OBJECTIVE To establish the characteristic chromatogram of the volatile oil in the standard decoction of Qingshang juantong decoction， and preliminarily infer the main active components of volatile oil that affect the clinical therapeutic effect. METHODS The volatile oil in the standard decoction of Qingshang juantong decoction was extracted by steam distillation. The ultra-high performance liquid chromatography （UPLC） characteristic chromatograms of 15 batches of samples were established by the Similarity Evaluation System of TCM Chromatographic Fingerprint （2012 edition）， and the similarity evaluation was carried out. The volatile oil of standard decoction was identified by UPLC coupled with quadrupole time-of-flight mass spectrometry （UPLC-Q-TOF/MS）. Then the volatile oil components were analyzed by GC-MS. RESULTS The similarities of UPLC characteristic chromatograms for volatile oil of 15 batches of Qingshang juantong decoction were between 0.949 and 0.997. A total of 12 common peaks were obtained. According to the UPLC-Q-TOF/MS， the main components were methyl eugenol， E-ligustilide， E-butylidenephthalide and so on. A total of 23 components were identified by GC-MS， which were mainly 3，4，5-trimethoxy- methylbenzene， patchouli alcohol， Z-ligustilide and so on. CONCLUSIONS The characteristic chromatograms of the volatile oil in the standard decoction of Qingshang juantong decoction is established， and it is inferred that methyl eugenol， ligustilide， E- butylidenephthalide， patchouli alcohol， 3，4，5-trimethoxy-methylbenzene might be the main active components affecting the clinical therapeutic effect of the volatile oil of Qingshang juantong decoction.

Objective:To explore the differences of fecal calprotectin (FC), C-reactive protein (CRP), and erythrocyte sedimentation rate (ESR) between colon and small intestinal Crohn′s disease, and their predictive values for disease activity and mucosal healing in patients with small intestinal Crohn′s disease.Methods:From January 2017 to January 2023, 64 patients with Crohn′s disease who underwent capsule endoscopy in the First Affiliated Hospital of Zhejiang Chinese Medical University were enrolled, among them 28 patients had only small intestinal lesions (small intestine group) and 36 patients had lesions involving both small intestine and colon or only colon involvement (ileocolon group). The FC, CRP, and ESR levels of the two groups were detected and compared 15 days before capsule endoscopy examination. Wilcoxon rank-sum test was used for statistical analysis. Receiver operating characteristic curve analysis was used to evaluate the predictive value of FC, CRP, and ESR for disease activity and mucosal healing in patients with small intestinal Crohn′s disease.Results:The FC, CRP, and ESR levels of the small intestine group during the active phase of the disease were 1 689.00 μg/g (727.75 μg/g, 1 800.00 μg/g), 5.67 mg/L (1.00 mg/L, 17.01 mg/L), and 4.50 mm/1 h (2.00 mm/1 h, 11.00 mm/1 h), respectively; while FC, CRP, and ESR levels during the mucosal healing phase were 112.00 μg/g (46.50 μg/g, 130.50 μg/g), 1.00 mg/L (1.00 mg/L, 1.62 mg/L), and 2.00 mm/1 h (2.00 mm/1 h, 5.50 mm/1 h), respectively. The FC, CRP, and ESR levels of the ileocolon group during the active phase of the disease were 1 800.00 μg/g (895.50 μg/g, 1 800.00 μg/g), 4.94 mg/L (3.10 mg/L, 14.80 mg/L), and 10.00 mm/1 h (2.00 mm/1 h, 27.75 mm/1 h), respectively, while FC, CRP, and ESR levels during the mucosal healing phase were 66.00 μg/g (32.50 μg/g, 97.50 μg/g), 1.00 mg/L (1.00 mg/L, 1.55 mg/L), and 2.00 mm/1 h (2.00 mm/1 h, 4.50 mm/1 h), respectively. There were no statistically significant differences in FC, CRP, and ESR between the small intestine group and the ileocolon group during the active phase of the disease and mucosal healing phase (all P> 0.05). In the small intestine group, the levels of FC and CRP of patients during the active phase of the disease were 1 173.00 μg/g (312.00 μg/g, 1 800.00 μg/g) and 2.10 mg/1 L (1.00 mg/L, 16.00 mg/L), which were both higher than those of patients during the mucosal healing phase (112.00 μg/g (46.50 μg/g, 130.50 μg/g) and 1.00 mg/L (1.00 mg/L, 1.62 mg/L)), and the differences were statistically significant ( Z=-4.35 and-2.67, P<0.001 and =0.008). In the small intestine group, the level of ESR of patients during the active phase of the disease was 4.00 mm/1 h (2.00 mm/1 h, 16.00 mm/1 h), and there was no significant difference compared with that of patients during the mucosal healing phase (2.00 mm/1 h (2.00 mm/1 h, 5.50 mm/1 h)) ( P>0.05). When the cut-off level of FC was 188.50 μg/g, the sensitivity, specificity, and area under the curve for predicting disease activity in patients with small intestinal Crohn′s disease was 93.3%, 100.0%, and 0.964, respectively. When the cut-off value of CRP was 3.12 mg/L, the sensitivity, specificity, and area under the curve for predicting disease activity in patients with small intestinal Crohn′s disease was 46.7%, 92.3%, and 0.744, respectively. When the cut-off level of ESR was 10.00 mm/1 h, the sensitivity, specificity, and area under the curve for predicting disease activity in patients with small intestinal Crohn′s disease was 33.3%, 100.0%, and 0.654, respectively. There were no statistically significant differences in the area under the curve between the combinations of FC and CRP, FC and ESR, FC, CRP and ESR, and FC alone for predicting disease activity in patients with small intestinal Crohn′s disease (0.964, 0.959, and 0.959 vs. 0.964, all P> 0.05). There was a statistically significant difference in the area under the curve between the combination of CRP and ESR and FC alone in predicting disease activity in patients with small intestinal Crohn′s disease (0.708 vs. 0.964, Z=-2.57, P=0.010). Conclusions:There are no statistically significant differences in FC, CRP, and ESR between colon and small intestinal Crohn′s disease. FC has a high predictive value for disease activity and mucosal healing in patients with small intestinal Crohn′s disease and has certain clinical application value.

