Bone growth and development is a major determinant of height.Height development in children is the result of the proliferation and endochondral ossification of growth plate chondrocytes located at the ends of long bones and vertebrae.Short stature，also known as dwarfism，means that a child's height is less than two standard deviations of the normal interval for children of the same race，region，and sex，or below the 3rd percentile of the normal child growth curve，which is a common problem in pediatric endocrinology.A variety of physiological regulatory pathways involving growth plate and long bone development have been reported，and related gene defects may be the key mechanisms leading to growth disorders and abnormal skeletal development in children.The ACAN gene，which is associated with osteochondral dysplasia，has garnered widespread attention from numerous scholars in recent years.This article reviews the genetic etiology，clinical phenotype，treatment and management of short stature caused by ACAN gene mutation in children.

Objective:To explore the application effect of multidisciplinary graded nutritional intervention in patients with cervical cancer undergoing radiochemotherapy.Methods:A convenience sampling method was used to select 160 patients with cervical cancer who received radiochemotherapy in the First Affiliated Hospital of Wenzhou Medical University from June 2022 to June 2024. According to the order of admission, 80 patients admitted between June 2022 and June 2023 were assigned to the control group, and 80 patients admitted between July 2023 and June 2024 were assigned to the observation group. The control group received routine nutritional intervention, while the observation group received multidisciplinary graded nutritional intervention. Serum albumin, prealbumin, hemoglobin, body mass index (BMI), and Functional Assessment of Cancer Therapy-General (FACT-G) scores were compared between the two groups before and after intervention.Results:Before the intervention, there were no statistically significant differences between the two groups in serum albumin, prealbumin, hemoglobin, BMI, and FACT-G dimension scores ( P>0.05). After the intervention, serum albumin, prealbumin, hemoglobin, BMI, and FACT-G dimension scores were all higher in the observation group than in the control group, and the differences were statistically significant ( P<0.05) . Conclusions:Multidisciplinary graded nutritional intervention can improve the nutritional status and quality of life of patients with cervical cancer undergoing radiochemotherapy, and is worthy of wider application.

Objective:To explore the application effect of multidisciplinary graded nutritional intervention in patients with cervical cancer undergoing radiochemotherapy.Methods:A convenience sampling method was used to select 160 patients with cervical cancer who received radiochemotherapy in the First Affiliated Hospital of Wenzhou Medical University from June 2022 to June 2024. According to the order of admission, 80 patients admitted between June 2022 and June 2023 were assigned to the control group, and 80 patients admitted between July 2023 and June 2024 were assigned to the observation group. The control group received routine nutritional intervention, while the observation group received multidisciplinary graded nutritional intervention. Serum albumin, prealbumin, hemoglobin, body mass index (BMI), and Functional Assessment of Cancer Therapy-General (FACT-G) scores were compared between the two groups before and after intervention.Results:Before the intervention, there were no statistically significant differences between the two groups in serum albumin, prealbumin, hemoglobin, BMI, and FACT-G dimension scores ( P>0.05). After the intervention, serum albumin, prealbumin, hemoglobin, BMI, and FACT-G dimension scores were all higher in the observation group than in the control group, and the differences were statistically significant ( P<0.05) . Conclusions:Multidisciplinary graded nutritional intervention can improve the nutritional status and quality of life of patients with cervical cancer undergoing radiochemotherapy, and is worthy of wider application.

