The growth and development of children is an important stage for health, and its monitoringand intervention are related to the long-term development of individuals. The construction of a standardized and multi-dimensional database of pediatric growth and development-related diseases is an important basis for realizing precise diagnosis and treatment and health management. Based on the needs of clinical practice, this study proposes to establish a specialized database of pediatric growth and development-related diseases that integrates facial phenotypes and clinical diagnosis and treatment information. This study elaborates on the construction process, including data sources, data collection content, and the operation and management of the database; and proposes key points for quality control, including the establishment of quality control nodes, database construction standards, and a full-process quality control framework. The above ensure the integrity, logic and effectiveness of the data, so that the database can provide an objective basis for the screening and diagnosis of pediatric growth and development-related diseases. On the basis of scientific data management and strict quality control, the database will help reveal the patterns of children's growth and development, and promote the level of children's health management.

Background: s/Aims: Sarcomatoid hepatocellular carcinoma (HCC) is a rare histological subtype of HCC characterized by extremely poor prognosis; however, its molecular characterization has not been elucidated. Methods: In this study, we conducted an integrated multiomics study of whole-exome sequencing, RNA-seq, spatial transcriptome, and immunohistochemical analyses of 28 paired sarcomatoid tumor components and conventional HCC components from 10 patients with sarcomatoid HCC, in order to identify frequently altered genes, infer the tumor subclonal architectures, track the genomic evolution, and delineate the transcriptional characteristics of sarcomatoid HCCs. Results: Our results showed that the sarcomatoid HCCs had poor prognosis. The sarcomatoid tumor components and the conventional HCC components were derived from common ancestors, mostly accessing similar mutational processes. Clonal phylogenies demonstrated branched tumor evolution during sarcomatoid HCC development and progression. TP53 mutation commonly occurred at tumor initiation, whereas ARID2 mutation often occurred later. Transcriptome analyses revealed the epithelial–mesenchymal transition (EMT) and hypoxic phenotype in sarcomatoid tumor components, which were confirmed by immunohistochemical staining. Moreover, we identified ARID2 mutations in 70% (7/10) of patients with sarcomatoid HCC but only 1–5% of patients with non-sarcomatoid HCC. Biofunctional investigations revealed that inactivating mutation of ARID2 contributes to HCC growth and metastasis and induces EMT in a hypoxic microenvironment. Conclusions We offer a comprehensive description of the molecular basis for sarcomatoid HCC, and identify genomic alteration (ARID2 mutation) together with the tumor microenvironment (hypoxic microenvironment), that may contribute to the formation of the sarcomatoid tumor component through EMT, leading to sarcomatoid HCC development and progression.

Background: s/Aims: Sarcomatoid hepatocellular carcinoma (HCC) is a rare histological subtype of HCC characterized by extremely poor prognosis; however, its molecular characterization has not been elucidated. Methods: In this study, we conducted an integrated multiomics study of whole-exome sequencing, RNA-seq, spatial transcriptome, and immunohistochemical analyses of 28 paired sarcomatoid tumor components and conventional HCC components from 10 patients with sarcomatoid HCC, in order to identify frequently altered genes, infer the tumor subclonal architectures, track the genomic evolution, and delineate the transcriptional characteristics of sarcomatoid HCCs. Results: Our results showed that the sarcomatoid HCCs had poor prognosis. The sarcomatoid tumor components and the conventional HCC components were derived from common ancestors, mostly accessing similar mutational processes. Clonal phylogenies demonstrated branched tumor evolution during sarcomatoid HCC development and progression. TP53 mutation commonly occurred at tumor initiation, whereas ARID2 mutation often occurred later. Transcriptome analyses revealed the epithelial–mesenchymal transition (EMT) and hypoxic phenotype in sarcomatoid tumor components, which were confirmed by immunohistochemical staining. Moreover, we identified ARID2 mutations in 70% (7/10) of patients with sarcomatoid HCC but only 1–5% of patients with non-sarcomatoid HCC. Biofunctional investigations revealed that inactivating mutation of ARID2 contributes to HCC growth and metastasis and induces EMT in a hypoxic microenvironment. Conclusions We offer a comprehensive description of the molecular basis for sarcomatoid HCC, and identify genomic alteration (ARID2 mutation) together with the tumor microenvironment (hypoxic microenvironment), that may contribute to the formation of the sarcomatoid tumor component through EMT, leading to sarcomatoid HCC development and progression.

