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.
View Article and Find Full Text PDFWilliams syndrome (WS; OMIM#194050) is a rare disorder, which is caused by the microdeletion of one copy of 25-27 genes, and WS patients display diverse neuronal deficits. Although remarkable progresses have been achieved, the mechanisms for these distinct deficits are still largely unknown. Here, we have shown that neural progenitor cells (NPCs) in WS forebrain organoids display abnormal proliferation and differentiation capabilities, and synapse formation.
View Article and Find Full Text PDFZhongguo Dang Dai Er Ke Za Zhi
October 2024
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.
Influenza A virus subtype H1N1 can cause severe acute respiratory distress syndrome and death in young children and elderly individuals. H1N1 initiates inflammatory responses that aim to contain and eliminate microbial invaders. Various lipid mediators (LMs) are biosynthesized and play a critical role in fighting viruses during inflammation; thus, by profiling the LMs in patients, researchers can obtain mechanistic insights into diseases, such as the pathways disrupted.
View Article and Find Full Text PDFThe waning of maternal antibodies may cause infants to lose protection against measles before receiving measles-containing vaccine (MCV). The aim of this study is to investigate the changing characteristics and influencing factors of measles antibodies in preterm infants (PT), and to provide scientific basis for optimizing MCV vaccination strategy of the target population. Blood samples were collected from PT and full-term infants (FT) at the chronological age (CA) of 3, 6, and 12 months.
View Article and Find Full Text PDFBackground: peripheral neuroblastic tumors (pNT) have high incidence and mortality, and infants are prone to various infectious diseases. The purpose of this study is to understand the immunization status of children with pNT in the real-world and the incidence of adverse reactions after vaccination, and to evaluate the feasibility of vaccination and the influencing factors of vaccination.
Methods: Children with pNT treated in the Children's Hospital Affiliated to Zhejiang University from January 1, 2011 to December 1, 2021 were included.
Front Public Health
June 2023
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.
View Article and Find Full Text PDFBackground: There is a high incidence and mortality rate in children with hematologic tumors (CHT), who are more prone to various infectious diseases. This study aims to clarify the real-world National Immunization Program (NIP) vaccination status of CHT before and after chemotherapy.
Methods: Medical records, NIP vaccination data, and the Adverse Event Following Immunization (AEFI) of CHT who were admitted to the Children's Hospital, Zhejiang University School of Medicine, from 1 January 2011 to 1 December 2021 were completely collected.
Background: This study aims to investigate the serum fat-soluble vitamins A, D, and E levels of children in Zhejiang Province, and to provide a reference range of fat-soluble vitamins for children in Zhejiang Province.
Methods: Between May 2019 and December 2019, 871 children who sent peripheral blood samples to Hangzhou Biozon Medical Institute Co., Ltd.
Background: The prevailing consensus from large epidemiological studies is that breastfeeding is associated with improved IQ and cognitive functioning in later childhood and adolescence. Current research is exploring the association between breastfeeding and early brain development in preterm infants.
Objective: To explore the differences in brain gray matter between breastmilk-fed and formula-fed preterm infants using structural and functional magnetic resonance imaging.
The quantitative identification and zoning management of land use functions (LUFs) are important starting points for solving the problems of resource allocation and sustainable development. In this study, with the Yangtze River Economic Belt (YREB) as the case study area, LUFs were grouped into three primary categories: economic function (ENF), social function (SCF), and ecological function (ELF). The least square error model was adopted to identify the morphological changes of LUFs.
View Article and Find Full Text PDFBackground: 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.
View Article and Find Full Text PDFCities are divided into different levels around the world, which is manifested as cities are managed according to the urban administrative level system in China, urban development boundary (UDB) has always been an spatial policy to limit the scope of urban sprawl. There is a lack of comparative studies on LUCC and influencing factors inside the UDBs of different level cities. Based on the methods of descriptive statistical analysis, transition matrix analysis, Pearson's correlation analysis, and OLS regression, this paper takes Hubei Province, China as an example to study the statistical regularities of LUCC and its influencing factors within the UDBs of different level cities from 2006 to 2020.
View Article and Find Full Text PDFZhongguo Dang Dai Er Ke Za Zhi
September 2022
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.
