Objective:To investigate the clinical characteristics of urea cycle disorder (UCD), the efficacy and safety of glyceryl phenylbutyrate (GPB) therapy in pediatric patients with UCD.Methods:This study was a retrospective, single-arm, multicenter clinical study. The clinical data of 20 pediatric patients with UCD who received GPB treatment at 9 hospitals nationwide between December 2021 and August 2024 were collected. The clinical manifestations, laboratory results, and molecular genetic characteristics were analyzed, ammonia levels and other laboratory results were evaluated pre-post GPB therapy by paired t-tests or Wilcoxon tests. Results:Among the 20 pediatric patients with UCD, there were 8 males and 12 females, and the onset age was 2.8 (1.4, 5.7) years. The ammonia levels were 174 (125, 342) μmol/L at first onset. The symptoms included vomiting in 6 cases, drowsiness in 5 cases, epilepsy in 5 cases, developmental delay in 5 cases, psychiatric and behavioral abnormalities in 3 cases, and lethargy in 1 case, and 18 cases exhibited abnormal liver function. Twenty cases included 6 UCD subtypes, with 11 cases being ornithine transcarbamylase deficiency. A total of 27 variants were identified, 11 (41%) of which were novel. The age of patients who began GPB therapy was 4.0 (1.5, 6.6) years. Ten cases stopped GPB after 4.2 (3.4, 5.3) months, with 4 patients undergoing liver transplantation and 6 discontinuing for financial reasons. The remaining ten patients continued GPB therapy for 11.6 (8.6, 14.0) months. The duration of GPB treatment was 6.0 (4.2, 12.3) months, at the final visit, the levels of ammonia, platelets and aspartate aminotransferase were lower compared to those of pre-treatment (all P<0.05). The serum albumin level was higher than that of pre-treatment ( P=0.016). Two patients suffered only one episode of acute hyperammonaemia, with ammonia levels of 232 and 141 μmol/L, respectively. Nine cases experienced adverse effects potentially related to GPB, decreased appetite in 6 cases, vomiting in 3 cases, abnormal skin oil odor in 2 cases, somnolence, fatigue and diarrhea each in 1 case, with symptoms improved within 6 (3, 10) days. Conclusions:UCD primarily manifests with neurological and gastrointestinal symptoms, and early diagnosis of UCD could be achieved through the analysis of ammonia. GPB may effectively reduce ammonia levels in UCD pediatric patients, with favorable safety and tolerability.

Purpose To investigate the predictive value of the ratio of the square of the cross-sectional perimeter(C2)to the area(A)of the venous lumen(C2/A)in relation to lower extremity deep venous thrombosis(DVT)among orthopaedic inpatients.Materials and Methods A total of 150 inpatients without DVT from the Orthopedics Department of Xijing Hospital Affiliated to the Air Force Military Medical University were prospectively chosen from June 2020 to June 2021.Before the operation,the parameters including A,C,C2/A,inner diameter values,venous flow velocity and blood flow volume of the common femoral vein(CFV),superficial femoral vein(SFV)and popliteal vein(POV)were examined by ultrasound.After the operation,the formation of thrombus was monitored using ultrasound.Subsequently,the clinical data and ultrasound parameters of the thrombus group and the non-thrombus group were compared.Moreover,the receiver operating characteristic(ROC)curve was plotted to assess the efficacy and cut-off values of each parameter in predicting DVT.Results There were statistically significant differences between the two groups in CFV inner diameter,CFV blood flow,CFV-C,CFV-A,CFV-C2/A,SFV blood flow,SFV-C,SFV-C2/A,POV blood flow,POV-C,POV-A and POV-C2/A(t=2.64-10.41,all P＜0.05).When the cut-off values of the ultrasonic parameters CFV-C2/A,SFV-C2/A,and POV-C2/A were＞16.01,＞16.53,and＞16.54 respectively,the area under the curve for predicting DVT was the largest,which were 0.906,0.920,and 0.870 respectively.The sensitivities were 83.8%,78.4%,and 81.1%respectively,and the specificities were 82.3%,89.4%,and 85.8%respectively.Conclusion The novel ultrasonic parameter C2/A exhibits a relatively high predictive efficacy for DVT,and it is capable of furnishing novel reference information for the implementation of early DVT prevention in the clinical setting.

