AIM: To observe the changes of retinal nerve fiber layer(RNFL)thickness and radial peripheral capillary(RPC)density in patients with unilateral branch retinal vein occlusion(BRVO), and further analyze the correlation between RPC density and RNFL thickness.METHODS: Observational study. Totally 37 patients with unilateral BRVO diagnosed at the ophthalmology department of First Hospital of Qinhuangdao from October 2020 to January 2022 were selected, the 37 affected eyes were the unilateral BRVO group, and 37 fellow healthy eyes were the contralateral unaffected group, and 35 healthy individuals(35 right eyes were selected)without ocular diseases during the same period were selected as the normal control group. The best corrected visual acuity, intraocular pressure, anterior segment, fundus and optical coherence tomography angiography(OCTA)were examined in both eyes of all BRVO patients and healthy individuals. The central macular thickness(CMT), the RNFL thickness, and the optic disc-AV crossing distance(DAVD)were measured by built-in software of the OCTA equipment. The optimized U-net algorithm was used to eliminate the large blood vessels, and then the RPC density was calculated. The CMT, RNFL thickness and RPC density were compared among the three groups. And the correlations of the RPC density with the CMT, RNFL thickness, and the DAVD were investigated.RESULTS: Compared with the contralateral unaffected group and the normal control group, the CMT and the RNFL thickness were significantly thickened in the unilateral BRVO group(all P<0.05); there were no statistical differences in the CMT and the RNFL thickness between the contralateral unaffected group and the normal control group(all P>0.05). The RPC density in the unilateral BRVO group increased compared with the contralateral unaffected group and decreased compared with the normal control group, but there was no statistically difference(all P>0.05). However, the RPC density in the contralateral unaffected group decreased compared with the normal control group(P<0.05). The RPC density in the unilateral BRVO group was not correlated with the CMT(P=0.960), but positively correlated with the RNFL thickness(r=0.401, P=0.014)and negatively correlated with the DAVD(r=-0.339, P=0.040).CONCLUSION: The RNFL thickened significantly and the RPC density did not change significantly in the optic disc area of BRVO patients. The RPC density is positively correlated with the RNFL thickness, indicating that the RNFL thickness can be used as a monitoring indicator to analyze and study the damage degree of the RPC density.

To report the first case of congenital ichthyosis with woolly hair in China, and to elucidate its genetic variant profile. A 28-year-old female patient presented with rashes on the trunk and limbs for 28 years. Dermatological examination revealed dry and rough skin over the entire body, with well-defined, polygonal, dark brown, adherent scales densely distributed over the trunk and extremities. No significant abnormalities were observed in the mucous membranes, teeth, or nails. The patient exhibited uniformly distributed, woolly and curly hair with a soft, dry texture and normal density; no significant hair breakage or brittleness was observed, while the eyebrows appeared sparse. Hair microscopy showed irregular axial twisting of hair strands at varying intervals; scanning electron microscopy (SEM) revealed irregular hair shafts with some segments appearing flat in a belt-like pattern, and magnified SEM images further demonstrated hair cuticle damage on the surface of hair shafts, manifesting as localized upward curling and longitudinal grooves, indicating structural damage to the hair shafts. Whole-exome sequencing identified a homozygous variant c.124dupC in exon 3 of the PRSS8 gene in the proband, resulting in a frameshift mutation (p.Gln42Profs*24) in the PRSS8 protein. According to the guidelines of the American College of Medical Genetics and Genomics as well as the patient's clinical manifestations, this variant could be preliminarily classified as pathogenic. A diagnosis of syndromic ichthyosis was made. This case represents the first report of syndromic ichthyosis caused by PRSS8 variants in China and the second report in the world, and this condition is proposed to be tentatively named as "ichthyosis-woolly hair syndrome".