OBJECTIVE: To explore the clinical characteristics and genetic basis of a child with Teebi hypertelorism syndrome 1 (TBHS1). METHODS: A child with TBHS1 who was admitted to the Children's Medical Center Affiliated to Shanghai Jiao Tong University School of Medicine on July 13, 2021 was selected as the study subject. Clinical data of the child was collected. Peripheral blood samples of the child and his parents were collected and subjected to whole exome sequencing (WES). Candidate variant was verified by Sanger sequencing and bioinformatic analysis. RESULTS: The child, a 13-year-old male, had manifested delayed growth and development. WES results revealed that he has harbored a heterozygous c.1244A>G variant of the SPECC1L gene, which was verified to be de novo in origin. The variant has not been included in the HGMD and gnomAD databases. As predicted by online software including PolyPhen-2, SIFT, and Mutation Taster, the variant may affect the function of protein domain. And PyMOL software has predicted that the structural stability of SPECC1L protein (p.Gln415Arg) might be reduced. Based on the guidelines of the American College of Medical Genetics and Genomics (ACMG), the variant was classified as pathogenic (PM6+PM1+PP4+PM2_Supporting+PP3). CONCLUSION The heterozygous c.1244A>G variant of the SPECC1L gene probably underlay the TBHS1 in this child. Above finding has expanded the genotypic and phenotypic spectrum of the SPECC1L gene and provided a basis for the clinical diagnosis of this child.
Adolescent ; Humans ; Male ; China ; Computational Biology ; Genomics ; Genotype ; Mutation