【Objective】 To understand the current situation of social emotional competence of infants and toddlers, and analyze its relationship with home rearing environment, in order to provide the basis for improving the level of infant social emotional development. 【Method】 A study was conducted on 390 individuals from a child health institution in Hubei and Henan provinces.The "Infant and Toddler Social-Emotional Assessment Scale" and "1 - 3 Years Child Home Rearing Environment Scale" were used to investigate the social emotions ability and home rearing environment of infants and toddlers. 【Results】 A total of 390 valid questionnaires were collected in this survey, of which 199 were boys (51.0%) and 191 girls (49.0%). The average age of infants was (27.13±6.86)months.The age distribution is mainly among infants and young children aged 24 to 36 months, with a total of 305 people (78.2%). The caregiver′s registered residence (Z=-3.570), father′s education level (H=17.106), mother′s education level (H=7.980), per capita monthly income of the family (H=13.986), and the home rearing environment (Z=-8.881) had statistical significance on the social emotional competence of infants(P<0.05 or <0.01).There was a significant positive correlation between family rearing environment and infants′ social emotion (r=0.582, P<0.01).Multiple regression analysis showed that the social adaptation/self-care(β=0.30, 95%CI: 0.18 - 0.52, P<0.01)and language cognition dimensions(β=0.22, 95%CI: 0.07 - 0.59, P<0.05) in the home rearing environment had a statistically significant impact on the social emotional ability of infants and toddlers. 【Conclusion】 The home rearing environment is closely related to the social emotional development of infants and young children.Improve the parenting knowledge and skills of the main caregivers of infants and young children, build a good family rearing environment for infants and young children, which is beneficial to promote the development of children′s social emotions.

The typical hallmark of tumor evolution is metabolic dysregulation. In addition to secreting immunoregulatory metabolites, tumor cells and various immune cells display different metabolic pathways and plasticity. Harnessing the metabolic differences to reduce the tumor and immunosuppressive cells while enhancing the activity of positive immunoregulatory cells is a promising strategy. We develop a nanoplatform (CLCeMOF) based on cerium metal-organic framework (CeMOF) by lactate oxidase (LOX) modification and glutaminase inhibitor (CB839) loading. The cascade catalytic reactions induced by CLCeMOF generate reactive oxygen species "storm" to elicit immune responses. Meanwhile, LOX-mediated metabolite lactate exhaustion relieves the immunosuppressive tumor microenvironment, preparing the ground for intracellular regulation. Most noticeably, the immunometabolic checkpoint blockade therapy, as a result of glutamine antagonism, is exploited for overall cell mobilization. It is found that CLCeMOF inhibited glutamine metabolism-dependent cells (tumor cells, immunosuppressive cells, etc.), increased infiltration of dendritic cells, and especially reprogrammed CD8⁺ T lymphocytes with considerable metabolic flexibility toward a highly activated, long-lived, and memory-like phenotype. Such an idea intervenes both metabolite (lactate) and cellular metabolic pathway, which essentially alters overall cell fates toward the desired situation. Collectively, the metabolic intervention strategy is bound to break the evolutionary adaptability of tumors for reinforced immunotherapy.

Objective:To survey the awareness status and its influencing factors among parents of preschool children.Methods:Data was collected from the National Nutrition and Health Systematic Survey for 0-18 Year Children in China. A total of 2 625 children aged 3-5 years and their parents living in northern and southern regions of China were randomly selected by multi-stage stratified cluster sampling. The physical measurements were performed for the children, including height and weight; and the body mass index (BMI) and age-for-BMI Z-score (BAZ) were calculated. The questionnaire survey was conducted for the parents, including the demographic characteristics of parents and their children, the perception of their child′s nutritional status (overweight/obesity, normal, wasting) etc. According to WHO standards, the nutritional status of the children was classified as wasting, normal and overweight/obesity. The awareness of parents on their children′s nutritional status was classified as underestimated, correct, overestimated and unclear. Parents′ inability to correctly judge children′s nutritional status was defined as cognitive bias, including underestimation bias and overestimation bias. Multivariate logistic regression model was used to analyze the influencing factors of parents′ underestimation or overestimation of children′s nutritional status.Results:Among 2 625 enrolled preschool children, there were 1 312 boys (50.0%) and 1 313 girls (50.0%); and 648 (24.7%), 944 (36.0%) and 1 033 (39.3%) children aged 3, 4 and 5 years, respectively. One parent (mother, farther or others) of each child was selected for survey, and most of them were mothers (1 998(76.1%)). The prevalence rate of overweight/obesity and wasting was 10.3% (270/2 625) and 1.4% (38/2 625), respectively; and 2 317 children (88.3%) were normal. Among all parents surveyed, 1 766 (67.3%) were correct about their children′s nutritional status (correct group), 612 (23.3%) underestimated their children′s nutritional status (underestimated group), 213 (8.1%) overestimated their children′s nutritional status (overestimated group) and 34 (1.3%) were not aware of their children′s nutritional status (unclear group). The multivariate logistic regression analysis showed that girls ( OR=0.817, 95% CI: 0.678-0.984), living in rural areas ( OR=0.801, 95% CI: 0.662-0.969), large birth weight of child ( OR=0.639, 95% CI: 0.420-0.970) were protective factors for parents underestimating children′s nutritional status. Living in the northern region ( OR=1.698, 95% CI: 1.260-2.290), large birth weight of children ( OR=1.826, 95% CI: 1.149-2.902), father with overweight/obesity ( OR=1.467, 95% CI: 1.089-1.977) and maternal overweight/obesity ( OR=1.778, 95% CI: 1.308-2.417) were the risk factors for parents to overestimate the nutritional status of children. Conclusions:The survey shows that parents of preschool children have a relatively high cognitive bias on the nutritional status of their children. Parents of girls, living in rural areas or having child with large birth weight are less likely to underestimate the nutritional status of children; parents living in northern regions, having a child with large birth weight, or with overweight/obese are likely to overestimate the nutritional status of children.

