Objective:To summarize the clinical and genetic characteristics of pseudoachondroplasia and multiple epiphyseal dysplasia caused by COMP gene variants in pediatric patients.Methods:This retrospective study concluded 15 pediatric patients with COMP-related pseudoachondroplasia and multiple epiphyseal dysplasia at Shanghai Children′s Medical Center, Shanghai Jiao Tong University School of Medicine from July 2013 to August 2024. This paper analyzed clinical manifestations, laboratory findings and genetic testing.Results:This cohort comprised 15 pediatric patients (8 males and 7 females) with a diagnostic age of 5.3 (1.8,9.3) years. The major clinical presentations included abnormal gait (15/15), brachydactyly (11/15), genu varum (12/15), irregular metaphyseal changes (14/14) and epiphyseal dysplasia (14/14). Genetic analysis revealed 13 cases of pseudoachondroplasia and 2 multiple epiphyseal dysplasias cases associated with COMP gene variants. Fifteen variants were identified (8 pathogenic and 7 likely pathogenic), including 2 novel variants (c.1223A>G, c.1378G>C). Thirteen of these patients had variations clustered in exons 8-14 encoding the calmodulin-like domains, with c.1414_1419dupGACGAC emerging as a hotspot variant.Conclusions:COMP-related pseudoachondroplasia and multiple epiphyseal dysplasia predominantly manifest with gait abnormalities and skeletal deformities. COMP gene pathogenic variations were mainly located in calmodulin-like domains.

OBJECTIVE To explore the clinical distribution and drug resistance of common species of pathogens iso-lated from a three-A hospital of Guangzhou from Jan.2017 to 2023 Dec.so as to provide bases for clinical diagno-sis and reasonable use of antibiotics.METHODS A total of 10,086 strains of aerobic bacteria were clinically isola-ted from the patients who were hospitalized in a three-A hospital of Guangzhou from 2017 to 2023.The constituent ratios of the common species of pathogens,specimen sources,distribution of departments and drug resistance rates to commonly used antibiotics were retrospectively analyzed.RESULTS Totally 10,086 strains of pathogens were isolated from the specimens of the hospitalized patients from 2017 to 2023.Klebsiella pneumoniae,Pseudo-monas aeruginosa,Escherichia coli,Acinetobacter baumannii and Staphylococcus aureus ranked the top 5 species of pathogens.The sputum,midstream urine and whole blood were the major specimen sources.The hospital-asso-ciated infection was highly prevalent in critical care medicine department,neurology department,geriatrics depart-ment,neurosurgery department and urology department.The result of drug resistance showed that the drug re-sistance rates of the K.pneumoniae and P.aeruginosa strains to various types of antibiotics showed upward trends(P＜0.05);the drug resistance rate of the A.baumannii strains to imipenem was decreased,while the drug resist-ance rates to most of the antibiotics were more than 45％.No gram-positive cocci strains that were resistant to vancomycin,teicoplanin or linezolid were found.CONCLUSIONS The common clinical isolates of pathogens are generally resistant to antibiotics.It is necessary for clinicians to attach great importance to the culture of pathogens and drug susceptibility testing and reasonably use antibiotics based on the result of drug susceptibility testing so as to reduce the occurrence and spread of drug-resistant strains.The hospital should strengthen the surveillance of drug resistance of bacteria so as to boost the clinical curative effect,standardize the management and use of antibi-otics and take effective measures to control of the hospital-associated infection.