ObjectiveTraditional Chinese medicine， namely Dahuang Zhechongwan （DHZCW） was used to treat myocardial fibrosis in model rats， observe its effect on myocardial fibrosis in rats， and explore its action mechanism. MethodThirty-six SPF male Kunming rats were divided into blank group， model group， low-， medium-， high-dose groups of DHZCW （0.056， 0.084， 0.168 g·kg^-1）， captopril group （10 mg·kg^-1）， with six rats in each group. Except for the blank group， the other groups were intraperitoneally injected isoproterenol solution of 5 mg·kg^-1 for 15 consecutive days to replicate the myocardial fibrosis model. At the beginning of modeling， the rats in each group took drugs， and they were sacrificed 28 days after administration. Serum and heart tissue were collected for the corresponding detection. Hematoxylin-eosin （HE） staining and Masson staining were used to observe tissue inflammation， cellular degeneration， necrosis， and fibrosis. The contents of hydroxyproline （HYP）， tumor necrosis factor-α （TNF-α）， interleukin-1β （IL-1β）， interleukin-6 （IL-6）， hyaluronic acid （HA）， laminin （LN）， type-Ⅲ procollagen （PC Ⅲ） in serum of rats and rats were determined by enzyme-related immunosorbent assay （ELISA）. The expression levels of key pathway proteins transforming growth factor-β₁ （TGF-β₁）， α-smooth muscle actin （α-SMA）， Smad2， Smad3， and Smad7 were detected by Western blot. The expression levels of key pathway genes TGF-β₁， α-SMA， Smad2， Smad3， Smad7， miR-29a-5p， miR-29b-2-5p， and miR-29c-5p were detected by Real-time quantitative polymerase chain reaction （Real-time PCR）. ResultCompared with the blank group， the pathological changes of fibrosis in the model group were obvious， the contents of serum HYP， TNF-α， IL-1β， IL-6， HA， LN， and PCⅢ were increased （P<0.01）， the protein expression levels of TGF-β₁， α-SMA， Smad2， and Smad3 were increased； the protein expression level of Smad7 was decreased （P<0.01）. The mRNA expression levels of TGF-β₁， α-SMA， Smad2， and Smad3 were increased （P<0.05， P<0.01）， while those of Smad7， miR-29a-5p， miR-29b-2-5p， and miR-29c-5p were decreased （P<0.01）. Compared with the model group， after 28 days of administration， serum HYP， TNF-α， IL-1β， IL-6， HA， LN， and PCⅢ in high-， medium-， and low-dose groups of DHZCW and captopril groups were decreased （P<0.01）. Except for the low-dose group， the protein contents of TGF-β₁， α-SMA， Smad2， and Smad3 were decreased， while the protein content of Smad7 was increased （P<0.01）. The mRNA expression levels of TGF-β₁， Smad2， α-SMA， and Smad3 in high-dose group of DHZCW were decreased （P<0.05，P<0.01）， while those of Smad7， miR-29a-5p， miR-29b-2-5p， and miR-29c-5p were increased （P<0.05）. The mRNA expressions of TGF-β₁， Smad2， and Smad3 in the medium-dose group of DHZCW were decreased （P<0.05， P<0.01）， while mRNA expression of Smad7 was increased （P<0.01）. The mRNA levels of TGF-β₁ and Smad2 in the low-dose group of DHZCW were decreased （P<0.01）. ConclusionDHZCW can improve myocardial fibrosis in rats， and its action mechanism may be related to the regulation of the TGF-β₁/Smads/miR-29 pathway. In addition， there is dose dependence in the range of 0.056-0.168 g·kg^-1， and the effect of the high-dose group is more stable.

OBJECTIVE To investigate the effects of different drying methods on the index components in wine-processed Cornus officinalis so as to optimize drying method.METHODS After processed with wine， C. officinalis decoction pieces were dried with different drying methods （blast drying， far infrared drying， microwave drying， freeze drying， sun drying， shade drying and combined drying）. The contents of 5 components such as gallic acid in wine-processed C. officinalis were determined by high- performance liquid chromatography. The contents of total flavonoids in wine-processed C. officinalis were determined by chromogenic method. Analytic hierarchy process was used to evaluate the effects of different drying methods on the contents of components in C. officinalis.RESULTS The contents of gallic acid， 5-hydroxymethylfurfural， monoside， loganin， cornuside and total flavonoids in 22 batches of wine-processed C. officinalis were 1.043 8-1.563 8， 0.648 5-2.358 8， 5.031 0-10.305 7， 6.681 2- 7.534 2， 0.986 5-1.148 8 and 33.657 2-50.741 5 mg/g， respectively. The comprehensive scoring results of analytic hierarchy process showed that the comprehensive score of each component in C. officinalis dried by microwave at 75 ℃ was higher ， followed by blast drying at 60 ℃ and far infrared drying at 60 ℃ .CONCLUSIONS The wine-processed C. officinalis could be dried by microwave drying at 75 ℃， blast drying at 60 ℃ or far infrared drying at 60 ℃.

