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Abstract Prader-Willi syndrome (PWS) is a complex and multisystem neurobehavioral disease, which is caused by the lack of expression of paternally inherited imprinted genes on chromosome15q11.2-q13.1. The clinical manifestations of PWS vary with age. It is characterized by severe hypotonia with poor suck and feeding difficulties in the early infancy, followed by overeating in late infancy or early childhood and progressive development of morbid obesity unless the diet is externally controlled. Compared to Western PWS patients, Chinese patients have a higher ratio of deletion type. Although some rare disease networks, including PWS Cooperation Group of Rare Diseases Branch of Chinese Pediatric Society, Zhejiang Expert Group for PWS, were established recently, misdiagnosis, missed diagnosis and inappropriate intervention were usually noted in China. Therefore, there is an urgent need for an integrated multidisciplinary approach to facilitate early diagnosis and optimize management to improve quality of life, prevent complications, and prolong life expectancy. Our purpose is to evaluate the current literature and evidences on diagnosis and management of PWS in order to provide evidence-based guidelines for this disease, specially from China.

As an important infrastructure, the floating platform plays a significant role in coastal and oceanic development. The single‐point mooring system anchored by piles has been widely used to support floating offshore platforms. However, few studies in the field of floating structures focused on the dynamic reliability and mechanical analysis of the mooring pile foundation under wind and wave vibration. A Monte Carlo‐based mooring pile reliability analysis method was introduced in this paper, which could consume the calculation source significantly. Based on the Monte Carlo simulation, the dynamic reliability of the mooring pile system of the floating platform was analyzed and discussed. Finally, considering the material cost, the design of the mooring pile of floating platform was optimized. The simulation work presented here also will contribute to a deeper understanding of civil engineering works' pile foundation. [ABSTRACT FROM AUTHOR]

Background: Axial spondyloarthritis (axSpA) is a group of inflammatory diseases that may lead to ankylosis of the sacroiliac joint and spine. Fat lesion in the sacroiliac joint is an important feature in diagnosis and disease progression of axSpA. However, whether there is alteration of fatty acids (FAs) composition has not been investigated using MRI. Purpose: To investigate bone marrow FA composition of the sacroiliac joint in patients with axSpA compared to controls. Study Type: Prospective. Subjects: Eighty five participants (mean age, 32.3 ± 6.1 years): 48 axSpA (25 male, 23 female) and 37 non‐SpA controls (18 male, 19 female). Field Strength/Sequence: 3.0 T/Two multiple gradient‐echo chemical shift‐encoded (CSE) MRI which differed only in echo times (TEs) were scanned consecutively. Assessment: Axial multi‐echo CSE MRI was performed in the sacroiliac joints in vivo. Regions of interest (ROIs) were manually placed on subchondral bone with and without fat lesion in axSpA patients, and on subchondral bone without fat lesion in controls. FA composition was computed within the ROIs using a nonlinear least square method from literature. Statistical Tests: Intergroup comparisons were performed using t tests. Results: In axSpA, male patients had significantly higher monounsaturated FA compared to controls in areas with fat lesion in the sacrum (+12%) and in the ilium (+9%), and in areas without fat lesion in the sacrum (+10%). Significantly lower polyunsaturated FAs were found in areas with fat lesion in the sacrum (−10%) and ilium (−11%), and lower saturated FAs were found in areas without fat lesion in the sacrum (−6%). In female, patients with axSpA had significantly higher saturated FAs in areas with fat lesion in the ilium (+7%) in comparison to controls. Data Conclusion: FA composition of the sacroiliac joint alters in patients with axSpA, and it can be detected using CSE MRI based analysis. [ABSTRACT FROM AUTHOR]

The fault diagnosis of rotating machinery is vital in industry but traditionally depends on manual expertise, requiring substantial resources. To improve diagnostic accuracy, enable effective condition monitoring, and minimize the impact of faults on operations, advanced diagnostic techniques are essential. Hence, we propose an advanced fault diagnosis framework that leverages improved particle swarm optimization (IPSO), variational mode decomposition (VMD), and probabilistic neural networks (PNN) to accurately diagnose faults in rotating machinery using gear and rolling bearing vibration signals. Initially, the vibration signals are decomposed into intrinsic mode functions via VMD, enabling the capture of subtle but critical fault features. To address parameter selection challenges in VMD, we employed IPSO to optimize the VMD parameters, ensuring the optimal decomposition effect. Further, we refined the feature set by applying Laplace fraction optimization and feature dimensionality reduction, isolating sensitive features that serve as input to a PNN-based fault classification model. Experimental results demonstrated that this IPSO-VMD-PNN framework achieves high diagnostic accuracy for various fault types, establishing it as an effective tool for fault identification in rotating machinery. [ABSTRACT FROM AUTHOR]

The impact of urban traffic on human health is significant. This research conducts field measurements in Guangzhou, China, focusing on a building situated near subgrade roads and viaducts to investigate the characteristics of airborne and structure-borne noise generated by these infrastructures. The analysis involves the use of both sound pressure level and overall sound pressure level, as well as an examination of the transfer function between outdoor and indoor noise levels. The findings indicate that traffic-related airborne noise demonstrates a characteristic frequency at 1000 Hz in this scenario, while viaduct- and building-generated structure-borne noise is predominantly distributed at lower frequencies. Additionally, it is worth noting that structural vibrations generate significantly less energy compared to airborne traffic noise sources. The variation in outdoor road noise across different floors over the entire frequency range demonstrates an initial increase followed by a decrease with rising floor height due to air damping effects as well as sound barriers' attenuation properties. These results enhance engineers' understanding of urban traffic-induced airborne or structure-borne noise while establishing foundational data for designing layouts integrating urban buildings with roads. [ABSTRACT FROM AUTHOR]

This paper presents a summary of results of an experimental investigation to assess the behavior of shear connectors encased in concrete-filled steel cells (CFSCs) that are used in steel-concrete joints with ultra-high performance concrete (UHPC) grout. In this study, a total of ten push-out specimens: including two plug-in type specimens and eight CFSC type specimens have been conducted. The slip between the steel plate and concrete and the strains for the steel studs and transverse rebar were measured and analyzed. Results obtained from the present and previously reported experiments showed that the average ultimate and residual bond strengths at the steel/concrete interface increased by 20% and 36%, respectively, as compared with specimens without confinement. Furthermore, experimental results indicated that providing a steel cell increased the ultimate strength of PBL in CFSC by 29%, as compared with unconstrained counterparts. The performance of the steel studs and transverse steel rebar were barely influenced by steel cells confinement. In comparison with the cases of shear connectors singly used, the interactions between the steel studs and PBL in CFSC decreased the shear capacity of the steel studs by 18% and increased that of PBL by 6%. A comparison between experimental and analytical results obtained from existing formulations indicated that the PBL shear capacity with confinement was underestimated when using analytical formulations that do not consider the confinement effects.