Background: s/Aims: Sarcomatoid hepatocellular carcinoma (HCC) is a rare histological subtype of HCC characterized by extremely poor prognosis; however, its molecular characterization has not been elucidated. Methods: In this study, we conducted an integrated multiomics study of whole-exome sequencing, RNA-seq, spatial transcriptome, and immunohistochemical analyses of 28 paired sarcomatoid tumor components and conventional HCC components from 10 patients with sarcomatoid HCC, in order to identify frequently altered genes, infer the tumor subclonal architectures, track the genomic evolution, and delineate the transcriptional characteristics of sarcomatoid HCCs. Results: Our results showed that the sarcomatoid HCCs had poor prognosis. The sarcomatoid tumor components and the conventional HCC components were derived from common ancestors, mostly accessing similar mutational processes. Clonal phylogenies demonstrated branched tumor evolution during sarcomatoid HCC development and progression. TP53 mutation commonly occurred at tumor initiation, whereas ARID2 mutation often occurred later. Transcriptome analyses revealed the epithelial–mesenchymal transition (EMT) and hypoxic phenotype in sarcomatoid tumor components, which were confirmed by immunohistochemical staining. Moreover, we identified ARID2 mutations in 70% (7/10) of patients with sarcomatoid HCC but only 1–5% of patients with non-sarcomatoid HCC. Biofunctional investigations revealed that inactivating mutation of ARID2 contributes to HCC growth and metastasis and induces EMT in a hypoxic microenvironment. Conclusions We offer a comprehensive description of the molecular basis for sarcomatoid HCC, and identify genomic alteration (ARID2 mutation) together with the tumor microenvironment (hypoxic microenvironment), that may contribute to the formation of the sarcomatoid tumor component through EMT, leading to sarcomatoid HCC development and progression.

Objective:To investigate the clinical efficacy of continuous veno-venous hemodia-filtration (CVVHDF) combined with hemoperfusion (HP) HA380 in the treatment of heat stroke patients with multiple organ dysfunction syndrome (MODS).Methods:A retrospective and observational study was conducted. A total of 15 patients with heat stroke combined with MODS who were admitted to the department of intensive care unit (ICU)of Suizhou Central Hospital/Hubei University of Medicine from July to September 2022 were selected as the study objects. All 15 patients were treated with CVVHDF combined with HA380 based on the comprehensive management strategy for severe illness. Organ function indicators [including total bilirubin (TBil), aspartate aminotransferase (AST), creatine kinase (CK), lactate dehydrogenase (LDH), creatinine (Cr), cardiac troponin T (cTnT), myoglobin (Myo), MB isoenzyme of creatine kinase (CK-MB), sequential organ failure assessment (SOFA)] and inflammatory indicators [including white blood cell count (WBC), neutrophil count (NEU), C-reactive protein (CRP), procalcitonin (PCT), and interleukin-6 (IL-6)] were collected. The improvements of the above indexes at admission, after the first HP, after the second HP, after the third HP, and on the 5th day of treatment were compared. Combined with the clinical outcome of patients, the comprehensive efficacy of CVVHDF combined with HA380 in the treatment of severe heat radiation disease was evaluated.Results:There were 10 males and 5 females among the 15 patients. The average age was (64.5±11.5) years old. There were 6 cases of classical heat stroke and 9 cases of exertional heat stroke. Glasgow coma scale (GCS) was 3-8 at admission; SOFA score was 9-17 within 12 hours after admission; acute physiology and chronic health evaluation Ⅱ(APACHEⅡ) was 25-45 within 24 hours after admission. After treatment, the IL-6 level and SOFA score gradually decreased, and there were significant differences in the decrease after the second HP compared to admission [IL-6 (ng/L): 48.37 (15.36, 113.03) vs. 221.90 (85.87, 425.90), SOFA: 8.3±3.3 vs. 11.1±2.4, both P < 0.05]. The PCT level reached its peak after the first HP [12.51 (6.07, 41.65) μg/L], and then gradually decreased, and the difference was statistically significant after the third HP [1.26 (0.82, 5.40) μg/L, P < 0.05]. Compared those at admission, Cr level significantly improved after the first HP (μmol/L: 66.94±25.57 vs. 110.80±31.13, P < 0.01), Myo significantly decreased after the second HP [μg/L: 490.90 (164.98, 768.05) vs. 3?000.00 (293.00, 3?000.00), P < 0.05], After the third HP, the CK level also showed significant improvement [U/L: 476.0 (413.0, 922.0) vs. 2?107.0 (729.0, 2?449.0), P < 0.05]. After CVVHDF combined with 3 times HP treatment, the patient's inflammatory response was gradually controlled and organ function gradually recovered. On the 5th day of the disease course, WBC, PCT and IL-6 levels were significantly improved compared to admission, and AST, CK, LDH, Cr, Myo, CK-MB, and SOFA score were significantly corrected compared with those on admission. The 24-hour survival rate of 15 patients was 86.67%, and the 24-hour, 7-day and 28-day survival rates were both as high as 73.33%. The average mechanical ventilation time of 11 surviving patients was (101.8±22.0) hours, the average continuous renal replacement therapy (CRRT) time was (58.8±11.0) hours, the average length of ICU stay was (6.3±1.0) days, and the average total hospitalization was (14.6±5.2) days. Conclusion:CVVHDF combined with HP HA380 in the treatment of heat stroke patients with MODS can effectively improve organ function and alleviate the inflammatory storm, which is an effective means to improve the rescue rate and reduce the mortality of severe heat stroke patients.