School-age children are in a specific development stage corresponding to juvenility, when the white matter of the brain experiences ongoing maturation. Diffusion-weighted magnetic resonance imaging (DWI), especially diffusion tensor imaging (DTI), is extensively used to characterize the maturation by assessing white matter properties in vivo. In the analysis of DWI data, spatial normalization is crucial for conducting inter-subject analyses or linking the individual space with the reference space. Using tensor-based registration with an appropriate diffusion tensor template presents high accuracy regarding spatial normalization. However, there is a lack of a standardized diffusion tensor template dedicated to school-age children with ongoing brain development. Here, we established the school-age children diffusion tensor (SACT) template by optimizing tensor reorientation on high-quality DTI data from a large sample of cognitively normal participants aged 6-12 years. With an age-balanced design, the SACT template represented the entire age range well by showing high similarity to the age-specific templates. Compared with the tensor template of adults, the SACT template revealed significantly higher spatial normalization accuracy and inter-subject coherence upon evaluation of subjects in two different datasets of school-age children. A practical application regarding the age associations with the normalized DTI-derived data was conducted to further compare the SACT template and the adult template. Although similar spatial patterns were found, the SACT template showed significant effects on the distributions of the statistical results, which may be related to the performance of spatial normalization. Looking forward, the SACT template could contribute to future studies of white matter development in both healthy and clinical populations. The SACT template is publicly available now ( https://figshare.com/articles/dataset/SACT_template/14071283 ).

A comprehensive profiling of the vaginal microbial communities and their variability enables an accurate description of the microbiome in women.However,there is a lack of studies available on Chinese women.In the present study,the composition of the vaginal microbiota during pregnancy and the 6-week postpartum period of 454 Chinese women was characterized by sequenc-ing the V3-V4 region of the 16S ribosomal RNA(rRNA)gene.The vaginal microbiome showed variations during pregnancy and the postpartum period based on the abortion history,hypertensive disorders,delivery mode,and maternal age.Co-variation of 22 bacterial taxa,including the Lacto-bacillus genus and two of its species,may account for the common characteristics of the vaginal microbiome under scenarios of different medical histories and pregnancy outcomes.In contrast,dis-criminant bacterial species were significantly different between women who had preterm birth(PTB)with and without premature rupture of membranes(PROM),and the community state type(CST)Ⅳ-A without any predominant Lactobacillus species in the microbiota was more prevalent during pregnancy in the PROM-PTB cases,suggesting that specific bacterial species could be considered to distinguish between different types of PTB.By providing data on Chinese women,this study will enrich the knowledge of the human microbiome and contribute to a better understanding of the association between the vaginal microbiome and reproductive health.

Objective To evaluate the relationship between different levels of central venous pres-sure (CVP) and blood loss during pediatric living donor liver transplantation (LDLT). Methods Pediat-ric patients underwent LDLT in Renji Hospital from 2006 to August 10, 2016 were retrospectively reviewed. The patients were divided into 2 groups according to the different CVP levels before peritoneum closure:central venous pressure<10 mmHg group ( group L) and CVP≥10 mmHg group ( group H) . The primary outcome measure was intraoperative blood loss. The secondary outcome measures were duration of mechani-cal ventilation in intensive care unit (ICU), duration of ICU stay, length of postoperative hospital stay, intraoperative blood loss, intraoperative blood transfusion, intraoperative volume of liquid infused, opera-tion time and anesthesia time. Results A total of 442 pediatric patients were enrolled in this study, with 209 cases in group L and 233 cases in group H. Compared with group H, the intraoperative blood loss was significantly decreased, the anesthesia time, operation time and length of postoperative hospital stay were shortened ( P<0. 05) , and no significant change was found in intraoperative blood transfusion, intraopera-tive volume of liquid infused, duration of mechanical ventilation in ICU or duration of ICU stay in group L ( P>0. 05) . Conclusion Maintaining intraoperative CVP<10 mmHg can markedly reduce the blood loss during LDLT and is helpful for postoperative recovery in pediatric patients.

