OBJECTIVES: To investigate the clinical features and prognosis of children with non-Down-syndrome-related acute megakaryoblastic leukemia (non-DS-AMKL). METHODS: A retrospective analysis was conducted on the medical data of 17 children with non-DS-AMKL who were admitted to Children's Hospital of The First Affiliated Hospital of Zhengzhou University from January 2013 to December 2023, and their clinical features, treatment, and prognosis were summarized. RESULTS: Among the 17 children with non-DS-AMKL, there were 8 boys and 9 girls. Fourteen patients had an onset age of less than 36 months, with a median age of 21 months (range:13-145 months). Immunophenotyping results showed that 16 children were positive for CD61 and 13 were positive for CD41. The karyotype analysis was performed on 16 children, with normal karyotype in 6 children and abnormal karyotype in 9 children, among whom 5 had complex karyotype and 1 had no mitotic figure. Detected fusion genes included EVI1, NUP98-KDM5A, KDM5A-MIS18BP1, C22orf34-BRD1, WT1, and MLL-AF9. Genetic alterations included TET2, D7S486 deletion (suggesting 7q-), CSF1R deletion, and PIM1. All 17 children received chemotherapy, among whom 16 (94%) achieved complete remission after one course of induction therapy, and 1 child underwent hematopoietic stem cell transplantation (HSCT) and remained alive and disease-free. Of all children, 7 experienced recurrence, among whom 1 child received HSCT and died of graft-versus-host disease. At the last follow-up, six patients remained alive and disease-free. CONCLUSIONS Non-DS-AMKL primarily occurs in children between 1 and 3 years of age. The patients with this disorder have a high incidence rate of chromosomal abnormalities, with complex karyotypes in most patients. Some patients harbor fusion genes or gene mutations. Although the initial remission rate is high, the long-term survival rate remains low.
Humans ; Male ; Female ; Leukemia, Megakaryoblastic, Acute/etiology* ; Child, Preschool ; Infant ; Child ; Retrospective Studies ; Prognosis ; Down Syndrome/complications*

Objective To investigate the auditory effects of cochlear implantation in quiet and noisy environ-ments in children with different bilateral intervention modes,as well as the factors influencing these effects.Methods A total of 185 children with bilateral severe to profound sensorineural hearing loss were divided into three groups:bimodal hearing mode group(BIM,n=55),simultaneous bilateral cochlear implantation group(SCI,n=70),and sequential bilateral cochlear implantation group(SBCI,n=60).The Parents' Evaluation of Aural/Oral Performance of Children(PEACH)was used to assess the PEACH scores of the three groups in quiet and noisy environments one year after binaural hearing aid intervention.Additionally,the effects of cochlear implantation age,preoperative residual hearing,hearing aid usage,rehabilitation training mode,family system,and other factors on auditory per-formance in quiet and noisy environments were analyzed.Results The PEACH scores in quiet environments were higher than those in noisy environments for all three groups(all P＜0.05).The SCI group had higher PEACH scores in both quiet and noisy environments compared to the BIM group(P＜0.05).Multifactorial analysis revealed differences in factors influencing auditory performance in quiet and noisy environments among the three groups.First cochlear implantation before 3 years of age,preoperative hearing aid usage,and home-based rehabilitation training mode were common favourable influencing factors for auditory performance in both environments.Preopera-tive residual hearing below 95 dB HL was an favourable influencing factor for auditory performance in quiet environ-ments in the BIM group.The higher the level of parental education,the better auditory performance in both quiet and noisy environments for the SCI and SBCI groups.Implantation interval of 24 months or less and hearing aid usage during the inter-implantation period were favourable influencing factors for auditory performance in both envi-ronments for the SBCI group.Conclusion Children with severe to profound prelingual deafness after simultaneous bilateral CI implantation had better hearing performance than bimodal listening in quiet and noise environments.Ear-ly implantation,preoperative or inter-implantation hearing aid usage are recommended to improve auditory perform-ance in noisy environments,regardless of the bilateral intervention mode.The interval between bilateral cochlear im-plantations should be less than 12 months.