Objective To investigate the effect of Ching Shum Pills(CSP)for alleviating non-alcoholic fatty liver disease(NAFLD)and the underlying mechanism.Methods In a mouse model of NAFLD,the therapeutic effect of CSP was evaluated by measuring serum glucose,lipid profiles(TC,TG,LDL-C,HDL-C),and hepatic function markers.Network pharmacology was employed to identify active compounds in CSP and their targets using TCMSP,HERB,SwissTargetPrediction,GeneCards,OMIM,and DisGeNET.Protein-protein interaction(PPI)networks,Gene Ontology(GO),and KEGG pathway analyses were conducted.Molecular docking(AutoDock Vina)was used to assess the compound-target binding affinities.Quantitative real-time PCR(qRT-PCR)was used to validate the mRNA expressions of the core genes in the liver tissue of the mouse models.Results In the mouse model of NAFLD,treatment with CSP significantly reduced body weight gain and serum TG levels of the mice,and high-dose CSP treatment resulted in obvious reduction of ALT levels and hepatic fat accumulation.Network pharmacology analysis identified quercetin and 2-monolinolenin as the key bioactives in CSP,which target TNF,AKT1,IL6,TP53,and ALB.Docking simulations suggested strong binding between the two core compounds and their target proteins.The results of qRT-PCR showed that high-fat diet induced significant downregulation of Tp53,Cpt1,and Ppara expressions in mice,which was effectively reversed by CSP treatment.Conclusion CSP can improve lipid metabolism disorders in NAFLD mice through a regulatory mechanism involving multiple targets and pathways to reduce liver fat accumulation and protect liver function.The key components in CSP such as quercetin and linolenic acid monoacylglycerol may participate in the regulation of such metabolic processes as fatty acid oxidation by targeting TP53.

OBJECTIVES: To investigate the effect of Ching Shum Pills (CSP) for alleviating non-alcoholic fatty liver disease (NAFLD) and the underlying mechanism. METHODS: In a mouse model of NAFLD, the therapeutic effect of CSP was evaluated by measuring serum glucose, lipid profiles (TC, TG, LDL-C, HDL-C), and hepatic function markers. Network pharmacology was employed to identify active compounds in CSP and their targets using TCMSP, HERB, SwissTargetPrediction, GeneCards, OMIM, and DisGeNET. Protein-protein interaction (PPI) networks, Gene Ontology (GO), and KEGG pathway analyses were conducted. Molecular docking (AutoDock Vina) was used to assess the compound-target binding affinities. Quantitative real-time PCR (qRT-PCR) was used to validate the mRNA expressions of the core genes in the liver tissue of the mouse models. RESULTS: In the mouse model of NAFLD, treatment with CSP significantly reduced body weight gain and serum TG levels of the mice, and high-dose CSP treatment resulted in obvious reduction of ALT levels and hepatic fat accumulation. Network pharmacology analysis identified quercetin and 2-monolinolenin as the key bioactives in CSP, which target TNF, AKT1, IL6, TP53, and ALB. Docking simulations suggested strong binding between the two core compounds and their target proteins. The results of qRT-PCR showed that high-fat diet induced significant downregulation of Tp53, Cpt1, and Ppara expressions in mice, which was effectively reversed by CSP treatment. CONCLUSIONS CSP can improve lipid metabolism disorders in NAFLD mice through a regulatory mechanism involving multiple targets and pathways to reduce liver fat accumulation and protect liver function. The key components in CSP such as quercetin and linolenic acid monoacylglycerol may participate in the regulation of such metabolic processes as fatty acid oxidation by targeting TP53.
Animals ; Non-alcoholic Fatty Liver Disease/drug therapy* ; Mice ; Drugs, Chinese Herbal/pharmacology* ; Lipid Metabolism/drug effects* ; Molecular Docking Simulation ; Disease Models, Animal ; Liver/metabolism* ; Male ; Lipid Metabolism Disorders/drug therapy* ; PPAR alpha/metabolism* ; Mice, Inbred C57BL ; Network Pharmacology

Objective:To explore the training experiences of palliative care nurses participating in a prognosis disclosure workshop based on Kolb's experiential learning model, and to provide references for future educational programs in palliative care nursing.Methods:Using purposive sampling, 11 nurses were recruited who had participated in the "Palliative Care Competency Training Program for Nurses" in Shanghai between March and May 2025 and selected the Third Affiliated Hospital of Naval Medical University as their clinical training site. Semi-structured interviews were conducted, and data were analyzed using Colaizzi's seven-step method.Results:A total of three major themes emerged: a sense of benefit from enhanced competencies; complex emotional experiences; recommendations for course optimisation.Conclusions:Workshops based on Kolb's experiential learning model offer positive value in palliative care education. They effectively improve core competencies such as communication and empathy. However, the emotional experiences of nurses must also be considered, and multifaceted optimizations to the training design are recommended to further enhance training outcomes.

