Metabolic profiling reveals altered amino acid and fatty acid metabolism in children with Williams Syndrome.

Williams Syndrome (WS) is a rare neurodevelopmental disorder with a prevalence of 1 in 7500 to 1 in 20,000 individuals, caused by a microdeletion in chromosome 7q11.23. Despite its distinctive clinical features, the underlying metabolic alterations remain largely unexplored.

[Characteristics of early cognitive development in children with Williams syndrome: a prospective cohort study].

Objectives: To investigate the early cognitive development characteristics of children with Williams syndrome (WS) at different age stages.

Methods: From September 2018 to June 2023, 106 children diagnosed with WS at the Department of Pediatric Health Care, Children's Hospital, Zhejiang University School of Medicine, aged 1 to <5 years, were prospectively enrolled. All children underwent Gesell developmental diagnostic assessments to analyze the cognitive development characteristics of WS children across different age groups.

Health-related quality of life of children with Williams syndrome and caregivers in China.

Introduction: Williams syndrome (WS) is a rare genetic disorder that impacts multiple systems and may cause developmental delays. These medical and developmental issues impose a heavy burden on affected children and their families. However, there was no study on children's health-related quality of life (HRQoL) with WS and only two studies about family quality of life globally.

The Inhibition of Adipose-Derived Stem Cells on the Invasion of Keloid Fibroblasts.

Keloids represent the dysregulation of cutaneous wound healing caused by aberrant fibroblast activities. Adipose-derived stem cells have been recognized as a promising treatment for keloids. However, the molecular mechanisms have not been fully elucidated.

Clinical phenotypes study of 231 children with Williams syndrome in China: A single-center retrospective study.

Background: Williams syndrome (WS) is a multisystem neurodevelopmental disorder caused by microdeletions in 7q11.23. This study aims to characterize the clinical phenotypes of Chinese children with WS to help for the early diagnosis and intervention of this disease.

[Early motor development in children with Williams syndrome].

Objectives: To study the early motor development of children with William syndrome (WS).

Methods: The medical data of 59 children with WS (40 males and 19 females) aged 0-24 months from September 2018 to August 2021 were retrospectively analyzed. Based on the test results of the Peabody Developmental Motor Scale II, the motor development ability of the children of different ages was analyzed.

Analysis of The Working Performance of Large Curvature Prestressed Concrete Box Girder Bridges.

Based on numerical shape functions and the structural stressing state theory, the mechanical properties of the curved prestressed concrete box girder (CPCBG) bridge model under different loading cases are presented. First, the generalized strain energy density () obtained from the measured strain data is used to represent the stressing state pattern of the structure; then, the stressing state of the concrete section is analyzed by plotting the strain and stress fields of the bridge model. The stressing state pattern and strain fields of the CPCBG are shown to reveal its mechanical properties.

A Research on Developmental Characteristics of Children With Language Delay in Zhejiang Province, China.

We used Sign-significant relations (S-S) to assess the developmental characteristics of 1- to 4-year-old children with language delays in Zhejiang Province and to provide scientific basis for early clinical detection and comprehensive intervention. A total of 1,113 children among the ages of 1 and 4 who complained of poor language skills were assessed in language competence using S-S. These children diagnosed with language delays were divided into six groups, with each group having an age difference of 6 months.

Indirect Regulation and Equilibrium of p35 and p40 Subunits of Interleukin (IL)-12/23 by Ustekinumab in Psoriasis Treatment.

BACKGROUND Ustekinumab, a human-derived monoclonal antibody that targets the p40 subunit of interleukin (IL)-12 and IL-23, has excellent clinical efficacy and safety in treating psoriasis, with a long half-life. However, no reports have described the use of human skin/serum samples to elucidate its molecular mechanisms. MATERIAL AND METHODS Twenty-four psoriasis patients were enrolled in our double-blind study and randomly divided into placebo and ustekinumab-administered groups.

Engineering Properties of Engineered Cementitious Composite and Multi-Response Optimization Using PCA-Based Taguchi Method.

The engineered cementitious composite (ECC) mixtures were prepared with Portland cement, ground fly ash, silica sand, and polyvinyl alcohol (PVA) fibers. Accordingly, four mix design factors with five levels each were designed using the Taguchi method. The engineering properties of ECC (flow expansion, compressive strength, flexural strength, charge passed, and maximum freeze-thaw cycle) were evaluated, and the single-response optimizations were conducted separately.

Stressing State Analysis of an Integral Abutment Curved Box-Girder Bridge Model.

This paper experimentally investigates the working behavior characteristics of an integral abutment curved box-girder (IACBG) bridge model based on the structural stressing state theory. First, the stressing state of the bridge model is represented by generalized strain energy density (GSED) values at each load and characterized by the normalized GSED sum . Then, the Mann-Kendall (M-K) criterion is adopted to detect the stressing state mutations of the bridge model from - curve in order to achieve the new definition of structural failure load.

Investigation on the Failure Behavior of Engineered Cementitious Composites under Freeze-Thaw Cycles.

This paper investigates the freeze-thaw performance of engineered cementitious composites (ECC) reinforced with polyvinyl alcohol (PVA) fibers, by applying an innovative criterion for judging the specimen's working state mutation. The ECC materials are prepared into 25 mixtures using the Taguchi method. Then, the fundamental transverse frequency, the flexural performance and the internal strain variation of ECC specimens subjected to freeze-thaw cycles are measured.

Analysis of plasma proteome from cases of the different traditional Chinese medicine syndromes in patients with chronic hepatitis B.

A proteomic analysis method, two dimensional gel electrophoresis (2-DE) followed by matrix-assisted laser desorption/ionization time-of-flight MS (MALDI-TOF-MS), was used to explore the link between plasma proteome and the different syndromes of traditional Chinese medicine (TCM) in patients with chronic hepatitis B (CHB). In compared with the plasma proteomes from health donors, the alterations in protein expression from cases of the five TCM syndromes, including damp heat stasis in the middle-Jiao syndrome, liver Qi stagnation and spleen deficiency syndrome, spleen and kidney Yang deficiency syndrome, liver and kidney Yin deficiency syndrome, and blood stasis into collateral syndrome with CHB were identified (P<0.05).