Objective: To analyze the clinical epidemiological characteristics including composition of pathogens , clinical characteristics, and disease prognosis acute bacterial meningitis (ABM) in Chinese children. Methods: A retrospective analysis was performed on the clinical and laboratory data of 1 610 children <15 years of age with ABM in 33 tertiary hospitals in China from January 2019 to December 2020. Patients were divided into different groups according to age,<28 days group, 28 days to <3 months group, 3 months to <1 year group, 1-<5 years of age group, 5-<15 years of age group; etiology confirmed group and clinically diagnosed group according to etiology diagnosis. Non-numeric variables were analyzed with the Chi-square test or Fisher's exact test, while non-normal distrituction numeric variables were compared with nonparametric test. Results: Among 1 610 children with ABM, 955 were male and 650 were female (5 cases were not provided with gender information), and the age of onset was 1.5 (0.5, 5.5) months. There were 588 cases age from <28 days, 462 cases age from 28 days to <3 months, 302 cases age from 3 months to <1 year of age group, 156 cases in the 1-<5 years of age and 101 cases in the 5-<15 years of age. The detection rates were 38.8% (95/245) and 31.5% (70/222) of Escherichia coli and 27.8% (68/245) and 35.1% (78/222) of Streptococcus agalactiae in infants younger than 28 days of age and 28 days to 3 months of age; the detection rates of Streptococcus pneumonia, Escherichia coli, and Streptococcus agalactiae were 34.3% (61/178), 14.0% (25/178) and 13.5% (24/178) in the 3 months of age to <1 year of age group; the dominant pathogens were Streptococcus pneumoniae and the detection rate were 67.9% (74/109) and 44.4% (16/36) in the 1-<5 years of age and 5-<15 years of age . There were 9.7% (19/195) strains of Escherichia coli producing ultra-broad-spectrum β-lactamases. The positive rates of cerebrospinal fluid (CSF) culture and blood culture were 32.2% (515/1 598) and 25.0% (400/1 598), while 38.2% (126/330)and 25.3% (21/83) in CSF metagenomics next generation sequencing and Streptococcus pneumoniae antigen detection. There were 4.3% (32/790) cases of which CSF white blood cell counts were normal in etiology confirmed group. Among 1 610 children with ABM, main intracranial imaging complications were subdural effusion and (or) empyema in 349 cases (21.7%), hydrocephalus in 233 cases (14.5%), brain abscess in 178 cases (11.1%), and other cerebrovascular diseases, including encephalomalacia, cerebral infarction, and encephalatrophy, in 174 cases (10.8%). Among the 166 cases (10.3%) with unfavorable outcome, 32 cases (2.0%) died among whom 24 cases died before 1 year of age, and 37 cases (2.3%) had recurrence among whom 25 cases had recurrence within 3 weeks. The incidences of subdural effusion and (or) empyema, brain abscess and ependymitis in the etiology confirmed group were significantly higher than those in the clinically diagnosed group (26.2% (207/790) vs. 17.3% (142/820), 13.0% (103/790) vs. 9.1% (75/820), 4.6% (36/790) vs. 2.7% (22/820), χ²=18.71, 6.20, 4.07, all P<0.05), but there was no significant difference in the unfavorable outcomes, mortility, and recurrence between these 2 groups (all P>0.05). Conclusions: The onset age of ABM in children is usually within 1 year of age, especially <3 months. The common pathogens in infants <3 months of age are Escherichia coli and Streptococcus agalactiae, and the dominant pathogen in infant ≥3 months is Streptococcus pneumoniae. Subdural effusion and (or) empyema and hydrocephalus are common complications. ABM should not be excluded even if CSF white blood cell counts is within normal range. Standardized bacteriological examination should be paid more attention to increase the pathogenic detection rate. Non-culture CSF detection methods may facilitate the pathogenic diagnosis.
Adolescent ; Brain Abscess ; Child ; Child, Preschool ; Escherichia coli ; Female ; Humans ; Hydrocephalus ; Infant ; Infant, Newborn ; Male ; Meningitis, Bacterial/epidemiology* ; Retrospective Studies ; Streptococcus agalactiae ; Streptococcus pneumoniae ; Subdural Effusion ; beta-Lactamases

