ObjectiveTo investigate vitamin D level in children with cholestatic liver disease， and to provide a theoretical basis for vitamin D supplementation therapy in children with this disease. MethodsA total of 116 children with cholestatic liver disease who attended Department of Traditional Chinese Medicine， Beijing Children’s Hospital， Capital Medical University， for the first time from January 2022 to January 2024 were enrolled and divided into groups for comparison based on sex， age， vitamin D supplementation dose， course of the disease， and etiology. The data on the serum level of 25-hydroxyvitamin D （25-OH-D） and related biochemical parameters were collected to assess the correlation between vitamin D level and biochemical parameters. The chi-square test or the Fisher’s exact test was used for comparison of categorical data between groups， and the Spearman rank correlation test was used for correlation analysis. ResultsAmong the 116 children， 76 （65.5%） had vitamin D deficiency or insufficiency. The children with vitamin D deficiency or insufficiency accounted for 65.7% （46/70） among boys and 65.2% （30/46） among girls， with no significant difference between boys and girls （χ²=0.003， P=0.956）. The children with vitamin D deficiency or insufficiency accounted for 83.3% （25/30） among the children who had never received vitamin D supplementation， 58.7% （27/46） among the children with a daily supplementation dose of 500 IU， 64.3% （18/28） among the children with a daily supplementation dose of 700 IU， and 50.0% （6/12） among the children with a daily supplementation dose of>700 IU， and there was no significant difference between these groups （χ²=6.460， P=0.091）. Comparison between the groups with different etiologies showed that the children with vitamin D deficiency or insufficiency accounted for 57.7% （15/26） in the infectious disease group， 66.7% （10/15） in the inherited metabolic disease group， 66.7% （6/9） in the drug-induced liver injury group， 100.0% （8/8） in the group with abnormal structure of the biliary system， and 63.8% （37/58） in the group with unknown etiology， and there was no significant difference between these groups （χ²=5.304， P=0.252）. Comparison between the groups with different courses of the disease showed that the children with vitamin D deficiency or insufficiency accounted for 78.4% （29/37） in the<1 month group， 54.3% （25/46） in the 1‍ — ‍3 months group， 53.3% （8/15） in the 3‍ — ‍6 months group， and 77.8% （14/18） in the>6 months group， with no significant difference between these groups （χ²=7.432， P=0.059）. Comparison between different age groups showed that compared with the infant group， the children group had a significantly higher proportion of children with vitamin D deficiency or insufficiency （χ²=9.504， P=0.018）. The correlation analysis showed that serum aspartate aminotransferase and alanine aminotransferase had no significant correlation with 25-OH-D （P>0.05）； serum alkaline phosphatase （ALP） （r=-0.286， P=0.002）， gamma-glutamyl transpeptidase （GGT） （r=-0.248， P=0.007）， total bilirubin （TBil） （r=-0.353， P<0.001）， direct bilirubin （DBil） （r=-0.299， P=0.001）， and total bile acid （r=-0.236， P=0.011） were negatively correlated with 25-OH-D， while serum calcium （r=0.263， P=0.004） and phosphorus （r=0.385， P<0.001） were positively correlated with 25-OH-D. ConclusionMost children with cholestatic liver disease have vitamin D deficiency or insufficiency， and the increase in serum ALP， GGT， TBil， DBil or total bile acid and the reduction in calcium or phosphorus may suggest vitamin D deficiency or insufficiency.

Apical sodium-dependent bile acid transporter （ASBT） is a key transporter responsible for intestinal reabsorption of bile acid and plays an important role in maintaining bile acid and cholesterol homeostasis， and its expression is regulated by various factors including transcription factors， nuclear receptors， and intestinal microflora. The abnormal expression and function of ASBT can lead to disorders in the metabolism of bile acid and cholesterol， causing a variety of hepatobiliary diseases. At present， ASBT has attracted wide attention as a therapeutic target. This article elaborates on the biological characteristics and expression regulation mechanism of ASBT and reviews the role of ASBT in hepatobiliary diseases， in order to provide a new direction for the treatment of related diseases.