Objective:To evaluate the efficacy of visualized precise lung expansion for determining the intersegmental plane in thoracoscopic segmentectomy.Methods:Sixty-four American Society of Anesthesiologists Physical Status classification Ⅰ or Ⅱ patients, regardless of gender, aged 33-77 yr, with body mass index of 16-34 kg/m 2, undergoing elective thoracoscopic segmentectomy under general anesthesia, were included. They were preoperatively reconstructed with 3D CT bronchovascularization to reconstruct the pulmonary vasculature, bronchus, and the virtual intersegmental planes of the lungs. The patients were divided into 2 groups ( n=32 each) using a random number table method: visualized precise lung expansion group (group V) and modified expansion and atrophy group (group E). Group V used visualized precision lung expansion to determine the intersegmental planes, and group E used the modified expansion and atrophy method to determine the intersegmental planes. The intraoperative intersegmental plane determination time, target segmental bronchus identification and treatment time, anesthesia time, operation time, postoperative air leakage, pulmonary atelectasis, fever, occurrence of lung infection, postoperative 24 h drainage volume, drain removal time and hospitalization time were recorded in the two groups. Results:Compared with group E, the intersegmental plane determination time, target segment bronchial identification and treatment time, anesthesia time and operation time were significantly shortened in group V( P<0.05).There were no significant differences between groups in the 24 h postoperative drainage volume, drain removal time, hospitalization time or incidence of postoperative pulmonary complications ( P>0.05). Conclusions:Compared with the modified expansion and atrophy method, visualized precise lung expansion can effectively shorten the intersegmental plane determination time in thoracoscopic segmentectomy.

Objective To investigate the incidence rate,clinical characteristics,and prognosis of neonatal stroke in Shenzhen,China.Methods Led by Shenzhen Children's Hospital,the Shenzhen Neonatal Data Collaboration Network organized 21 institutions to collect 36 cases of neonatal stroke from January 2020 to December 2022.The incidence,clinical characteristics,treatment,and prognosis of neonatal stroke in Shenzhen were analyzed.Results The incidence rate of neonatal stroke in 21 hospitals from 2020 to 2022 was 1/15 137,1/6 060,and 1/7 704,respectively.Ischemic stroke accounted for 75％(27/36);boys accounted for 64％(23/36).Among the 36 neonates,31(86％)had disease onset within 3 days after birth,and 19(53％)had convulsion as the initial presentation.Cerebral MRI showed that 22 neonates(61％)had left cerebral infarction and 13(36％)had basal ganglia infarction.Magnetic resonance angiography was performed for 12 neonates,among whom 9(75％)had involvement of the middle cerebral artery.Electroencephalography was performed for 29 neonates,with sharp waves in 21 neonates(72％)and seizures in 10 neonates(34％).Symptomatic/supportive treatment varied across different hospitals.Neonatal Behavioral Neurological Assessment was performed for 12 neonates(33％,12/36),with a mean score of(32±4)points.The prognosis of 27 neonates was followed up to around 12 months of age,with 44％(12/27)of the neonates having a good prognosis.Conclusions Ischemic stroke is the main type of neonatal stroke,often with convulsions as the initial presentation,involvement of the middle cerebral artery,sharp waves on electroencephalography,and a relatively low neurodevelopment score.Symptomatic/supportive treatment is the main treatment method,and some neonates tend to have a poor prognosis.

Bone organoids can simulate the complex structure and function of the bone tissues, which makes them a frontier technology in organoid researches. Bone organoids show a tremendous potential of applications in bone disease modeling, bone injury repair, and medicine screening. Although advancements have been made so far in constructing bone organoids with functional structures like mineralization, bone marrow, trabecular bone, callus, woven bone, etc, the researches in this field are confronted with numerous challenges such as lack of standardized construction strategies and unified evaluation criteria, which limits their further promotion and application. To standardize researches in bone organoids, the Orthopedic Expert Committee of Geriatric Branch of Chinese Association of Gerontology and Geriatrics, the Youth Osteoporosis Group of Orthopedic Branch of Chinese Medical Association, the Osteoporosis Group of Orthopedic Surgeon Branch of Chinese Medical Doctor Association, and the Osteoporosis Committee of Shanghai Association of Integrated Traditional Chinese and Western Medicine organized related experts to formulate Expert consensus on the construction, evaluation, and application of bone organoids ( version 2024) based on an evidence-based approach. A total of 17 recommendations were put forth, aiming to standardize researches and clinical applications of bone organoids and enhance their value in scientific research and clinical practice.