Association fibers connect the cortical regions and experience rapid development involving myelination and axonal growth during infancy. Yet, the spatiotemporal patterns of microstructural changes along these tracts, as well as the developmental interaction between the white matter (WM) tracts and the cortical gray matter (cGM) connected to them, are mostly unknown during infancy. In this study, we performed a diffusion MRI-based tractography and microstructure study in a cohort of 89 healthy preterm-born infants with gestational age at birth between 28.
View Article and Find Full Text PDFExuberant axon growth and competitive pruning lead to dramatic and comprehensive changes in white matter pathways of the infant brain during the first few postnatal months, yet the development of structural configuration in early infancy has not been fully characterized. This study aimed to investigate the developmental trajectory of structural connectivity reflecting relative fiber density in 43 preterm-born infants aged 0-3 months of corrected age without any complications utilizing probabilistic tractography based on fiber orientation distribution and to explore the potential function correlation associated with the network properties based on the Chinese Communication Development of Infant at 10 months of corrected age. The findings revealed significant increases in global efficiency, local efficiency, normalized clustering coefficient, and small-worldness ( < 0.
View Article and Find Full Text PDFEnviron Sci Pollut Res Int
July 2022
Research on construction land use efficiency with carbon emissions provides valuable insights for effective regulation of land use planning and low-carbon development. This study explores construction land use efficiency (CLUE) in China and the USA by employing a slack-based measure (SBM) that incorporates carbon emissions as undesirable output to evaluate CLUE at the provincial/state level. The abatement potential (AP) of construction land use and carbon emissions through the land utilization process are further explored.
View Article and Find Full Text PDFInt J Environ Res Public Health
December 2021
Exploring the interaction relationship between urbanization and ecological security is the key issue to achieve regional sustainable development. This study used coupling coordination model and vector auto-regression model to comprehensively investigate the interaction relationship between urbanization and ecological security in Wuhan City Circle from 2005 to 2018. The results showed that urbanization quality in Wuhan City Circle increased from 0.
View Article and Find Full Text PDFInt J Environ Res Public Health
November 2021
With the rapid urbanization in recent decades, resource shortage and environmental damage have hindered the process of urban sustainable development (SD). As a yardstick of sustainable development, the evaluation of resources and environment carrying capacity (RECC) and its decoupling relationship with social comprehensive development index (SCDI) are of great significance. In this paper, RECC and SCDI are taken as research objects to establish resource and environment system evaluation index system and social comprehensive development level evaluation index system, respectively.
View Article and Find Full Text PDFThe aim of the study is to demonstrate the characteristic of motor development and MRI changes of related brain regions in preterm infants with different iron statuses and to determine whether the daily iron supplementation can promote motor development for preterm in early infancy. The 63 preterm infants were grouped into non-anemia with higher serum ferritin (NA-HF) group and anemia with lower serum ferritin (A-LF) group according to their lowest serum Hb level in the neonatal period as well as the sFer at 3 months old. Forty-nine participants underwent MRI scans and Infant Neurological International Battery (INFANIB) at their 3 months.
View Article and Find Full Text PDFObjectives: Delayed vaccination in children with seizures was common in China. This study aims to describe the vaccination status, reasons for vaccination deferral, vaccination recommendations and the safety for these patients in Zhejiang.
Methods: 1539 children included were divided into febrile seizure (FS) group, epilepsy (EP) group, unclassified seizure group and other symptomatic seizure group.
The human brain demonstrates anatomical and functional lateralization/asymmetry between the left and right hemispheres, and such asymmetry is known to start from the early age of life. However, how the asymmetry changes with brain development during infancy remained unknown. In this study, we aimed to systematically investigate the spatiotemporal pattern of brain asymmetry in healthy preterm-born infants during the first-half-year of development, using high angular resolution diffusion MRI.
View Article and Find Full Text PDFThe aim of this study was to investigate the effects of unified iron supplementation and identify the factors related to the iron homeostasis among preterm infants. A total of 250 preterm infants were divided into neonatal anemic (NA, = 154) and non-neonatal anemic group (NNA, = 96). Iron supplements at a dose of 2 mg/kg per day were given from 40 weeks' gestational age to 6 months.
View Article and Find Full Text PDFThere has been a considerable controversy about vaccination practices in Children with congenital heart disease (CHD) in China. This study aims to identify the reasons for deferring vaccination among the patient population attending the Vaccination Consultation Clinic in Zhejiang Province and the safety of their vaccination. We analyzed the data of 2442 children with CHD, who visited to our clinic from January 2016 to March 2019.
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