Objective:To explore the clinical and genetic characteristics of three children with Leguis syndrome.Methods:Three children suspected as Legius syndrome at the Henan Children′s Hospital for precocious puberty or short stature from June 6, 2019 to August 25, 2022 were selected as the study subjects. Clinical data of the children were collected. All children were subjected to whole exome sequencing, and candidate variants were verified by Sanger sequencing.Results:All of the children (including 2 females and 1 male, and aged 4 years and 6 months, 8 years, and 14 years and 8 months, respectively) had typical café de lait spots. Child 1 also had precocious puberty, and children 2 and 3 had short statures. Genetic testing revealed that all of them had harbored heterozygous variants of the SPRED1 gene, including c. 751C>T (p.Arg251Ter194) in child 1, c. 229A>T (p.Lys77Ter368) in child 2, and c. 1044_1046delinsC (p.R349fs *11) in child 3. Based on the guidelines from the American College of Medical Genetics and Genomics (ACMG), the c. 751C>T (p.Arg251Ter194) variant was predicted to be likely pathogenic, whilst the other two were known pathogenic variants. Conclusion:All of the three children were diagnosed with Leguis syndrome due to variants of the SPRED1 gene, which had manifested as multiple café de lait spots in conjunct with precocious puberty or short statures.

Currently, the application rate of virtual reality technology in the fields of medical education and medical treatment is relatively low. In this study, based on the structure of the human stomach, virtual reality technology, sensing technology, big data technology, and cloud computing technology were integrated. A new form of medical education was established to include the online website platform for data storage and analysis and the offline VR glasses for the physical operation. Through the use of 3d Max technology, Unity3D technology, and C# language, we constructed a three-dimensional model of the human stomach to present the stomach in three dimensions. This enables the users to immerse in it to achieve true human-computer interactive learning. This study updates the concept of medical education, effectively improves the quality and efficiency of medical education, and facilitates the development of surgical programs and reduction in the risk of surgery, as well as provides experimental materials for scientific research. In the future, it can be further developed to include the three-dimensional structures of the circulatory system and other major systems in the human body.

Objective To observe the effects of Wuhu Decoction on autophagy and the expressions of IL-8,IL-23 and muc5ac in lung tissue of asthmatic mice induced by exosomes of bone marrow mesenchymal stem cells infected by RSV(BMSCs-Exo-RSV);To explore its mechanism in the treatment of asthma.Methods Totally 40 male SPF C57 mice were randomly divided into blank group,model group,Wuhu Decoction group and ribavirin group,with 10 mice in each group.The blank group was given PBS nasal drops,and the other groups were given BMSCs-Exo-RSV nasal drops once every other day for 7 times.24 hours after the end of modeling,Wuhu Decoction group was given Wuhu Decoction,and ribavirin group was given ribavirin solution,the blank group and model group were given distilled water,once a day,for consecutive 7 d.The general behavior of mice was observed,the peak expiratory flow(PEF)and forced vital capacity(FVC)were detected,HE and Masson staining were used to observe the inflammatory infiltration and collagen deposition in the lung tissue,RT-qPCR was used to detect the expressions of LC3A/B,beclin-1,p62,IL-8,IL-23 and muc5ac mRNA in lung tissue,Western blot was used to detect the expressions of LC3B,beclin-1 and p62 proteins in lung tissue,immunohistochemstry was used to detect the expression of IL-8,IL-23,muc5ac proteins in lung tissue.Results Compared with the blank group,the body mass of mice in the model group decreased,and showed behavioral changes such as shortness of breath,nodding wheezing,lifting of upper limbs,shrugging,vertical hair,scratching nose,etc.,the PEF and FVC were decreased(P<0.01),inflammatory infiltration and collagen deposition in lung tissue increased,the expressions of LC3A/B,beclin-1,IL-8,IL-23 and muc5ac mRNA in lung tissue increased,the expression of p62 mRNA decreased(P<0.01),while the expression of LC3BⅡ,beclin-1,IL-8,IL-23 and muc5ac proteins and the ratio of LC3BⅡ/Ⅰ increased(P<0.01),while the expression of LC3BⅠand p62 protein decreased(P<0.01).Compared with the model group,the symptoms of Wuhu Decoction group and ribavirin group were improved,the PEF and FVC were increased(P<0.01,P<0.05),the inflammatory infiltration and collagen deposition in lung tissue were reduced,the expression of LC3A/B,beclin-1,IL-8,IL-23 and muc5ac mRNA in lung tissue decreased(P<0.01),the expression of p62 mRNA increased(P<0.01),the expressions of LC3BⅡ,beclin-1,IL-8,IL-23 and muc5ac protein and the ratio of LC3BⅡ/Ⅰ decreased(P<0.05,P<0.01),while the expressions of LC3BⅠ and p62 protein increased(P<0.01).Conclusion BMSCs-Exo-RSV can promote autophagy and the expressions of IL-8,IL-23 and muc5ac in lung tissue of mice to induce asthma changes.Wuhu Decoction has therapeutic effect on asthmatic mice by inhibiting autophagy and reducing the expressions of IL-8,IL-23 and muc5ac.