【Objective】 To analyze the height growth of children aged 6 - 15 years in Nanning, Guangxi Zhuang Autonomous Region, so as to provide evidence for the assessment of local children′s height development. 【Methods】 A total of 25 225 children aged 6 - 15 years were selected to get their physical examination data from 41 primary schools in Nanning by stratified cluster sampling method in December 2021.Then the height data were compared with the current domestic standards. 【Results】 The average height of boys in Nanning was lower than the national standard before the age of 10 years and 7 months, and the gap with the national standard gradually narrowed after the age of 10 years and 7 months. The average height of boys in Nanning City exceeded the national standard between the age of 11 years and 1 month and 13 years and 6 months, and then lagged behind the national standard again after the age of 13 years and 7 months. The mean height of girls in Nanning City was lower than the national standard height in several age groups, and it was more obvious before the age of 9 years and 7 months. The proportion of height ≤-2s,≤-s,≥ +s and ≥+2s in boys aged 6 to 15 years in Nanning City fluctuated from 2.59% to 6.04%, 12.09% to 23.43%, 7.18% to 18.79% and 0.93% to 3.14%, respectively; the total proportions were 4.56%, 17.46%, 11.35% and 1.74%, respectively; the minimum/maximum proportion values of each height group were at 11 years old /8 years old, 14 years old/8 years old, 8 years old/12 years old, and 6 years old/11 years old, respectively. The proportions of girls aged 6 - 15 years in Nanning City with height ≤-2s, ≤-s, ≥+s, and ≥+2s fluctuated from 2.06% to 5.19%, 9.35% to 25.15%, 8.21% to 15.80% and 1.23% to 3.49%, respectively; the total proportions were 3.38%, 16.91%, 11.97% and 2.29%, respectively; and the minimum/maximum proportion values of each height group were at 13 years old/6 years old, 12 years old/6 years old, 7 years old/12 years old, and 6 years old/11 years old, respectively. 【Conclusions】 The overall height level of children in Nanning is still lower than the national level, with short prepubertal basal heights, an earlier age of onset of accelerated pubertal height, and a shorter duration of accelerated pubertal height in boys. Strengthening pre-pubertal height management and emphasizing the onset and duration of children′s pubertal development, especially the height development of boys during puberty, can help improve the adult lifelong height of children in this region.

OBJECTIVE To evaluate the effectiveness of Polyene phosphatidylcholine capsules （PPC） as adjuvant therapy for chronic hepatitis B （CHB）. METHODS Retrospective data were collected from the patients diagnosed with CHB， treated with hepatoprotective drugs combined with antiviral drugs or antiviral drugs alone， and underwent long-term follow-up in the outpatient department of Sichuan Academy of Medical Sciences & Sichuan Provincial People’s Hospital from January 1， 2017 to December 31， 2022. After balancing confounding factors through propensity score matching， the effectiveness of PPC combined with antiviral therapy versus antiviral therapy alone （PPC+antiviral group versus antiviral group） and PPC+Diammonium glycyrrhizinate enteric- coated capsules （DGC） combined with antiviral therapy versus DGC combined with antiviral therapy （PPC+DGC+antiviral group versus DGC+antiviral group） as therapy for CHB were compared under real medical conditions. RESULTS Totally 382 patients with CHB who received hepatoprotective agents based on antiviral therapy （221， 63 and 98 patients who received DGC， PPC and combination therapy， respectively） and 400 patients who received antiviral therapy alone were ultimately included. After propensity score matching， there were 47 patients each in the PPC+antiviral group and the antiviral group， respectively； after treatment， the alanine transaminase （ALT） levels in the PPC+antiviral group were significantly reduced compared to before treatment and the antiviral group at the same time （P＜0.05）， while there was no statistically significant difference in the ALT normalization rate between the two groups （P＞0.05）. There were 74 patients each in the PPC+DGC+antiviral group and the DGC+antiviral group， respectively； after treatment， the ALT levels of patients in both groups were significantly reduced compared to before treatment， the ALT levels of PPC+DGC+antiviral group were significantly lower than those in the DGC+antiviral group at the same time， and the ALT normalization rate was significantly higher in the PPC+DGC+antiviral group than that in the DGC+antiviral group （P＜0.05）. CONCLUSIONS Based on using antiviral drugs to treat CHB， adjuvant therapy combined with PPC has a significant advantage in reducing liver enzymes； in addition， compared with the DGC combined antiviral regimen， the dual hepatoprotective drug of DGC and PPC combined with an antiviral regimen has better effects on liver protection and reduction of liver enzymes.