Objective:To investigate the association between blood pressure variability (BPV) and all-cause mortality and cardiovascular events in patients undergoing continuous ambulatory peritoneal dialysis (CAPD), and provide reference for clinical management in CAPD patients.Methods:This retrospective cohort study included patients who received CAPD at Guangdong Provincial People's Hospital between May 1, 2010, and July 31, 2023. Baseline and clinical data of the patients were collected. Coefficient of variation of systolic blood pressure (CVSBP) was used to assess BPV. The patients were divided into CVSBP T1, CVSBP T2 and CVSBP T3 groups based on CVSBP tertiles, and the differences among the three groups were compared. Diastolic blood pressure and mean arterial pressure were used to further assess BPV and sensitivity analysis was conducted. The primary endpoint was the composite outcome of all-cause mortality and cardiovascular events. Kaplan-Meier survival curve and Cox regression analysis were used to analyze the association between CVSBP and the primary endpoint.Results:A total of 358 CAPD patients were included, with age of (43.6±13.3) years, and 197 males (55.0%). The proportion of males, proportion of smoking, baseline blood urea nitrogen, serum creatinine and serum albumin in CVSBP T2 (9.08%≤CVSBP<12.55%, n=120) group and CVSBP T3 (CVSBP≥12.55%, n=119) group were lower than those in CVSBP T1 group (CVSBP<9.08%, n=119), and baseline systolic blood pressure, residual kidney Kt/V and total Kt/V were higher than those in CVSBP T1 group, with statistically significant difference among the three groups (all P<0.05). During follow-up of 37(23, 76) months, 49 patients (13.7%) experienced the composite endpoint events, including 12 patients (3.4%) of all-cause deaths and 42 patients (11.7%) of cardiovascular events. Kaplan-Meier survival analysis showed that the incidence of composite endpoint events in CVSBP T3 group was higher than that in CVSBP T1 group and CVSBP T2 group, but the difference was not statistically significant (Log-rank χ2=3.795, P=0.150). Multivariate Cox regression analysis showed that, after adjusting for age, sex, diabetes, baseline systolic blood pressure, residual renal function, and serum albumin, as a continuous variable, CVSBP was not associated with the risk of composite outcome in CAPD patients ( HR=1.058, 95% CI 0.985?1.135, P=0.122); as a categorical variable, with CVSBP T1 group as reference, CVSBP T2 group and CVSBP T3 group were not associated with the risk of composite outcome ( HR=1.222, 95% CI 0.471?3.167, P=0.681; HR=1.827, 95% CI 0.737?4.530, P=0.193). The sensitivity analysis showed that increased variability of diastolic blood pressure ( HR=1.162, 95% CI 1.063?1.270, P=0.021) and increased variability of mean arterial pressure ( HR=1.114, 95% CI 1.030?1.204, P=0.007) were correlated with higher risk of composite outcome in CPAD patients. Conclusions:Systolic blood pressure variability during follow-up is not associated with risk of composite outcome of all-cause mortality and cardiovascular events in CAPD patients. Increased variability of diastolic blood pressure and increased variability of mean arterial pressure are associated with a higher risk of composite outcome in CPAD patients. Interventions to reduce BPV may be helpful to improve the long-term prognosis of CAPD patients.

Objective To observe the clinical effect of doing Huatuo five-animal play treating blood-stasis-type silent myocardial ischemia(SMI)and its influence on coronary hemodynamics.Methods Ninety patients diagnosed as silent myocardial ischemia were randomly divided into a five-animal play group(n=45,age:51.56±11.00 years,24 males and 21 females)and a regular exercise group(n=45,age:52.44±7.19 years old,24 males and 21 females)according to a random number table.The five-animal play group practised the traditional five-animal play,while the regular exercise group con-ducted daily 30-minute moderate-intensity treadmill aerobic exercise,five times a week,for 4 weeks.The changes in the number of abnormal ST-T segment occurrences,myocardial oxygen consumption(CMO),coronary ischemia threshold(CIT),and blood smoothness index(BSD)within 24-hour ambu-latory electrocardiogram before and after the intervention were observed in both groups,with the thera-peutic effect and hemodynamic characteristics of both groups evaluated.Results The average number of ST-T segment abnormalities decreased after intervention in both groups(P<0.001),with significantly greater improvement in the five-animal play group than the regular exercise group(P<0.001).More-over,the average CMO decreased significantly,while the average CMR and BSD increased significant-ly in both groups after intervention(P<0.05),with significantly greater improvement in the five-ani-mal play group than the other group.Meanwhile,the total effective rate in the five-animal play group was significantly higher than the regular exercise group(P<0.01).Conclusion Undergoing the five-ani-mal play and moderate intensity treadmill aerobic exercise both are effective in treating silent myocardi-al ischemia.However,the former therapy is superior to the latter in bettering CMO,CIT and BSD.

Objective:To evaluate the efficacy of defocus incorporated multiple segments (DIMS) on spherical equivalent (SE) and axial length (AL) in myopic children.Methods:Clinical studies on the progress of DIMS lens intervention in myopia reported in CNKI, Wanfang Data, VIP, China Biomedical Literature Database, PubMed, Embase, Cochrane Library, and Web of Science from the establishment of the database to June 2023 were searched.The experimental group and control group used DIMS lens and single vision lens (SVL) for intervention, respectively.Two researchers independently conducted the literature search, screening and data extraction based on the inclusion and exclusion criteria.Literature quality and risk of bias were evaluated in accordance with the requirements of the Cochrane Manual.Meta-analysis was performed using Revman 5.4 software.Results:A total of 7 clinical studies using DIMS lenses to intervene in the development of myopia were included .Seven studies provided complete SE data for analysis, with sample sizes of 1 164 eyes and 1 140 eyes for the DIMS group and SVL group, respectively.Five studies provided complete AL data for analysis, with sample sizes of 339 eyes and 315 eyes for the DIMS group and SVL group, respectively.There were statistically significant differences in SE and AL between DIMS group and SVL group (SE: WMD=0.40, 95% CI: 0.32-0.47, P<0.001; AL: WMD=-0.14, 95% CI: -0.17--0.11, P<0.001).DIMS lens could effectively delay SE progression and AL growth in myopic patients. Conclusions:DIMS lenses could delay the increase of SE and AL to a certain extent.