Objective To investigate the correlation between serum hepcidin level and iron, mineral metabolism in maintenance hemodialysis (MHD) patients. Methods Seventy-five MHD patients were selected. The serum hepcidin, serum iron and mineral metabolism indexes were detected by enzyme-linked immunosorbent double antibody sandwich method, and their correlation was analyzed. Results The level of serum hepcidin in 75 MHD patients was 87- 264 μg/L. The patients were divided into 3 groups according to serum hepcidin level. In group A, the serum hepcidin level of 26 cases was＜120 μg/L; in group B, the serum hepcidin level of 24 cases was 120- 200 μg/L; in group C, the serum hepcidin level of 25 cases was>200 μg/L. There were no significant differences in age, gender, albumin and serum calcium among 3 groups (P > 0.05). The levels of ferritin, transferrin saturation (TS), serum phosphorus and parathyroid hormone (PTH) increased in the 3 groups as the hepcidin level increased, while hemoglobin, serum iron, total iron binding capacity (TIBC), and 25 hydroxy vitamin D decreased significantly, and there were statistical differences (P＜0.05). The Pearson correlation analysis result showed that serum hepcidin was positively correlated with ferritin, PTH, serum phosphorus (r = 0.862, 0.536 and 0.320; P＜0.01); and serum hepcidin was negative correlation with serum iron, TIBC, hemoglobin, 25 hydroxy vitamin D (r=-0.358,-0.270,-0.284 and-0.614; P＜0.01); but there was no correlation between serum hepcidin and albumin and serum calcium (r=0.018 and-0.005, P＞0.05). Conclusions The serum hepcidin level in MHD patients is closely related to iron and mineral metabolism.

Objective To evaluate the clinical value of fasting blood glucose (FBG) in the diagnosis of multiple organ dysfunction syndrome (MODS) caused by pulmonary infection in elderly patients with diabetes. Methods Patients over 65 years old with diabetic pulmonary infection admitted to Department of Cadres Ward of Bethune International Peace Hospital of PLA from July 2015 to December 2016 were enrolled. According to the patient's highest FBG level during pulmonary infection, all patients were divided in two groups. The cases whose FBG ≥8.83 mmol/L were served as suspicious multiple organ dysfunction syndrome in the elderly (MODSE) group, and those had FBG < 8.83 mmol/L were served as suspicious non-MODSE group. The incidence of MODSE in suspicious MODSE group and suspicious non-MODSE group was compared. The receiver operating characteristic curve (ROC) was drawn, and the clinical value of highest FBG in the diagnosis of MODSE caused by pulmonary infection in elderly patients with diabetes was assessed. Results 119 elderly patients with diabetes and pulmonary infection were enrolled in the analysis. All patients were male, with age of 76-105 years with an average of (89.41±4.16) years. Among 119 patients, 55 of them with suspected MODSE, 64 of them with suspected non-MODSE. The incidence of MODSE in suspicious MODSE group was significantly higher than that in suspicious non-MODSE group (78.2% vs. 12.5%), with statistically significant difference (χ2 = 52.108, P = 0.000). The area under the ROC curve of the highest FBG for diagnosis of MODSE was 0.895, and 95% confidence interval (95%CI) was 0.839-0.951 (P < 0.001). When using FBG ≥ 8.83 mmol/L to diagnose MODSE induced by pulmonary infection in elderly patients with diabetes, the sensitivity was 84.3% and the specificity was 82.4%. Conclusion FBG ≥ 8.83 mmol/L, as an indicator reflecting the changes of metabolic function, have a diagnostic value for MODSE caused by pulmonary infection in elderly patients with diabetes.