AIM:To investigate the alleviating effect of ethyl acetate extract from Platycladus orientalis leaves(EAEPOL)on diabetic cardiomyopathy(DCM)and its mechanisms.METHODS:Healthy adult C57BL/6 mice were ran-domly divided into normal control group,DCM group,and EAEPOL group.Cardiac structure and function of the mice were assessed by echocardiography.Myocardial fibrosis was evaluated though myocardial hydroxyproline content determi-nation,myocardial Masson and Sirius red staining,and collagen type I(Col I)and collagen type Ⅲ(Col Ⅲ)immunohis-tochemistry.The degree of myocardial oxidative stress was assessed by measuring malondialdehyde(MDA),superoxide dismutase(SOD)and total antioxidative capacity(T-AOC)levels using kits,as well as detection of nuclear factor E2-re-lated factor-2(Nrf-2)and heme oxygenase-1(HO-1)expression by qRT-PCR and Western blot.Endothelial-mesenchy-mal transition(EndMT)was evaluated by detecting CD31 and α-smooth muscle actin(α-SMA)protein expression by Western blot,and cadherin 5(CDH5)and fibroblast specific protein 1(FSP1)mRNA expression by qRT-PCR,as well as α-SMA immunofluorescence staining.RESULTS:(1)Mouse echocardiography revealed that compared with normal control group,heart rate(HR)and ejection fraction(EF)were significantly reduced in DCM group(P<0.05 or P<0.01),while left ventricular anterior wall thickness at systole and diastole(LVAWs and LVAWd)and left ventricular pos-terior wall thickness at systole and diastole(LVPWs and LVPWd)were significantly increased(P<0.05).Compared with DCM group,the mice in EAEPOL group showed significant increases in HR and EF(P<0.01),and marked decreases in LVAWs,LVAWd,LVPWs and LVPWd(P<0.05).(2)Compared with normal control group,the content of hydroxypro-line in mouse myocardium,the collagen area ratio shown by Sirius red and Masson staining,and the immunohistochemical positive area ratio of Col I and Col Ⅲ in DCM group were significantly increased(P<0.01).Compared with DCM group,the above myocardial fibrosis indicators in EAEPOL group were significantly reduced(P<0.05 or P<0.01).(3)Com-pared with normal control group,the myocardial MDA content and the expression of Nrf-2 in DCM group were significantly increased,while the SOD activity,the T-AOC and the expression of HO-1 was significantly decreased(P<0.01).Com-pared with DCM group,the myocardial MDA content in EAEPOL group was significantly reduced,while the SOD activity,the T-AOC,and the HO-1 and Nrf-2 expression were significantly enhanced(P<0.05 or P<0.01).(4)Compared with normal control group,the myocardial expression of CD31 and CDH5 in DCM group was significantly reduced,the expres-sion of α-SMA and FSP1 was significantly enhanced(P<0.05 or P<0.01),and the α-SMA positive area ratio by immuno-fluorescence staining was also increased(P<0.01).Compared with DCM group,EAEPOL significantly up-regulated the expression of CD31 and CDH5 and down-regulated the expression of α-SMA and FSP1,and the α-SMA positive area ratio by immunofluorescence staining was evidently decreased(P<0.05 or P<0.01).CONCLUSION:EAEPOL may attenuate myocardial fibrosis and improve cardiac function in DCM mice by suppressing oxidative stress and alleviating EndMT.

Following the principles of evidence-based medicine,in accordance with the structure and drafting rules of standardized documents,based on literature research,according to the characteristics of chronic cough in children and issues that need to form a consensus,the TCM Guidelines for Diagnosis and Treatment of Chronic Cough in Children was formulated based on the Delphi method,expert discussion meetings,and public solicitation of opinions.The guideline includes scope of application,terms and definitions,eti-ology and diagnosis,auxiliary examination,treatment,prevention and care.The aim is to clarify the optimal treatment plan of Chinese medicine in the diagnosis and treatment of this disease,and to provide guidance for improving the clinical diagnosis and treatment of chronic cough in children with Chinese medicine.