Medical Immunology,characterized by its fundamental and cutting-edge nature,not only lays a solid foundation for students to learn other basic medical courses and clinical medicine curricula,but also serves as a powerful platform for cultivating deep learning and scientific thinking.This study aims to leverage Medical Immunology as a knowledge platform,adopt an AI+en-hanced flipped classroom approach,and build a thought-oriented classroom to enhance students'thinking skills,thereby meeting the high-quality requirements of the new medical education initiative for medical students.

Medical Immunology,characterized by its fundamental and cutting-edge nature,not only lays a solid foundation for students to learn other basic medical courses and clinical medicine curricula,but also serves as a powerful platform for cultivating deep learning and scientific thinking.This study aims to leverage Medical Immunology as a knowledge platform,adopt an AI+en-hanced flipped classroom approach,and build a thought-oriented classroom to enhance students'thinking skills,thereby meeting the high-quality requirements of the new medical education initiative for medical students.

Purpose To investigate the predictive value of the ratio of the square of the cross-sectional perimeter(C2)to the area(A)of the venous lumen(C2/A)in relation to lower extremity deep venous thrombosis(DVT)among orthopaedic inpatients.Materials and Methods A total of 150 inpatients without DVT from the Orthopedics Department of Xijing Hospital Affiliated to the Air Force Military Medical University were prospectively chosen from June 2020 to June 2021.Before the operation,the parameters including A,C,C2/A,inner diameter values,venous flow velocity and blood flow volume of the common femoral vein(CFV),superficial femoral vein(SFV)and popliteal vein(POV)were examined by ultrasound.After the operation,the formation of thrombus was monitored using ultrasound.Subsequently,the clinical data and ultrasound parameters of the thrombus group and the non-thrombus group were compared.Moreover,the receiver operating characteristic(ROC)curve was plotted to assess the efficacy and cut-off values of each parameter in predicting DVT.Results There were statistically significant differences between the two groups in CFV inner diameter,CFV blood flow,CFV-C,CFV-A,CFV-C2/A,SFV blood flow,SFV-C,SFV-C2/A,POV blood flow,POV-C,POV-A and POV-C2/A(t=2.64-10.41,all P＜0.05).When the cut-off values of the ultrasonic parameters CFV-C2/A,SFV-C2/A,and POV-C2/A were＞16.01,＞16.53,and＞16.54 respectively,the area under the curve for predicting DVT was the largest,which were 0.906,0.920,and 0.870 respectively.The sensitivities were 83.8%,78.4%,and 81.1%respectively,and the specificities were 82.3%,89.4%,and 85.8%respectively.Conclusion The novel ultrasonic parameter C2/A exhibits a relatively high predictive efficacy for DVT,and it is capable of furnishing novel reference information for the implementation of early DVT prevention in the clinical setting.

Objective:To investigate the clinical characteristics of urea cycle disorder (UCD), the efficacy and safety of glyceryl phenylbutyrate (GPB) therapy in pediatric patients with UCD.Methods:This study was a retrospective, single-arm, multicenter clinical study. The clinical data of 20 pediatric patients with UCD who received GPB treatment at 9 hospitals nationwide between December 2021 and August 2024 were collected. The clinical manifestations, laboratory results, and molecular genetic characteristics were analyzed, ammonia levels and other laboratory results were evaluated pre-post GPB therapy by paired t-tests or Wilcoxon tests. Results:Among the 20 pediatric patients with UCD, there were 8 males and 12 females, and the onset age was 2.8 (1.4, 5.7) years. The ammonia levels were 174 (125, 342) μmol/L at first onset. The symptoms included vomiting in 6 cases, drowsiness in 5 cases, epilepsy in 5 cases, developmental delay in 5 cases, psychiatric and behavioral abnormalities in 3 cases, and lethargy in 1 case, and 18 cases exhibited abnormal liver function. Twenty cases included 6 UCD subtypes, with 11 cases being ornithine transcarbamylase deficiency. A total of 27 variants were identified, 11 (41%) of which were novel. The age of patients who began GPB therapy was 4.0 (1.5, 6.6) years. Ten cases stopped GPB after 4.2 (3.4, 5.3) months, with 4 patients undergoing liver transplantation and 6 discontinuing for financial reasons. The remaining ten patients continued GPB therapy for 11.6 (8.6, 14.0) months. The duration of GPB treatment was 6.0 (4.2, 12.3) months, at the final visit, the levels of ammonia, platelets and aspartate aminotransferase were lower compared to those of pre-treatment (all P<0.05). The serum albumin level was higher than that of pre-treatment ( P=0.016). Two patients suffered only one episode of acute hyperammonaemia, with ammonia levels of 232 and 141 μmol/L, respectively. Nine cases experienced adverse effects potentially related to GPB, decreased appetite in 6 cases, vomiting in 3 cases, abnormal skin oil odor in 2 cases, somnolence, fatigue and diarrhea each in 1 case, with symptoms improved within 6 (3, 10) days. Conclusions:UCD primarily manifests with neurological and gastrointestinal symptoms, and early diagnosis of UCD could be achieved through the analysis of ammonia. GPB may effectively reduce ammonia levels in UCD pediatric patients, with favorable safety and tolerability.