Objective To preliminarily study the effectiveness and safety of stereotactic ablative brachytherapy (SABT) for lung metastases from cervical cancer. Methods We analyzed the clinical data of 18 patients with cervical cancer with lung metastasis treated with SABT to compare gross tumor volume (V_GTV) and squamous cell carcinoma (SCC) antigen before and after SABT. The clinical benefit rate (CBR) and adverse reactions were recorded. Results After SABT treatment, there were significant decreases in V_GTV (t=1.708, P<0.05) and the SCC antigen level (t=1.704, P<0.05). CBR reached 94.4%. Adverse reactions of grades 3-4 did not occur in any patient. Fourteen patients had mild complications, including 1 case of bloody sputum and 1 case of a small pneumothorax. Ten cases developed mild radiation-induced lung injury, with grade 2 radiation pneumonitis in 4 cases. The Karnofsky performance status score and needle depth were not associated with the occurrence of adverse reactions, while the radius of GTV and interstitial lung disease were associated with the occurrence of adverse reactions. Conclusion SABT is a safe and effective alternative to the treatment of lung metastases from cervical cancer.

Objective:To construct a health education program for patients with permanent enterostomy based on the information motivation behavioral skills model, and to test its effectiveness in improving patient readiness for discharge and reducing the incidence of early ostomy complications after discharge.Methods:From February 2021 to January 2022, convenience sampling was used to select 68 patients with low rectal cancer who were diagnosed by the Colorectal Surgery Department at the First Affiliated Hospital of Wenzhou Medical University and underwent abdominal perineal combined radical resection of rectal cancer (Miles) as research subjects. Randomized number series were generated using the digital table method, and the subjects were randomly divided into control group (33 cases) and intervention group (35 cases) using the envelope method. The control group was housed in the first ward, while the intervention group was housed in the second ward. The control group implemented the conventional health education, while the intervention group carried out the health education based on the information motivation behavioral skills model. The discharge readiness scores, ostomy self-efficacy and ostomy complications were compared between the two groups on the day of discharge and one month after discharge.Results:Patients in the intervention group had higher discharge readiness scores and higher discharge readiness levels than those in the control group, with statistical differences ( P<0.01) . The intervention group had higher levels of ostomy self-efficacy than the control group one month after discharge, with statistically significant difference ( P<0.01) . At the time of discharge for one month, the overall incidence of ostomy complications in the intervention group was 11.43% (4/35) , lower than 42.42% (14/33) in the control group, with a statistically significant difference ( P<0.01) . Conclusions:The health education program based on the information motivation behavioral skills model can effectively increase the readiness of patients with permanent enterostomy for discharge, reduce the incidence of complications of the ostomy and surrounding skin, improve the ostomy self-efficacy and promote patient rehabilitation.

Objective:To explore the effect of introducing mixed reality technology into traditional atlas teaching to teach airway anatomy under bronchoscopy.Methods:A total of 30 Batch 2017 fifth-year clinical medicine students from Shanghai Jiao Tong University School of Medicine were randomly divided into control group and test group by RAND function in Excel, with 15 students in each group. The control group was taught with the traditional bronchoscopic atlas teaching, and the test group was combined with mixed reality technology. The two groups had the same class time. After teaching, the teaching effect was evaluated by examination and evaluation questionnaire. SPSS 25.0 software was conducted for t test and Mann-Whitney U test. Results:The average score after teaching of test group was (61.67±20.15), and that of control group was (36.67±13.32), with statistically significant differences ( t=4.01, P<0.001). According to the questionnaire results, the scores of the test group on course understanding, course concentration, participation, mastery and satisfaction were better than those of the control group, and the differences were statistically significant ( P<0.05). Conclusion:Using mixed reality technology to assist the clinical teaching of airway anatomy under bronchoscopy can improve the quality of students' study and enhance their understanding of the teaching content and students' participation passion, achieving better teaching effect.