Objective:To evaluate the efficacy and safety of Xiao′er Huangjin Zhike Granules in the treatment of cough caused by acute bronchitis in children, which is defined in TCM terms as a syndrome of phlegm-heat obstructing the lung.Methods:This was a block-randomized, double-blind, placebo-controlled, multicenter clinical trial.From January 2022 to September 2023, 359 children aged 3 to 7 years old diagnosed as acute bronchitis (lung-obstructing phlegm-heat syndrome) were enrolled from 21 participating hospitals and randomly assigned to the experimental group and placebo group in a 3︰1 ratio, and respectively treated with Xiao′er Huangjin Zhike Granules and its matching placebo.Cough resolution/general resolution rate after 7 days of treatment was used as the primary efficacy outcome for both groups.Results:(1)On the seventh day of treatment, the rate of cough disappearance/basically disappearance in the experimental group and placebo group were 73.95% and 57.61% retrospectively, which had statistically significance ( P=0.001).(2)After 7 days of treatment, the median duration of cough disappearance/basic disappearance were 5 days and 6 days in the two groups , with a statistically significant difference ( P=0.006).The area under the curve of cough symptom severity time was 7.20 ± 3.79 in the experimental group and 8.20±4.42 in the placebo group.The difference between the two groups was statistically significant ( P=0.039).(3) After 7 days of treatment, the difference between TCM syndrome score and baseline was -16.0 (-20.0, -15.0) points in the experimental group and -15.0 (-18.0, -12.0) points in the placebo group, with significant difference between the two groups ( P=0.004).In the experimental group, the clinical control rate, the markedly effective rate, the effective rate and the ineffective rate were 49.04%, 28.35%, 16.48% and 6.13% severally; and in the placebo group, the clinical control rate, the markedly effective rate, the effective rate and the ineffective rate were 38.04%, 26.09%, 29.35%, and 6.52% separately, which had statistically significant ( P=0.014).(4) There was no significant difference in the incidence of adverse events or adverse reactions during the trial between both groups.Moreover, while adverse reactions in the form of vomiting and diarrhea were occasionally reported, no serious drug-related adverse event or adverse reaction was reported.(5)The tested drug provided good treatment compliance, showing no statistically significant difference from the placebo in terms of compliance rate. Conclusions:Based on the above findings, it can be concluded that Xiao′er Huangjin Zhike Granules provides good safety, efficacy, and treatment compliance in the treatment of cough caused by acute bronchitis, and lung-obstructing phlegm-heat syndrome, in children.

Objective:Although Wu Hu Tang has the effect of treating cough variant asthma(CVA),its specific mechanism of action remains unclear.Methods:Predicted targets,autophagy genes,cough variant asthma genes and differentially expressed genes(DEGs)of CVA of Wu Hu Tang were obtained and mined from the Traditional Chinese Medicine Systematic Pharmacology Data-base and Analysis Platform(TCMSP),Bioinformatics Analysis Tool for Molecular Mechanism of Traditional Chinese Medicine(BAT-MAN-TCM)and TCM Integrated Pharmacology Research Platform v2.0(TCMIP),PubChem,Chemspider,Swiss Target Prediction,GEO,GeneCards and other databases.The intersection tool of line Venn diagram was used to obtain the key genes.Using Cyto-scape3.8.0 software build active ingredient-key network;Wu Hu Tang intervention CVA autophagy targets with autophagy genes corre-lation analysis;Use the STRING database,Cytohubba plug-in build protein interaction network and core gene screening.GO and KEGG enrichment analysis of key targets were performed using R Biomanager and ClusterProfiler package,and finally molecular dock-ing was performed.Results:A total of 42 active ingredients,536 potential action targets,7 236 autophagy-related genes,1 987 CVA genes and 460 DEGs of Wu Hu Tang Tang were collected and screened.Thirteen key targets were obtained after taking the intersec-tion,and 12 genes were found to be statistically significant by validated used wilcoxon non-parametric test.The results of the enrich-ment analysis showed that these target genes mainly functioned in cellular autophagy through the VEGF signaling pathway,and some amino acid metabolic pathways.Conclusion:Wu Hu Tang is capable of multi-component,multi-level and multi-target involvement in autophagy-related processes to achieve intervention in CVA,which promotes the development of the idea of combining Chinese and Western medicine in the treatment of CVA and provides a new idea for the development of new clinical drugs.

