To summarize the clinical characteristics and genetic mutation spectrum of patients with SELENON-related myopathy(SELENON-RM), in order to improve awareness among physicians.

Objective: To understand the current status of psychological sub health among secondary vocational school students and explore its related factors, so as to provide a scientific basis for improving their mental health. Methods: From September to December 2024, a convenience sampling method was used to conduct a questionnaire survey among 5 141 secondary vocational school students from 16 schools across 8 provinces and municipalities (Fujian, Chongqing, Guangdong, Guangxi, Hubei, Jiangxi, Shanghai, Zhejiang). A self designed questionnaire was used to collect data on students demographic characteristics and lifestyle behaviors. The Multidimensional Sub health Questionnaire of Adolescents was adopted to assess psychological sub health status. Multivariate binary Logistic stepwise regression analysis was performed to explore the related factors of psychological sub health among secondary vocational school students. Results: The detection rate of psychological sub health among secondary vocational school students was 11.0%, with detection rates of emotional problems, conduct problems, and social adaptation difficulty being 12.3 %, 12.5%, and 9.5%, respectively. Logistic regression analysis revealed that boys and students whose fathers had an educational level of primary school or below, and junior high school, had relatively lower risks of developing psychological sub health or emotional problems, conduct problems, and social adaptation difficulty ( OR =0.55-0.64, all P <0.05). Conversely, students with urban household registration, those with left behind experience, those with poor self rated family economic status, those from other family structures, those classified as overweight and obesity, and those with sleep disorders, frequent consumption of sugar sweetened beverages, frequent consumption of takeout/delivery food, smoking, alcohol consumption, and low to moderate physical activity levels exhibited significantly higher risks across psychological sub health or emotional problems,conduct problems, and social adaptation difficulty ( OR =1.25-4.79, all P <0.05). Conclusions The psychological sub health condition of secondary vocational school students should not be neglected. Psychological sub health of secondary vocational school students is related to multiple factors such as demographic characteristics and lifestyle behaviors. Multi dimensional intervention measures should be implemented to improve their mental health.

Objective:To analyze the efficacy of endovascular treatment for venous outflow tract obstruction after liver transplantation.Methods:A retrospective analysis was conducted on the data of 7 patients with venous outflow tract obstruction after liver transplantation admitted to Beijing Friendship Hospital, Capital Medical University from November 2020 to December 2024. Among them, there were 5 males and 2 females, with the age of (22.3±8.1) years. The primary diseases included 2 cases of Budd-Chiari syndrome, 1 case of hepatic veno-occlusive disease, 1 case of portal veno-hepatic sinus vascular disease, 1 case of ornithine carbamoyltransferase deficiency, 1 case of primary biliary cirrhosis, and 1 case of autoimmune cirrhosis. Analyze the patient's clinical manifestations, obstruction of venous outflow tract, hemoglobin levels within one week before and one week after the operation, endovascular treatment conditions, and intraoperative complications such as abdominal hemorrhage and vascular injury. Ultrasound was used to measure the depth of ascites and pleural effusion. All patients were followed up immediately after the operation through phone calls or follow-up visits. The clinical symptoms, abdominal vascular ultrasound, enhanced CT and survival status of the patients were followed up.Results:All 7 patients were diagnosed with venous outflow tract obstruction by intraoperative angiography, including 1 cases of inferior vena cava obstruction, 2 cases of hepatic vein obstruction, and 4 cases of vena cava combined with hepatic vein obstruction. A total of 12 endovascular treatments were performed on 7 patients. Among them, 4 patients received balloon dilation and angioplasty once, 1 patient received balloon dilation and angioplasty twice, 1 patient underwent hepatic vein stent implantation after 2 hepatic vein balloon dilation and angioplasty, and 1 patient underwent intrahepatic portosystemic shunt via jugular vein after 2 hepatic vein balloon dilation and angioplasty. The abdominal distensionof the patients were all relieved after the operation, the ascites and pleural effusion decreased, and the edema symptoms of the lower extremities disappeared. There were no intraoperative complications. The preoperative hemoglobin of 7 patients was (113.4±34.0) g/L, and the postoperative hemoglobin was (126.6±34.8) g/L, which increased significantly compared with that before the operation, and the difference was statistically significant ( t=-0.71, P=0.038). Seven patients were followed up for 6 to 24 months, with a median of 12 months. None of them had obvious symptoms including abdominal distension. Abdominal ultrasound and CT indicated that the blood flow of the transplanted liver was unobstructed, and no patient died. Conclusion:Venous outflow tract obstruction after liver transplantation can cause severe symptoms. Endovascular treatment is an effective treatment for venous outflow tract obstruction after liver transplantation.