Child ; Consensus ; Drugs, Chinese Herbal ; Humans ; Medicine, Chinese Traditional ; Respiratory System

Objective: To explore the heterogeneity and correlation of clinical phenotypes and genotypes in children with disorders of sex development (DSD). Methods: A retrospective study of 1 235 patients with clinically proposed DSD in 36 pediatric medical institutions across the country from January 2017 to May 2021. After capturing 277 DSD-related candidate genes, second-generation sequencing was performed to analyzed the heterogeneity and correlation combined with clinical phenotypes. Results: Among 1 235 children with clinically proposed DSD, 980 were males and 255 were females of social gender at the time of initial diagnosis with the age ranged from 1 day of age to 17.92 years. A total of 443 children with pathogenic variants were detected through molecular genetic studies, with a positive detection rate of 35.9%. The most common clinical phenotypes were micropenis (455 cases), hypospadias (321 cases), and cryptorchidism (172 cases) and common mutations detected were in SRD5A2 gene (80 cases), AR gene (53 cases) and CYP21A2 gene (44 cases). Among them, the SRD5A2 mutation is the most common in children with simple micropenis and simple hypospadias, while the AMH mutation is the most common in children with simple cryptorchidism. Conclusions: The SRD5A2 mutation is the most common genetic variant in Chinese children with DSD, and micropenis, cryptorchidism, and hypospadias are the most common clinical phenotypes. Molecular diagnosis can provide clues about the biological basis of DSD, and can also guide clinicians to perform specific clinical examinations. Target sequence capture probes and next-generation sequencing technology can provide effective and economical genetic diagnosis for children with DSD.
3-Oxo-5-alpha-Steroid 4-Dehydrogenase/genetics* ; Child ; China/epidemiology* ; Cryptorchidism/genetics* ; Disorders of Sex Development/genetics* ; Female ; Genital Diseases, Male ; Genotype ; Humans ; Hypospadias/genetics* ; Male ; Membrane Proteins/genetics* ; Penis/abnormalities* ; Phenotype ; Retrospective Studies ; Steroid 21-Hydroxylase/genetics*