OBJECTIVE: To explore the feasibility of using indocyanine green angiography in mapping the superficial temporal vessels and assisting design and harvesting of the superficial temporal artery based forehead flap. METHODS: A clinical data of 14 patients with facial soft tissue defects repaired with superficial temporal artery based forehead flaps between October 2015 and November 2022 was retrospectively analyzed. There were 9 males and 5 females with a median age of 9.5 years (range, 3-38 years). The forehead flaps were used to reconstruct facial soft tissue defects following excision of facial scar (8 cases) or congenital melanocyte nevus (6 cases). The size of defects ranged from 3 cm×2 cm to 24 cm×9 cm. Before operation, the indocyanine green angiography was used to map the superficial temporal artery and vein, and to analyze the relationship of the arteries and veins. The forehead flaps with unilateral superficial temporal fascia as the pedicle was transferred to repair the small facial defect in 2 cases. The facial pedicle contained the frontal branch of the superficial temporal artery and 2 cm of the superficial temporal fascia around the vessel, and the tiny accompanying vein of the frontal branch of the superficial temporal artery was used as the outflow of the flap. The forehead flaps with the skin pedicle including bilateral or unilateral superficial temporal fascia and the overlying skin was pre-expanded and transferred to repair the large facial defect in 12 cases. The skin pedicle contained the frontal branch of superficial temporal artery and one of main branches of superficial temporal vein. Among the 12 cases, the frontal branch of superficial temporal vein was used as the outflow in 4 cases, and the parietal branch was used as the outflow in 8 cases. The size of the flaps ranged from 3 cm×2 cm to 30 cm×13 cm. The skin pedicles were divided at 3 weeks after the flap transfer. RESULTS: Indocyanine green angiography could clearly showed the course and branching of the superficial temporal artery and vein. Individual differences existed in the location where the frontal branch of the superficial temporal artery entered the forehead. The superficial temporal vein had great variability and did not follow the artery. One patient had expander-related complication, which resulted in 3-cm flap necrosis. The necrotic tissue was debrided and repaired with skin grafting. The other flaps totally survived and the incisions healed by first intention. All patients were followed up 2-24 months, with a median of 11.5 months. The color, texture, and thickness of the flaps matched well with those of recipient sites. Hypertrophic scar was not observed in recipient or donor site. All patients were satisfied with the reconstructive outcomes. CONCLUSION Indocyanine green angiography can clearly visualize the course and the branches of the superficial temporal arteries and veins, which can help surgeons understand the position, distribution, and concomitant relationship of the superficial temporal vessels, and make a rational surgical plan of the forehead flap.
Male ; Female ; Humans ; Child, Preschool ; Child ; Adolescent ; Young Adult ; Adult ; Temporal Arteries/surgery* ; Indocyanine Green ; Forehead/surgery* ; Retrospective Studies ; Skin Transplantation ; Angiography ; Soft Tissue Injuries/surgery* ; Perforator Flap/blood supply* ; Treatment Outcome

Objective By summarizing the literature on the treatment of bronchial asthma with"Tonifying the spleen to help the lung"as the guiding ideology and"tonifying spleen drugs"published in recent 25 years,analyzing the domestic research situation,to understanding the research status of the mechanism of"Tonifying the spleen to help the lung"in the treatment of asthma,so as to provide ideas for the research of traditional Chinese medicine in the treatment of asthma.Methods Taking"Tonifying the spleen to help the lung","tonifying the spleen","strengthening the spleen","transporting the spleen","benefiting the spleen","Shenling Baizhu Powder","Yigong Powder","Ginseng Schisandra Decoction","Maimendong Decoction","Liujunzi Decoction"and"asthma"as the keywords,searching the literature in the Chinese Journal Full-text Database(CNKI),extracting the data recorded in the literature,including prescriptions,drug composition,test indexes and experimental results,and summarize and analyze the data.Results A total of 54 literatures were included in this literature study.The prescriptions of"Tonifying the spleen to help the lung"method in the treatment of asthma are complex.The classic prescriptions often involved include Liujunzi Decoction,Yupingfeng Powder,Yigong Powder,Buzhong yiqi decoction,Shenling baizhu powder,Maimendong decoction,Ginseng Schisandra decoction and Erchen decoction.The commonly used drugs include Atractylodes macrocephala,Astragalus membranaceus,Poria cocos,licorice,tangerine peel,etc.Experimental research mainly involves inflammatory cells,immunoglobulins,cytokines,signaling pathway,T cell subsets,cell receptors,intestinal flora and other aspects,and the specific detection indicators are complex and diverse.Conclusion The method of"Tonifying the spleen to help the lung"is effective in the treatment of bronchial asthma.Many studies show that its effect has a clear scientific basis,but its mechanism is complex and diverse and is gradually becoming clear.

Sepsis is currently the leading cause of death in the intensive care unit, and its survivors also experience long-term immunosuppression and high rates of recurrent infections. At present, the clinical treatment of sepsis is still based on antibiotics, intravenous rehydration, and vasopressors, and there is no targeted drug treatment. However, as the rate of antibiotic resistance continues to increase, immunotherapy is highly anticipated as a new treatment. Patients with sepsis are often accompanied by acute leukocyte immune dysfunction and immunosuppression, which may be an important risk factor for the increasing morbidity and mortality of patients. Targeted inhibition of specific cell surface inhibitory immune checkpoint receptors and ligands, such as programmed death receptor-1 (PD-1), programmed death-ligand 1 (PD-L1), and other targets, can improve the host’s resistance to infection. In this paper, the research progress of PD-1 and PD-L1 in the immune response to sepsis was summarized to provide a theoretical basis for their further application in the treatment of sepsis in the future.