To report the first case of congenital ichthyosis with woolly hair in China, and to elucidate its genetic variant profile. A 28-year-old female patient presented with rashes on the trunk and limbs for 28 years. Dermatological examination revealed dry and rough skin over the entire body, with well-defined, polygonal, dark brown, adherent scales densely distributed over the trunk and extremities. No significant abnormalities were observed in the mucous membranes, teeth, or nails. The patient exhibited uniformly distributed, woolly and curly hair with a soft, dry texture and normal density; no significant hair breakage or brittleness was observed, while the eyebrows appeared sparse. Hair microscopy showed irregular axial twisting of hair strands at varying intervals; scanning electron microscopy (SEM) revealed irregular hair shafts with some segments appearing flat in a belt-like pattern, and magnified SEM images further demonstrated hair cuticle damage on the surface of hair shafts, manifesting as localized upward curling and longitudinal grooves, indicating structural damage to the hair shafts. Whole-exome sequencing identified a homozygous variant c.124dupC in exon 3 of the PRSS8 gene in the proband, resulting in a frameshift mutation (p.Gln42Profs*24) in the PRSS8 protein. According to the guidelines of the American College of Medical Genetics and Genomics as well as the patient's clinical manifestations, this variant could be preliminarily classified as pathogenic. A diagnosis of syndromic ichthyosis was made. This case represents the first report of syndromic ichthyosis caused by PRSS8 variants in China and the second report in the world, and this condition is proposed to be tentatively named as "ichthyosis-woolly hair syndrome".

Objective:To compare the efficacy of hepatic artery infusion chemotherapy (HAIC) combined with targeted therapy and immunnotherapy and transarterial chemoembolization (TACE) combined with targeted therapy and immunnotherapy in the treatment of unresectable hepatocellular carcinoma HCC.Methods:We retrospectively analyzed the clinical data of 40 patients with unresectable HCC treated with HAIC combined with targeted therapy and immunnotherapy and TACE combined with targeted therapy and immunnotherapy in Tongde Hospital of Zhejiang Province and Zhejiang Provincial People's Hospital.The patients were divided into HAIC group ( n=14) and TACE group ( n=26) according to the different treatment methods. Baseline data, surgical conversion and intraoperative situation, tumor response, portal vein cancer thrombus control rate, leukocyte reduction rate, platelet reduction rate, incidence of liver function abnormalities, objective remission rate, and disease control rate were compared between the two groups. Results:The HAIC group had a later baseline tumor staging than the TACE group (higher percentage of portal vein cancer thrombus, CNLC stage Ⅲa).The surgical conversion rate of the HAIC and TACE groups were 28.6%(4/14) and 26.9%(7/26), respectively, with the difference of no statistical significance ( P>0.05);The operation time and intraoperative bleeding were (329.5±19.9) min vs.(413.4±26.4) min, (272.2±49.9) ml vs.(536.0±123.6) ml, and the differences were statistically significant ( P<0. 05); The maximum tumor diameter reduction rate [(30.7%±15.1%) vs.(7.2%±12.6%)] and portal vein cancer thrombus control rate [100% (12/12) vs. 64.3% (9/14)], the differences were statistically significant ( P<0.05);The incidences of leukocyte and platelet decrease in the two groups during the course of treatment were 71.4%(10/14) vs. 34.6%(9/26)、78.5%(11/14) vs. 38.5%(10/26), and the incidences of liver function abnormalities were 35.7%(5/14) vs. 69.2%(18/26), the differences were statistically significant ( P<0.05);The objective response rate and disease control rate were 57.1%(8/14) vs. 30.8% (8/26)、71.4% (10/14) vs. 53.8%(14/26), all statistically significant. Conclusion:HAIC combined with targeted therapy and immunnotherapy is a safe and effective treatment for middle and advanced HCC, especially suitable for patients with portal vein tumor thrombus(PVTT), large tumor, or poor liver function.