OBJECTIVE: To explore the strategy of pregnancy-preserving and maternal- fetal management in patients with primary gynecologic neuroendocrine tumors (gNETs) during pregnancy. METHODS: We performed whole genome sequencing (WGS) for analyzing maternal and fetal somatic and germline single nucleotide variations (SNVs) and small insertions and deletions (InDels) for a 29-year-old pregnant woman diagnosed with stage IB2 large cell neuroendocrine carcinoma (LCNEC) and adenocarcinoma in the cervix. A systematic literature review was performed to explore the strategies for treatment of such rare histological type while maintaining pregnancy. RESULTS: Global case analysis of cervical NETs during pregnancy suggested that negative lymph node metastasis and an early FIGO stage were potentially associated with a good prognosis of the patients. In the case presented herein, a pregnancy-preserving strategy was adopted and favorable maternal-fetal outcomes were achieved after neoadjuvant chemotherapy, radical surgery and postoperative systemic chemotherapy. At 35 CONCLUSIONS Although gNETs in pregnancy are rare and highly risky, pregnancy-preserving managements of gNETs can still be considered and favorable maternalfetal outcomes are possible with proper assessment of the clinical indications and implementation of multimodal treatments. Precise treatment and follow-up strategies based on the results of WGS for risk-reducing intervention of cancer recurrence or occurrence can potentially benefit the patient and the neonate.
Adenocarcinoma ; Adult ; Carcinoma, Neuroendocrine/genetics* ; Child ; Female ; Humans ; Infant, Newborn ; Neoplasm Recurrence, Local ; Pregnancy ; Uterine Cervical Neoplasms/genetics*

Entropy model is widely used in epileptic electroencephalogram (EEG) analysis, but there are few reports on how to objectively select the parameters to compute the entropy model in the analysis of resting-state functional magnetic resonance imaging (rfMRI). Therefore, an optimization algorithm to confirm the parameters in multi-scale entropy (MSE) model was proposed, and the location of epileptogenic hemisphere was taken as an example to test the optimization effect by supervised machine learning. The rfMRI data of 20 temporal lobe epilepsy (TLE) patients with hippocampal sclerosis, positive on structural magnetic resonance imaging, were divided into left and right groups. Then, the parameters in MSE model were optimized by the receiver operating characteristic curves (ROC) and area under ROC curve (AUC) values in sensitivity analysis, and the entropy value of the brain regions with statistically significant difference between the groups were taken as sensitive features to epileptogenic hemisphere lateral. The optimized entropy values of these bio-marker brain areas were considered as feature vectors input into the support vector machine (SVM). Finally, combining optimized MSE model with SVM could accurately distinguish epileptogenic hemisphere in TLE at an average accuracy rate of 95%, which was higher than the current level. The results show that the MSE model parameter optimization algorithm can accurately extract the functional imaging markers sensitive to the epileptogenic hemisphere, and achieve the purpose of objectively selecting the parameters for MSE in rfMRI, which provides the basis for the application of entropy in advanced technology detection.
Brain/diagnostic imaging* ; Brain Mapping ; Entropy ; Epilepsy, Temporal Lobe/diagnostic imaging* ; Humans ; Magnetic Resonance Imaging