Objective:To analyze the extraction, chemical composition, antioxidant, and anti-inflammatory activity of the TCM formula essential oil for the treatment of upper respiratory tract infections (URTI); To provide a scientific basis for its further development.Methods:The formula essential oil was extracted using the steam distillation method and analyzed for chemical composition by gas chromatography-mass spectrometry (GC-MS). The DPPH, ABTS scavenging ability, and hydroxyl radical scavenging ability of volatile oils were measured. The effect of the essential oil on the viability of RAW264.7 cells was assessed using the CCK-8 assay. ELISA and Western blot methods were used to determine the effects of volatile oil on LPS induced inflammatory cytokines IL-6 and TNF-α.Results:The average extraction rate of the formula essential oil was 1.12%, with a density of 0.973 2 g/ml. Twelve main chemical components were identified, with 1,8-cineole (42.9%) and patchoulol (19.9%) being the predominant constituents. The essential oil exhibited DPPH and ABTS radical scavenging capacities of 52% and 59%, respectively, and a hydroxyl radical scavenging capacity exceeding 70%. Essential oil could reduce the levels of IL-6 and TNF-α ( P<0.05). Conclusion:TCM formula essential oil for the treatment of URTI contains multiple bioactive components and demonstrates significant antioxidant and anti-inflammatory effects.

[Objective] To explore the predictive value of HALP score for prognosis in patients with multiple myeloma (MM). [Methods] A retrospective analysis was conducted on laboratory indicators and related clinical data of newly diagnosed multiple myeloma (NDMM) patients, treated at the Affiliated Hospital of North Sichuan Medical College from January 2016 to October 2023, prior to their first treatment. The HALP score was calculated, and the optimal cutoff value for HALP was determined using X-tile software. Survival analysis was performed using Kaplan-Meier curves for high HALP and low HALP groups. Univariate and multivariate analyses were conducted using the Cox regression model, and a forest plot was generated using Graphpad Prism to illustrate factors that may impact patient prognosis. The predictive ability of HALP score combined with β2-microglobulin and ECOG score for prognosis in MM patients was evaluated using receiver operating characteristic curve (ROC) analysis. [Results] A total of 203 MM patients were included, with the optimal cutoff value for HALP score being 29.15 (P<0.05). Among them, 101 patients were in the low HALP score group, and 102 patients were in the high HALP score group. The results of univariate and multivariate analysis using the Cox regression model showed that a HALP score <29.15 was an independent risk factor for progression-free survival (PFS) and overall survival (OS) (P<0.05). ROC curve analysis indicated that the combination of HALP score with β2-microglobulin and ECOG score had a higher predictive value for prognosis in MM patients compared to using HALP score alone. [Conclusion] The HALP score is closely related to the prognosis of patients with NDMM. A low HALP score indicates a poorer prognosis, while the combination of HALP score with β2-microglobulin and ECOG score provides a higher predictive value when assessed together.

Objective To establish a method for inducing mouse bone marrow monocytes differentiating into Kupffer cells in vitro,for the study of Kupffer cells.Methods Monocytes were separated from mouse bone marrow and induced by transforming growth factor 1(TGF-β1),macrophage colony-stimulating factor(M-CSF)and delta-like protein 4(DLL4).Induced monocyte-derived Kupffer cells(iMoKCs)were identified by real-time fluorescent quantitative polymerase chain reaction(qPCR),immunofluorescence staining and flow cytometry.Immunofluorescence staining and flow cytometry were used to evaluate the phagocytic capacity of iMoKCs.Cell counting kit-8(CCK-8)was used to evaluate the proliferation capacity.Results Bone marrow monocytes were isolated,after 24 h combined induction,iMoKCs express C-type lectin domain family 4,member f(CLEC4F),C-type lectin domain family 1,member b(CLEC1B)and V-set and immunoglobulin domain containing 4(VSIG4)at mRNA level,and protein CLEC4F.Phagocytic function of iMoKCs were detected by flow cytometry,and nearly 80% of total iMoKCs show bioparticle acceptance.The number of iMoKCs were detected by CCK-8,the counts were 1.4,2.0 and 2.9 times of the initial number(0 h)at 24,48 and 72 h.Conclusion These cells display the immunophenotype of Kupffer cells and show the ability of phagocytosis and proliferation induction through M-CSF,TGF-β1 and DLL4,which could replace primary Kupffer cells for in vitro research.