Objective:To explore the mediating effect of dyadic coping between perceived social support and family cohesion and adaptability and happiness in spouses of patients with post-stroke motor impairments.Methods:From December 2023 to May 2024, a total of 366 spouses of patients with post-stroke motor impairments who received treatment at the First Affiliated Hospital of Zhengzhou University were recruited as study participants. Data were collected using the general information, perceived social support scale, dyadic coping inventory, Chinese version of the family cohesion and adaptability scale(FACESⅡ-CV), and memorial University of Newfoundland scale of happiness. The AMOS 21.0 software was utilized to construct a structural equation model to examine the mediating effect of dyadic coping between perceived social support, family cohesion and adaptability and happiness.Results:The spouses of patients with post-stroke motor impairments reported overall scores for perceived social support (62.80±12.80), dyadic coping (117.07±20.78), family cohesion and adaptability (115.96±19.51), and happiness (22.27±7.29). Family cohesion and adaptability was positively correlated with dyadic coping and perceived social support ( r=0.614, 0.520, both P<0.01).Spousal happiness was positively correlated with dyadic coping and perceived social support ( r=0.296, 0.202, both P<0.01).The spouses recognized that perceived social support had a positive correlation with dyadic coping ( r=0.479, P<0.01).Dyadic coping played a partial mediating effect between perceived social support and family cohesion and adaptability as well as happiness, accounting for 32.41% (0.211/0.651) and 44.39% (0.190/0.428) of the total effect, respectively. Conclusion:Dyadic coping is the mediating variable of family cohesion, adaptability and happiness of spouses of patients with post-stroke motor impairments. Medical staff should try to construct a dyadic coping intervention plan to improve the dyadic coping level of spouses of patients with post-stroke motor impairments, so as to improve their family happiness index.

Objective:To investigate the iodine nutrition level and the prevalence of thyroid nodules in pregnant women in Hubei Province, and to provide a basis for prevention and treatment of iodine deficiency disorders.Methods:According to the requirements of the National Iodine Deficiency Disorders Monitoring Program (2016 Edition), a cross-sectional survey of iodine nutrition status of pregnant women ( n = 321) was conducted from July to October 2020 in two mountainous counties (Tongcheng County and Xingshan County) and two plain counties (Liangzihu District and Xinzhou District) in Hubei Province. Among them, there were 43, 114, and 164 pregnant women in the early, middle, and late stages of pregnancy, respectively. Edible salt samples and once random urine samples were collected to detect salt iodine and urinary iodine, and thyroid ultrasound was performed to calculate the detection rate of thyroid nodules. Results:The coverage rate of iodized salt, qualified rate of iodized salt, and consumption rate of qualified iodized salt in Hubei Province were 99.69% (320/321), 95.94% (307/320) and 95.64% (307/321), respectively. The median urinary iodine level for pregnant women was 164.80 μg/L. Among them, the median urinary iodine levels in Liangzihu District, Tongcheng County, Xinzhou District, and Xingshan County were 175.90, 178.25, 155.80 and 143.00 μg/L, respectively. There was a statistically significant difference in urinary iodine levels among different regions ( H = 8.51, P = 0.037). The median urinary iodine levels of pregnant women in the early, middle, and late stages of pregnancy were 187.20, 144.45, and 172.05 μg/L, respectively. There was no statistically significant difference in urinary iodine levels among pregnant women in different stages of pregnancy ( H = 2.94, P = 0.230). Urinary iodine < 150, 150 - < 250, 250 - < 500, ≥500 μg/L accounted for 45.48% (146/321), 33.33% (107/321), 19.63% (63/321), 1.56% (5/321), respectively. The detection rate of thyroid nodules was 16.82% (54/321), and the goiter rate was 0.93% (3/321). Conclusions:In 2020, Hubei Province is in an appropriate state of iodine, and there are still a considerable proportion of pregnant women in a state of iodine deficiency. The detection rate of thyroid nodules is relatively low. It is necessary to continuously monitor the iodine nutrition of pregnant women, strengthen health promotion on the hazards of iodine deficiency during pregnancy, and minimize maternal and infant health damage caused by iodine deficiency.