OBJECTIVE: The relationship between non-high-density lipoprotein (NHDL) cholesterol to high-density lipoprotein cholesterol (HDL-C) ratio (NHHR) and stoke remains unknown. This study aimed to evaluate the association between the adult NHHR and stroke occurrence in the United States of America (USA). METHODS: To clarify the relationship between the NHHR and stroke risk, this study used a multivariable logistic regression model and a restricted cubic spline (RCS) model to investigate the association between the NHHR and stroke, and data from the National Health and Nutrition Examination Survey (NHANES) from 2005 to 2018. Subgroup and sensitivity analyses were conducted to test the robustness of the results. RESULTS: This study included 29,928 adult participants, of which 1,165 participants had a history of stroke. Logistic regression analysis of variables demonstrated a positive association between NHHR and stroke ( OR 1.24, 95% CI: 1.03-1.50, P = 0.026). Compared with the lowest reference group of NHHR, participants in the second, third, and fourth quartile had a significantly increased risk of stroke after full adjustments ( OR: 1.35, 95% CI: 1.08-1.69) ( OR: 1.83, 95% CI: 1.42-2.36) ( OR: 2.04, 95% CI: 1.50-2.79). In the total population, a nonlinear dose-response relationship was observed between the NHHR and stroke risk ( P non-linearity = 0.002). This association remained significant in several subgroup analyses. Further investigation of the NHHR may enhance our understanding of stroke prevention and treatment. CONCLUSION Our findings suggest a positive correlation between the NHHR and an increased prevalence of stroke, potentially serving as a novel predictive factor for stroke. Timely intervention and management of the NHHR may effectively mitigate stroke occurrence. Prospective studies are required to validate this association and further explore the underlying biological mechanisms.
Humans ; Stroke/blood* ; United States/epidemiology* ; Male ; Female ; Middle Aged ; Cross-Sectional Studies ; Nutrition Surveys ; Adult ; Aged ; Cholesterol, HDL/blood* ; Cholesterol/blood* ; Risk Factors

AIM:To investigate the effects of urolithin A on motor function and muscle fibrosis in dystrophin-deficient mdx mice,a model of Duchenne muscular dystrophy.METHODS:Twelve 26-week-old SPF male dystrophin gene deficient C57BL/10ScSnJNju-Dmdem3Cd4/Gpt(mdx)mice were randomly divided into model group and urolithin A treatment group,with 6 mice in each group.Additionally,six wild-type SPF male mice were selected as the normal con-trol.The motor ability of the mice was evaluated by pole climbing test,inverted suspension test,grip strength test and en-durance test.The body mass,mitochondrial relative copy number,ATP level and malondialdehyde(MDA)level were compared among the mice in different groups.Hematoxylin-eosin staining,Masson staining and immunohistochemistry were performed to analyze the atrophy and pathological changes of the gastrocnemius muscle.RESULTS:Compared with normal control group,the mdx mice in model group exhibited significantly impaired motor function,as evidenced by pro-longed pole-climbing time(P<0.01),reduced suspension time and forelimb/hindlimb grip strength(P<0.01),and in-creased number of electrical stimuli required to induce movement(P<0.01).Additionally,mitochondrial relative copy number and ATP level were significantly decreased(P<0.01),while MDA level was significantly elevated(P<0.01).Histological analysis revealed marked inflammatory cell infiltration and extensive tissue fibrosis in the gastrocnemius mus-cle.In contrast,urolithin A treatment significantly improved motor performance(P<0.01),attenuated inflammatory cell infiltration and muscle fibrosis,increased mitochondrial copy number,restored ATP level(P<0.05),and reduced MDA level(P<0.01).CONCLUSION:Urolithin A ameliorates motor dysfunction and alleviates muscle fibrosis in mdx mice,suggesting its potential therapeutic benefits for Duchenne muscular dystrophy.