Objective::To analyze fetal renal abnormality genetic features and the prenatal characteristics of the 17q12 microdeletion syndrome.Methods::This prospective cohort study examined prenatal ultrasound findings of renal abnormalities in pregnant women who underwent single nucleotide polymorphism (SNP) array or copy number variation sequencing (CNV-seq) testing on amniotic fluid or fetal tissue at Tianjin Central Obstetrics and Gynecology Hospital between January 2016 and August 2022. The study cohort comprised women with advanced maternal age, fetal ultrasound anomalies, high-risk non-invasive prenatal testing results, or suspected 17q12 microdeletion syndrome. Comprehensive clinical data, including maternal age, detailed ultrasound findings, and pregnancy outcomes, were systematically collected. SNP-array analysis was conducted using an Affymetrix CytoScan 750 K Array Chip to identify CNVs and loss of heterozygosity, while CNV-seq was performed on the Illumina HiSeq 2000 platform. Detected variants were classified according to the American College of Medical Genetics and Genomics guidelines. Statistical analyses were performed using SPSS version 27.0.Results::Abnormal renal development was identified in 141 patients, among whom 26 exhibited hyperechogenic kidneys (HCK). Of these, 12 cases were associated with 17q12 microdeletion syndrome, while the remaining 14 were linked to other chromosomal abnormalities. When excluding patients with HCK, those diagnosed with polycystic kidney disease demonstrated a higher prevalence of chromosomal abnormalities compared to those with multicystic dysplastic kidney and renal dysplasia. Although isolated conditions such as horseshoe kidney, hydronephrosis, ectopic kidney, and unilateral kidney typically presented with normal chromosomal findings, the incidence of chromosomal abnormalities increased when these conditions coexisted with other anomalies. A detailed analysis of the correlation between 17q12 microdeletion syndrome and HCK revealed that 12 out of the 14 patients diagnosed with 17q12 microdeletion syndrome exhibited HCK. Genetic testing confirmed the syndrome in seven patients, with five cases attributed to novel mutations and two cases resulting from inherited mutations.Conclusion::Fetal HCK was closely associated with the 17q12 microdeletion syndrome, and polycystic kidney disease showed a higher rate of chromosomal abnormalities. Chromosome test results were mostly normal in patients with other renal abnormalities, such as kidney dysplasia, horseshoe kidneys, hydronephrosis, kidney deficiency, and ectopic kidneys. Prenatal diagnosis is recommended, especially in cases of non-isolated fetal renal abnormalities. This study provides strong evidence supporting a link between fetal renal abnormalities and genetic syndromes.

The mortality rates are significantly elevated with the rapid progression of acute liver failure in the absence of timely diagnosis and treatment. Liver transplantation is an effective therapeutic approach that can halt disease progression, but transplantation timing is a crucial factor affecting prognosis. Patients with acute liver failure should be promptly transferred to hospitals equipped for liver transplantation while simultaneously preparing for the procedure during the course of treatment to avoid missing the opportunity to save lives when the condition suddenly worsens. Auxiliary liver transplantation preserves the patient's native liver while transplanting a new liver. Therefore, patients are expected to gradually reduce immunosuppressants following the regeneration of the autologous liver, so avoiding the problem of lifelong use of immunosuppressants. This is also a unique advantage, offering benefits to patients undergoing auxiliary liver transplantation therapy for acute liver failure, while simultaneously presenting challenges for clinicians in terms of technical skill and comprehensive management.