Objective:To explore the training experiences of palliative care nurses participating in a prognosis disclosure workshop based on Kolb's experiential learning model, and to provide references for future educational programs in palliative care nursing.Methods:Using purposive sampling, 11 nurses were recruited who had participated in the "Palliative Care Competency Training Program for Nurses" in Shanghai between March and May 2025 and selected the Third Affiliated Hospital of Naval Medical University as their clinical training site. Semi-structured interviews were conducted, and data were analyzed using Colaizzi's seven-step method.Results:A total of three major themes emerged: a sense of benefit from enhanced competencies; complex emotional experiences; recommendations for course optimisation.Conclusions:Workshops based on Kolb's experiential learning model offer positive value in palliative care education. They effectively improve core competencies such as communication and empathy. However, the emotional experiences of nurses must also be considered, and multifaceted optimizations to the training design are recommended to further enhance training outcomes.

Objective:To explore the mediating effect of dyadic coping between perceived social support and family cohesion and adaptability and happiness in spouses of patients with post-stroke motor impairments.Methods:From December 2023 to May 2024, a total of 366 spouses of patients with post-stroke motor impairments who received treatment at the First Affiliated Hospital of Zhengzhou University were recruited as study participants. Data were collected using the general information, perceived social support scale, dyadic coping inventory, Chinese version of the family cohesion and adaptability scale(FACESⅡ-CV), and memorial University of Newfoundland scale of happiness. The AMOS 21.0 software was utilized to construct a structural equation model to examine the mediating effect of dyadic coping between perceived social support, family cohesion and adaptability and happiness.Results:The spouses of patients with post-stroke motor impairments reported overall scores for perceived social support (62.80±12.80), dyadic coping (117.07±20.78), family cohesion and adaptability (115.96±19.51), and happiness (22.27±7.29). Family cohesion and adaptability was positively correlated with dyadic coping and perceived social support ( r=0.614, 0.520, both P<0.01).Spousal happiness was positively correlated with dyadic coping and perceived social support ( r=0.296, 0.202, both P<0.01).The spouses recognized that perceived social support had a positive correlation with dyadic coping ( r=0.479, P<0.01).Dyadic coping played a partial mediating effect between perceived social support and family cohesion and adaptability as well as happiness, accounting for 32.41% (0.211/0.651) and 44.39% (0.190/0.428) of the total effect, respectively. Conclusion:Dyadic coping is the mediating variable of family cohesion, adaptability and happiness of spouses of patients with post-stroke motor impairments. Medical staff should try to construct a dyadic coping intervention plan to improve the dyadic coping level of spouses of patients with post-stroke motor impairments, so as to improve their family happiness index.

Objective:To analyze the clinical characteristics of acute leukemia complicated with arterial ischemic stroke (AIS) in children, and to provide a reference for its diagnosis, treatment, and prognosis.Methods:Case summary.This report presents three children with acute leukemia complicated with AIS admitted to the First Affiliated Hospital of Zhengzhou University from April 2015 to August 2024, and reviews the relevant literature at home and abroad to analyze the clinical characteristics, pathogenesis, and treatment of the disease.Results:All three cases were female, aged 4-14 years; two had acute lymphoblastic leukemia (ALL) and one had acute myeloid leukemia (AML). Hemiparesis was the main presenting symptom in all cases, occurring during induction therapy.Symptoms resolved completely after anticoagulant and symptomatic treatment, with no sequelae and good prognoses.A literature search identified 8 reported cases of pediatric acute leukemia complicated with AIS.Combining these with our 3 cases yielded a total of 11 cases: 5 males and 6 females; median age 7 years (range 2-15 years); 8 with ALL and 3 with AML.Clinically, all presented with hemiparesis.Vascular imaging in 6 patients showed involvement of the middle cerebral artery.In 8 cases of ALL complicated with AIS, the event occurred during induction therapy, which was considered associated with the use of Asparaginase and intrathecal Cytarabine.Anticoagulation was the main treatment.Symptoms resolved in 10 cases, 3 had neurologic sequelae, and 1 died.Conclusions:AIS complicating acute leukemia in children is often the first clinical manifestation of hemiparesis, which mainly occurs in the process of induction therapy, and may be related to the adverse reactions of chemotherapy drugs such as hypercoagulable state of the blood caused by mendonuclease and insufficient cerebral perfusion caused by intrathecal injection of Cytarabine, etc.; once hemiplegic neurological symptoms appear in the process of induction therapy of children′s acute leukemia, it is highly suspicious of the concomitant AIS, and earlycranial magnetic resonance examination can help to clarify the diagnosis.Although most symptoms resolve with treatment, some patients may develop neurological sequelae.