Objective:To explore the value of triple fusion positron emission tomography (PET)/MRI in location of epileptogenic focus in patients with focal cortical dysplasia (FCD).Methods:Three patients with refractory partial epilepsy, admitted to our hospital from December 2016 to June 2017, were chosen in our study. The raw MRI and PET images of these patients were processed using Freesurfer and FSL image processing softwares. After extraction and coregistration, precise PET/MRI fusion images were obtained; and the grey-white matter dividing line was highlighted on this fusion image to form triple-fusion images to observe the hypometabolic area and clarify the location.Results:Triple-fusion images of these 3 patients were acquired. In patient 1, a marked decrease in metabolism was noted in the gyrus region delineated by the gray-white matter boundary in the right cingulate gyrus. In patient 2, the area with slightly increased local signal in the right superior frontal gyrus (MRI FLAIR sequence) was the area with reduced metabolism. In patient 3, an area of local decreased metabolism was noted in the right cingulate gyrus. The preoperative evaluation of all 3 patients showed that the above areas were epileptic foci; the patients were followed up for 2 years after surgical resection, no clinical seizures occurred in all patients, and antiepileptic drugs were gradually stopped. All 3 patients were diagnosed as having FCD by postoperative pathology.Conclusion:Triple fusion PET/MRI is a powerful way to assist FCD diagnosis, especially for those FCD cases which are difficult to be diagnosed by other imaging methods, and has a potential clinical application value in epilepsy patients.

Objective: To reveal the clinical and genetic features of neonatal/infantile epileptic disorders caused by KCNQ2 mutations and to provide a clue for the treatment and prognosis evaluation. Methods: Twenty-two patients were collected in the Department of Pediatrics, Peking University First Hospital from April 2007 to July 2016.The phenotype-genotype analysis was conducted of the neonatal/infantile epileptic patients in whom a KCNQ2 mutation was identified by the targeted next generation sequencing. Results: Twenty-two de novo KCNQ2 missense mutations from 22 patients with neonatal/infantile epileptic disorders were found.These patients had an onset of epilepsy in early infancy (median age: 2 days). The seizure type of the first onset was mainly focal seizure.Atypical absence epilepsy, a novel phenotype of KCNQ2 mutation-induced epilepsies was found.The mortality of these patients was high, as 5 patients of the 22 patients died in the follow-up period, 4 of which might result from sudden unexpected death in epilepsy.In the 22 patients, 8 patients with anti-epileptic monotherapy became seizure-free.Of the 8 patients with a monotherapy, 3 patients were treated with valproic acid and no clinical onset was observed. Conclusions This study expands the phenotype of KCNQ2-related epileptic disorders.These patients have high mortality.Valproate acid is the potentially effective monotherapy for these patients.

Objective To investigate the correlation between the level of presenilin-associated rhomboidlike protein (PARL) and the sperm motility,survival rate and deformity rate of sperm in workers exposed to aluminum.Methods A total of 162 male workers exposed to aluminum in a large aluminum enterprise in Guangxi were selected as the exposure group,and the 162 staff members of the service company were selected as the control group by matching the age and length of service.The concentration of aluminum in the working environment and the aluminum content in the blood and urine were determined by graphite furnace atomic absorption spectrometry and high performance liquid chromatography.Enzyme linked immunosorbent assay was used to measure the level of PARL protein in sperm and sperm function was evaluated.Results The average concentrations of aluminum in the batching,electrolysis and casting of aluminum exposure group were (6.72± 1.45),(7.23± 1.50) and (7.35± 1.72)mg/m3,which were significantly higher than those of the control group (F=8.314,P＜0.001).The content of aluminum in blood and urine of exposed group were significantly higher than those of control group (P＜0.05),while the levels of PARL of exposed group were significantly lower than those of control group (P＜0.05).Moreover,the sperm survival rate and sperm motility of exposed group were significantly lower than those of the control group,and the sperm deformity rates of exposed group were significantly higher than those of the control group(P＜0.05).Pearson correlation analysis showed that there was a positive correlation of sperm motility,sperm survival rate and protein level of PARL (rsprm motility=0.713,P=0.012;rsperm survival rate =0.628,P=0.008);while the sperm deformity rate and protein level of PARL showed a significant negative correlation (rsperm deformity rate =0.953,P =0.002).Conclusion The sperm function was significantly impaired in aluminum exposed workers,and the changes of sperm motility,survival rate and malformation rate were closely related to the protein level of PARL.