Accurate characterization of the chemical composition of complex traditional Chinese medicine (TCM) is an essential foundation for the modern scientific interpretation of TCM principles. Mass spectrometry is the most dominant technique in current research on the material basis of TCM, offering the highest sensitivity and the richest information provision. Establishing mass spectrometry databases represents the most effective approach to facilitating the structural analysis of TCM chemical components. This paper systematically searches and reviews literature published from January 2005 to January 2025 through online databases such as China National Knowledge Infrastructure, PubMed, and Web of Science, using “mass spectrometry database” and “traditional Chinese medicine” as keywords. It reviews the current status of seven TCM chemical component mass spectrometry databases and seven natural product mass spectrometry databases. The key advancements of these mass spectrometry databases for natural products are summarized, detailing their characteristics, search methodologies, included information, and data sources. Additionally, challenges related to data quality, standardization, timely updates, database interaction, retrieval functionality, and data sharing and security are discussed in depth. Furthermore, the paper explores prospective development directions for TCM mass spectrometry databases, emphasizing the importance of open data sharing, technological innovation, and data security. Through this analysis, the paper aims to offer theoretical guidance and practical recommendations for the precise identification of TCM components, as well as for the construction and application of these databases.

Energy metabolism reprogramming is one of the pathological mechanisms involved in the occurrence and development of tumors.This article focused on the theory of spleen deficiency in TCM and energy metabolism reprogramming to elaborate the pathological mechanism of tumors.TCM believes that spleen deficiency is an important organ pathogenesis that causes cancer due to deficiency,and spleen deficiency runs through the occurrence and development of tumors.The spleen is the source of qi and blood transformation and dominates the energy metabolism of the whole body.Mitochondria are the regulatory center of energy metabolism in the body,and abnormal mitochondrial energy metabolism is the manifestation of spleen deficiency at the cellular micro level,which is mainly manifested as the limitation of mitochondrial oxidative phosphorylation and the conversion to aerobic glycolysis to provide energy for the rapid growth of tumors.Therefore,based on the theory of spleen deficiency,the treatment based on spleen strengthening is used to prevent and treat tumors by improving energy metabolism,which not only enriches the modern medical connotation of spleen deficiency in TCM,but also provides a new perspective for TCM to treat malignant tumors from the perspective of spleen strengthening.

Objective·To investigate the changes in transcriptome and chromatin accessibility during the differentiation of human embryonic stem cells(hESCs)into neural progenitor cells(NPCs)using in vitro differentiation models and high-throughput multi-omics sequencing technologies.Methods·hESCs were first induced to differentiate into NPCs in vitro using the embryoid body formation method,and cells at both stages were collected.The cell phenotypes were identified by reverse transcription-quantitative real-time PCR(RT-qPCR)and immunofluorescence(IF)staining.Transcriptome sequencing(RNA-seq)was conducted to detect and analyze the differentially expressed genes(DEGs)between hESCs and NPCs.The assay for transposase-accessible chromatin with high-throughput sequencing(ATAC-seq)was employed to assess chromatin accessibility changes between hESCs and NPCs.Motif enrichment analysis was performed on differentially accessible chromatin regions to discover potential regulatory transcription factors.Finally,an integrated analysis of RNA-seq and ATAC-seq data and the protein-protein interaction(PPI)network were performed to identify key genes and regulatory pathways involved in the early stages of neural differentiation in vitro.Results·Both RT-qPCR and IF results indicated that the expression levels of pluripotency markers(NANOG and POU5F1)were high at the hESC stage but significantly decreased at the NPC stage,while early neural differentiation markers(PAX6,SOX1,and NES)were minimally expressed at the hESC stage but markedly upregulated at the NPC stage.RNA-seq analysis revealed that compared to the hESC stage,there were 5 597 genes upregulated and 3 654 genes downregulated at the NPC stage.Gene function enrichment analysis showed that the upregulated genes at the NPC stage were enriched in the functions related to neural development.ATAC-seq analysis demonstrated a total of 27 491 genomic regions had significant changes in chromatin accessibility during the differentiation from hESC to NPC,with 12 381 regions showing increased accessibility and 15 110 regions showing decreased accessibility.Motif enrichment analysis revealed that transcription factor genes such as DLX1 and LHX2 might play an important role in the differentiation process from hESCs into NPCs.Integrated analysis of RNA-seq and ATAC-seq data revealed that overlapping genes with high expression at the NPC stage were mainly enriched in axon guidance,forebrain development,and neuron migration.After neural differentiation,the expression levels of CTNND2 and LHX2 genes increased,and the chromatin accessibility of related genomic regions also increased.PPI network analysis indentified candidate downstream genes including PRKACA,CDH2,and ERBB4.Conclusion·The in vitro differentiation model of hESCs combined with high-throughput multi-omics sequencing technologies can be used to depict the changes in transcriptome and chromatin accessibility during the differentiation of hESCs into NPCs.In this process,the expression levels of genes related to axon guidance,forebrain development,and neuronal migration pathways increase and related chromatin accessibility is enhanced.