Objective:To evaluate the safety and efficacy of using small and ultra-small sized grafts for in situ auxiliary liver transplantation in the treatment of portal hypertension.Methods:A prospective single-arm cohort study was conducted. Patients who underwent liver transplantation at Beijing Friendship Hospital from December 2014 to July 2025 were included. Intraoperative portal vein pressure was routinely monitored, with the target regulation for portal vein blood flow set at<15 mmHg (1 mmHg=0.133 kPa) and follow-up continued until September 2025. The primary endpoints were the patient's status and graft survival. The secondary endpoints were small-for-size syndrome and perioperative complications. The small-for-size syndrome was graded according to the 2023 International Liver Transplantation Society consensus statement.Results:A total of 33 cases were enrolled. Among them, 22 had ultra-small size grafts, 11 had small-size grafts, 28 had living donor grafts, and five had split grafts. The graft-to-recipient weight ratio in living donor liver transplantation was 0.31%～0.79%, while in split liver transplantation it was 0.45%～1.02%. Intraoperative portal vein pressure of ≥15 mmHg was observed in 11 cases, who underwent portal vein blood flow adjustment via splenic artery ligation (2 cases), partial splenectomy (8 cases), and/or restrictive portocaval shunting (1 case), after which all patients achieved the target portal vein pressure. All cases completed at least one month of follow-up, with 28 cases following for more than one year, and the median follow-up period was 36.5 months. Early-stage postoperative small-for-size syndrome occurred in eight cases (24.2%, 8/33), all classified as grade A, with improvements following supportive treatment. Severe complications (Clavien-Dindo≥Ⅲ) occurred in three cases (9.1%, 3/33). The one-year survival rate was 92.9% (26/28). The overall survival rate at the end of follow-up was 90.9% (30/33). No patients experienced graft loss or death due to small-for-size syndrome. Graft tissue tested negative for hepatitis B core antibody and covalently closed circular DNA, and hepatitis B surface antigen seroconversion was achieved following second-stage residual liver resection and under a combined strategy of potent nucleos(t)ide analogs and hepatitis B immunoglobulin in ten cases of hepatitis B-related disease.Conclusions:With standardized portal vein blood flow monitoring and individualized portal vein blood flow adjustment, in situ auxiliary liver transplantation can safely and effectively use small and even ultra-small sized grafts, thereby significantly expanding graft sources and ensuring donor and recipient safety. These findings warrant further validation and promotion in multicenter controlled studies.

Objective To investigate the impacts of percutaneous endoscopic interlaminar discectomy(PEID)on pain level and lumbar function in patients with lumbar disc herniation(LDH).Methods From January 2022 to August 2023,84 patients were diagnosed with L4/5 LDH based on clinical data and imaging information were selected as the study subjects.And they were assigned into 42 cases in experimental group and 42 cases in traditional groups complying with treatment methods.The traditional group underwent traditional open approach treatment,while the experimental group underwent PEID.The surgical indicators(including hospitalization time,surgical time,bed rest time,and intraoperative fluoroscopy frequency),the levels of interleukin-6(IL-6)and tumor necrosis factor-α(TNF-α)were compared between the two groups,the visual analogue scale(VAS)score and Oswestry disability index(ODI)were used to quantify the pain level of waist and lower limbs and lumbar function,and the excellent and good rate of clinical satisfaction and the incidence of complications were compared.Results The hospitalization time and bed rest time of the experimental group were obviously shorter than those of the traditional group,while the fluoroscopy frequency was less than that of the traditional group,and surgical time was longer than that of the traditional group,the differences were statistically significant(P＜0.05).After treatment,the pain VAS score and ODI of waist and lower limbs,the IL-6,and TNF-α in the two groups were lower than those before treatment,the experimental group was lower than the traditional group,the differences were statistically significant(P＜0.05).The excellent and good rate of the experimental group and the traditional group were 88.10％and 83.33％,respectively,with no statistically significant difference(P＞0.05).There was no statistically significant difference in the total incidence of complications between the two groups(P＞0.05).Conclusion PEID surgery can effectively alleviate nerve compression,alleviate pain of waist and lower limbs,promote the recovering of lumbar function,and facilitate postoperative recovery in LDH patients.