors to T2DM, a case-control study was performed and 11 SNPs ofADIPOQ, ADIPOR1, and ADIPOR2 were genotyped in 985 T2DM and 1,050 control subjects, rs 16861194 （-11426 A〉G） in the putative promoter of ADIPOQ was associated with T2DM （P = 0.007; OR = 1.29, 95% CI 1.08-1.55）. None of the other 10 SNPs were associated with T2DM in this study, although rs2241766 and rs1501299 were reported to be associated with T2DM in previous Chinese studies. There was also no significant difference found from the ADIPOQ haplotype analysis, which contains rs 16861194. In addition, we also assessed potential gene-gene interactions in three genes and no interactions were found. In conclusion, our results supported the ADIPOQ gene as a possible risk factor for type 2 diabetes in Han Chinese population.

Study on the controllable synthesis of SH-MCM-41 mesoporous materials and their adsorption properties of the La3+, Gd3+and Yb3+

Sulfhydryl MCM-41(SH-MCM-41) mesoporous materials were prepared via a hydrothermal method,and-SH was successfully imported by a post-grafting method.The structure and surface properties of the materials were characterized using Fourier Transform infrared spectroscopy,X-ray diffraction and Transmission Electron Microscopy analysis.The low concentrations of La^3+,Gd^3+ and Yb^3+ adsorption on the material were investigated.This paper discusses the effects of system factors,such as pH and the solid-liquid ratio,on the performance of the adsorption process.The adsorption thermodynamics and kinetics were also explored.Experimental results indicated that the materials were in good order and had high specific surface area(956 m^2/g) with an average pore diameter of 2.1 nm;the mercapto groups were successfully grafted onto a molecular sieve,and the best grafted amount was 1.46×10^-3 mol/g.The materials showed preferable adsorption of La^3+,Gd^3+ and Yb^3+ with maximum adsorption capacities of560.56 mg/g,467.60 mg/g and 540.68 mg/g,respectively.The adsorption process can be described by the Freundlich isotherm model,and the adsorption data fits pseudo-second-order kinetics.After repeating the elution-regeneration cycle four times,the adsorption capacity of rare earth ions was mostly maintained,indicating that the adsorbent can be regenerated well and recycled to save costs.It has potential in practical application.

Preparation and properties of GO-based lanthanum ion-imprinted polymer,La-IIP-MAA/Fe_(3)O_(4)-GO

In this article,ion-imprinting technology was used to prepare the lanthanum ion-imprinted polymer,LaIIP-MAA/Fe_(3)O_(4)-GO with magnetic graphene oxide as a carrier and methacrylic acid as a functional monomer.A series of characterizations were performed on the imprinted materials,the adsorptive and selective properties of the imprinted materials for lanthanum ions were explored,and the adsorption process was analyzed with respect to kinetics and thermodynamics.Finally,the reusability of the imprinted materials was studied.The results show that La-IIP-MAA/Fe_(3)O_(4)-GO has good adsorption effects for lanthanum ions,and adsorption equilibrium can be attained in about 20 min.When pH is 6 and the temperature is 35℃,the adsorption capacity reaches 110.98 mg/g.The adsorption process of lanthanum ions is pretty much in line with the quasi-second-order kinetic model and Freundlich isotherm adsorption model.La-IIP-MAA/Fe_(3)O_(4)-GO has good selectivity to lanthanum ions and maintains good effects after repeated use five times.

302 W triple-frequency,single-mode,linearly polarized Yb-doped all-fiber amplifier

Stimulated Brillouin scattering(SBS) effect is currently the major limitation for the power scaling of singlefrequency/narrow linewidth fiber laser systems. A single-mode linearly polarized all-fiber amplifier system is set up to investigate SBS effect in triple-frequency high-power amplifiers. With this amplifier, up to 302 W output power with 83% slope efficiency is achieved and the SBS threshold is scaled up to 12 d B. To the best of our knowledge, this is the highest output power of multifrequency laser from a single-mode polarization maintaining fiber. Good spectral properties and high brightness make this laser source available for the application of second harmonic generation, coherent beam combining.