Objective: To investigate the values of serum 8-hydroxydeoxyguanosine (8-OHdG) in predicting disease progression and prognosis of patients with sepsis. Methods: The prospective observational research methods were used. A total of 124 patients with sepsis who met the inclusion criteria were admitted to the Department of Emergency of the First Affiliated Hospital of Wenzhou Medical University from April 2015 to July 2016, including 79 males and 45 females, aged (62±15) years. The sepsis-related organ failure assessment (SOFA) scores of all patients on admission and on the second day of admission and their difference (ΔSOFA) were calculated. The patients were divided into non-progression group with ΔSOFA score <2 (n=101) and progression group with ΔSOFA score ≥2 (n=23), and according to the survival during hospitalization, the patients were divided into survival group (n=85) and death group (n=39). Data of patients between non-progression group and progression group, survival group and death group were compared, including the gender, age, days in emergency intensive care unit (ICU), smoking, hypertension, diabetes mellitus, serum white blood cell count, serum C-reactive protein, and serum procalcitonin on admission, and serum 8-OHdG within 24 h of admission. The multivariate logistic regression analysis was used to screen the independent risk factors of disease progression and death during hospitalization in 124 patients with sepsis, the receiver's operating characteristic (ROC) curves were drawn according to the independent risk factors, and the area under the curve (AUC), the best threshold, and the sensitivity and specificity under the best threshold were calculated. The patients were divided into high 8-OHdG group (n=35) and low 8-OHdG group (n=89) according to the best threshold in ROC curve of death during hospitalization. The data including the gender, age, SOFA score on admission, SOFA score on the second day of admission, and ΔSOFA score of patients in the two groups were compared. The survival rates of patients within 90 d of admission in the two groups were compared by the Kaplan-Meier method. Data were statistically analyzed with independent sample t test, Mann-Whitney U test, chi-square test, and Log-rank test. Results: The gender, age, days in emergency ICU, smoking, complicated with hypertension, complicated with diabetes mellitus, serum white blood cell count, serum C-reactive protein, and serum procalcitonin on admission of patients in non-progression group and progression group were similar (P>0.05). The serum 8-OHdG within 24 h of admission of patients in progression group was significantly higher than that in non-progression group (Z=-2.31, P<0.05). Multivariate logistic regression analysis showed that the serum 8-OHdG within 24 h of admission was the independent risk factor for disease progression of 124 patients with sepsis (odds ratio=1.06, with 95% confidence interval of 1.01-1.11, P<0.05). The AUC under the ROC curve of serum 8-OHdG within 24 h of admission to predict disease progression of 124 patients with sepsis was 0.65 (with 95% confidence interval of 0.52-0.79, P<0.05), the optimal threshold was 32.88 ng/mL, and the sensitivity and specificity under the optimal threshold was 52.2% and 79.2%, respectively. The gender, age, days in emergency ICU, smoking, complicated with hypertension, complicated with diabetes mellitus, and serum white blood cell count, serum C-reactive protein, and serum procalcitonin on admission of patients in survival group and death group were similar (P>0.05). The serum 8-OHdG within 24 h of admission of patients in death group was significantly higher than that in survival group (Z=-2.37, P<0.05). Multivariate logistic regression analysis showed that the serum 8-OHdG within 24 h of admission was the independent risk factor for death of 124 patients with sepsis (odd ratio=1.04, with 95% confidence interval of 1.00-1.09, P<0.05). The AUC under the ROC curve of serum 8-OHdG within 24 h of admission to predict death of patients during hospitalization was 0.63 (with 95% confidence interval of 0.52-0.75, P<0.05), the optimal threshold was 32.43 ng/mL, the sensitivity and specificity under the optimal threshold was 51.3% and 84.7%, respectively. The gender and age of patients in high 8-OHdG group and low 8-OHdG group were similar (P>0.05). The SOFA score on admission, SOFA score on the second day of admission, and ΔSOFA score of patients in high 8-OHdG group were significantly higher than those in low 8-OHdG group (with Z values of -2.49, -3.01, and -2.64, respectively, P<0.05 or P<0.01). The survival rate within 90 d of admission of patients in low 8-OHdG group was significantly higher than that in high 8-OHdG group (χ²=14.57, P<0.01). Conclusions: Serum 8-OHdG level is an independent risk factor for disease progression and death in sepsis patients with limited ability for predicting disease progression and prognosis of sepsis of patients. The patients with higher serum 8-OHdG level have higher death risk within 90 d of admission.
8-Hydroxy-2'-Deoxyguanosine ; Aged ; Disease Progression ; Female ; Humans ; Male ; Middle Aged ; Prognosis ; ROC Curve ; Retrospective Studies ; Sepsis