Objective:To understand the awareness of influenza and influenza vaccine, influenza incidence, influenza vaccination status and intention and related factors in primary and secondary school teachers.Methods:Cross-sectional study design and stratified random sampling method were used to select 5 teachers from each of 51 primary schools and 40 middle schools in a district of Beijing from April 17 to 21, 2024. A questionnaire survey was conducted in the selected teachers with an estimated sample size of 1 040. The survey collected the information about their demographic characteristics, awareness of influenza and influenza vaccine, influenza incidence, influenza vaccination status and intention. Multivariate logistic regression model was used to analyze the factors related to influenza vaccination status and intention.Results:In the 975 primary and secondary school teachers, the awareness of susceptibility and severity of influenza and the benefits of influenza vaccination were high. In terms of safety of influenza vaccine, 48.00% (468/975) of the teachers believed that adverse reaction would occur after the vaccination. Influenza like illness occurred in 41.13% (401/975) of the teachers during 2023-2024, and in these teachers, 40.15% (161/401) asked for leave from work due to illness, 40.90% (164/401) sought medical care and 32.93% (54/164) were influenza virus positive. In the 975 teachers, 13.95% (136/975) were vaccinated against influenza during 2023-2024 and 34.46% (336/975) were willing to be vaccinated against influenza during 2024-2025. Multivariate logistic regression analysis showed that being teacher in charge of a class (a OR=1.58, 95% CI: 1.06-2.35), believing that influenza vaccination could effectively prevent influenza (a OR=1.99, 95% CI: 1.05-3.80), believing that teachers' vaccination could improve students' willingness to be vaccinated (a OR=1.98, 95% CI: 1.12-3.50) were the positive factors for teachers' vaccination, and worrying about adverse reactions after the vaccination (a OR=0.52, 95% CI: 0.34-0.80) was the negative factor. Doctors' recommendation (a OR=2.62, 95% CI: 1.41-4.86) and free influenza vaccination (a OR=10.26, 95% CI: 5.17-20.39) were positively correlated with teachers' intention to influenza vaccination. Conclusions:Primary and secondary school teachers in a district of Beijing had high awareness of influenza and influenza vaccine, but their awareness of influenza vaccine safety should be improved. The incidence of influenza-related infection was high in the primary and secondary school teachers. The influenza vaccination rate was low and the willingness to receive influenza vaccination in following year was not strong. For the better prevention and control of influenza in schools, it is necessary to strengthen the health education in primary and secondary school teachers to improve their awareness of influenza vaccine safety and vaccination rate.

Objective:To explore the efficacy and its related factors of rituximab (RTX) in the treatment of children with frequently relapsing nephrotic syndrome/steroid-dependent nephrotic syndrome (FRNS/SDNS).Methods:It was a single-center retrospective study. The clinical data of FRNS/SDNS children first treated with RTX in the First Affiliated Hospital of Sun Yat-sen University from November 1, 2016 to September 1, 2023 were collected. The number of relapse within 1 year before and after RTX treatment, the time to first relapse after RTX treatment, and the time to B-cell reconstitution were analyzed. At the first treatment, a single dose of RTX was given at 375 mg/m 2, with a maximum dose of 500 mg, once a week, for 1 to 4 doses. The count of CD19 + lymphocytes in the peripheral blood of the children was continuously monitored. If B-cell reconstruction was performed, the decision on whether to proceed to the next course of RTX treatment was made based on clinical manifestations. Kaplan-Meier method was used to analyze relapse-free survival rate after receiving RTX. Cox proportional hazards regression model was used to analyze the related factors of relapse after RTX treatment. Results:A total of 98 FRNS/SDNS children receiving RTX treatment were enrolled, including 75 males (76.5%). The age at onset was 4.0 (1.9, 7.1) years and age of receiving RTX was 11.3 (8.5, 13.5) years. There were 90 children (91.8%) achieving complete remission, while 8 patients (8.2%) did not respond to RTX treatment, and 3 patients (3.1%) progressed to end-stage kidney disease after receiving RTX. The relapse-free survival rates at 6 months and 1 year after RTX treatment were 83.3% (75/90) and 57.9% (22/38), respectively. The frequency of relapse 1 year after RTX treatment decreased compared to 1 year before RTX treatment ( Z=-7.398, P<0.001). Compared with children without relapse during the period of B-cell depletion, relapsed children had a higher number of relapse within one year after RTX treatment ( Z=5.246, P<0.001). The time to first relapse after RTX treatment was 8.3 (4.6, 13.9) months in 51 relapse patients. Compared with children receiving 1 dose of RTX in the first course, those receiving 2 or more doses had a longer time to the first relapse ( Z=2.983, P=0.003). There was no statistically significant difference in time to the first relapse between children who received mycophenolate mofetil therapy after RTX treatment and those who didn't ( P>0.05). The reconstruction time of B cells after the first course of RTX was 6.9 (5.3, 9.0) months. Compared to children receiving one dose of RTX in the first course, those receiving two or more doses had a longer B-cell reconstitution time ( Z=2.739, P=0.006). There was no statistically significant difference in B-cell reconstitution time between children who received mycophenolate mofetil therapy after RTX treatment and those who didn't ( P>0.05). Univariate Cox regression analysis showed that recurrence after calcineurin inhibitor (CNI) treatment before RTX treatment and the number of recurrence in one year before RTX treatment were correlated factors of recurrence after RTX treatment (both P<0.05). Multivariate Cox regression analysis showed that recurrence after CNI treatment before RTX treatment was an independent correlated factor of relapse after RTX therapy ( HR=3.496, 95% CI 1.245-9.818, P=0.018). Infusion reactions occurred in 10 patients (10.2%) and infections were observed in 24 patients (24.5%) during B cell depletion. No serious adverse events occurred. Conclusions:RTX is well tolerated and effective in treating FRNS/SDNS. Recurrence after CNI treatment before RTX treatment may be an independent related factor of relapse after RTX treatment.

Objective:To explore the mediating effect of psychological resilience between perceived stress and job burnout among standardized gastroenterology nursing training students.Methods:A total of 156 nursing trainees who received standardized training in the Department of Gastroenterology at Shaanxi Provincial People's Hospital between December 2022 and July 2024 were selected by the convenience sampling method. Data were collected using a general information questionnaire, the Connor-Davidson Resilience Scale, the Chinese Perceived Stress Scale, and the Maslach Burnout Inventory-General Survey. A Pearson correlation analysis was used to examine the relationship among perceived stress, psychological resilience, and job burnout in standardized gastroenterology nursing training students. AMOS 24.0 statistical software was used to construct a structural equation model to analyze the mediating effect of psychological resilience between perceived stress and job burnout in standardized gastroenterology nursing training students. The bias-corrected bootstrap method was applied to test the mediating effect of psychological resilience.Results:The averaged total scores of 156 standardized gastroenterology nursing training students were (29.89±0.30) for perceived pressure, (62.45±2.44) for psychological resilience, and (57.85±3.44) for job burnout. Of the trainees, 88 (56.41%) exhibited job burnout, including 39 (25.00%) with mild burnout, 41 (26.28%) with moderate burnout, and 8 (5.13%) with severe burnout. The Pearson correlation analysis showed that perceived stress was positively correlated with job burnout ( r=0.543, P<0.05) and negatively correlated with psychological resilience ( r=-0.443, P<0.05), and psychological resilience was negatively correlated with job burnout ( r=-0.478, P<0.05). The results of bias-corrected bootstrap test showed that psychological resilience had a partial mediating effect between perceived stress and job burnout among standardized gastroenterology nursing training students ( P<0.05), accounting for 32.63% of the total effect. Conclusions:Moderate job burnout was observed in standardized gastroenterology nursing training students. Job burnout was closely related to perceived stress and psychological resilience. Psychological resilience partly mediated the relationship between perceived stress and job burnout. Therefore, clinical managers may alleviate the perceived psychological pressure and subsequently reduce the occurrence of job burnout among standardized nursing training students by increasing psychological resilience.

Aberrant expression of Colgalt1 is closely associated with tumorigenesis and tumor progres-sion;however,the mechanism by which it regulates macrophages to influence tumor development remains poorly understood.This study aimed to establish a macrophage-specific Colgalt1 gene knockout mouse model to delve into the mechanisms through which Colgalt1 modulates macrophage function and subse-quently affects the occurrence and progression of tumor-related diseases.Initially,Colgalt1flox+mice were generated using gene editing techniques,followed by crossing with Lyz2-Cre+mice,which exhibit tissue-specific expression in the myeloid lineage(including monocytes and mature macrophages).Through this strategy,mice with the genotype Colgalt1-/-Lyz2-Cre+were successfully obtained,achieving conditional knockout of the Colgalt1 gene in macrophages.Colgalt1flox/flox Lyz2-Cre-mice were used as control.PCR and agarose gel electrophoresis were employed to identify the Flox and Cre genotypes of the knockout mice.RT-qPCR and Western Blot techniques were utilized to detect the expression levels of Colgalt1 in BMDMs from knockout mice at both the mRNA and protein levels,respectively.Western Blot results re-vealed a significant downregulation of Colgaltl expression in BMDMs from knockout mice compared to controls(P＜0.01).RT-qPCR results demonstrated a significant reduction in Colgalt1 mRNA levels in BMDMs from knockout mice compared to contro1s(P＜0.001),while no significant differences in Col-galt1 mRNA expression were observed in liver,lung,or spleen tissues between the two groups.Addition-ally,immunohistochemistry was employed to detect Colgalt1 expression in liver-specific macrophages,re-vealing an absence of Colgalt l-positive staining in liver macrophages from knockout mice.HE staining was used to observe cellular morphology in liver tissues from both groups of mice,showing no significant differences in cellular morphology or obvious pathological changes in tissues and organs.Moreover,the o-verall survival of the mice was not affected.Finally,RT-qPCR was used to assess the expression of mac-rophage-related inflammatory factors in BMDMs from both groups of mice.The results indicated that com-pared to controls,knockout mice exhibited downregulated expression of TNF-α(P＜0.05)and signifi-cantly upregulated expression of IL-10(P＜0.01),Arginase1(P＜0.001),and CD206(P＜0.001)in BMDMs,suggesting an anti-inflammatory trend and M2 polarization of macrophages following Colgalt 1 knockout.In summary,this study successfully established a macrophage-specific Colgalt1 gene knockout mouse model,providing a more reliable experimental animal model for in-depth exploration of the specific roles of Colgalt1 in macrophage functional regulation and the pathogenesis of tumor-related diseases.This model holds promise for identifying novel therapeutic targets and strategies for tumors and other diseases.

Corneal neovascularization(CNV)is a pathological condition characterized by the invasion of new blood vessels into the normally avascular corneal area from the corneal periphery,leading to severe vision loss and potentially blindness.Currently,surgical,physical,and pharmacological therapies are the main clinical approaches for treating CNV.Surgical treatment aims to remove abnormal vascular tissue or perform corneal trans-plantation to inhibit angiogenesis;however,it carries a risk of postoperative rejection.Physical therapy involves the direct application of non-invasive modalities,such as laser treatment,to the neovascularized area to suppress vascular growth.Nevertheless,this approach may cause damage to surrounding healthy tissues.Pharmacotherapy has recently become a research hotspot in CNV treatment due to its convenient administration.Clinically,the drugs used for CNV treatment mainly include anti-inflammatory agents,anti-VEGF drugs,and immunosuppressants,which inhibit CNV progression by targeting angiogenesis-related signaling pathways.However,these drugs often lead to drug resistance and toxic side effects.Therefore,there is an urgent need to develop more effective and safer therapeutic agents for CNV.This article reviews the current clinical treatment status of CNV and highlights recent advances in the use of small molecule extracts from traditional Chinese medicine for CNV therapy,aiming to provide potential candidate drugs and a scientific theoretical basis for clinical management of CNV.