Objective To investigate the effect of radiation on cardiomyocyte apoptosis and its related mechanism.Methods Rat H9C2 cardiomyocytes were divided into blank control group,X-ray irradiation group(X-ray group),X-ray irradiation+microRNA(miRNA)-134-5p inhibitor group(X-inhibitor group)and X-ray irradiation+miRNA-134-5p inhibitor negative control group(X-NC group).H9C2 cardiomyocytes were irradiated with 6 Gy X-ray,and the changes of various indexes were detected 48 h after irradiation.Cell viability was detected by cell counting kit 8 assay.The apoptosis rate was detected by flow cytometry and Hoechst 33342 staining.The level of reactive oxygen species(ROS)in cells was detected by DCFH-DA fluorescence probe.The mitochondrial membrane potential was detected by JC-1 method.The activity of superoxide dismutase(SOD)and the level of malondialdehyde(MDA)in cells were measured by kits.The expression of miRNA-134-5p was detected by quantitative polymerase chain reaction.The protein expression of brain-derived neurotrophic factor(BDNF),protein kinase B(Akt),phosphorylated Akt(p-Akt),Bcl2 and Bax was detected by Western blotting.Results Compared with the blank control group,in the X-ray group the levels of ROS and MDA were significantly increased,the activity of SOD was significantly decreased,the decreased percentage in mitochondrial membrane potential was significantly increased,the number of micronuclei of DNA damage was significantly increased,and the apoptosis rate was significantly increased(all P＜0.01).Compared with the X-ray group,all the indexes of the X-inhibitor group were reversed(P＜0.05 or P＜0.01),while there was no significant difference in the above parameters in the X-NC group(all P＞0.05).Compared with the blank control group,the X-ray group had a significant increase in the miRNA-134-5p level and significant reductions in the protein level of BDNF,Bcl2/Bax ratio,and p-Akt/Akt ratio(all P＜0.01).Compared with the X-ray group,the X-inhibitor group had a significant reduction in the level of miRNA-134-5p and significant increases in the protein level of BDNF,Bcl2/Bax ratio,and p-Akt/Akt ratio(all P＜0.01),and there was no significant difference in all parameters in the X-NC group(all P＞0.05).Conclusion X-ray irradiation can induce oxidative stress,mitochondrial damage,and DNA damage,eventually leading to apoptosis in rat cardiomyocytes,and the mechanism may involve miRNA-134-5p/BDNF/Akt signaling pathway.

Objective: Victims with shock-able initial arrest rhythms (ventricular fibrillation or pulse-less ventricular tachycardia, VF/VT) have much better outcomes from out-of-hospital cardiac arrest (OHCA) than those with non-shock-able initial rhythms (pulse-less electrical activities, PEA or asystole). Prompt defibrillation is believed pivotal to terminate lethal shock-able arrest rhythms. In this study, we were to identify those pre-hospital epidemiological factors which were associated with likelihood of shock-able rhythms first recorded when out-of-hospital cardiac arrest occurred. Methods: A multicenter, secondary, retrospective and observational analysis was performed on all adult non-traumatic OHCA in the Resuscitation Outcome Consortium (ROC) PRIMED study from June 2007 through November 2009. Of a total of 17 177 OHCA cases in PRIMED study, 13 421 of them were enrolled in this study when those OHCA patients with DNR order, or initial rhythms unknown, or arrest caused by obvious cause were excluded. We reported their demographics and episode characteristics for all enrolled cases. Multivariable logistic regression was employed to identify factors which were associated with likelihood of VF/VT recorded as shock-able initial rhythms. Results: Among 13 421 adult non-traumatic OHCA cases with initial rhythms recorded, 3 527 (26.3%) of them had shock-able rhythms, while 9 894 (73.7%) patients were in non-shock-able rhythms. Of the OHCA with shock-able arrest rhythms, 1 850(52.5%) obtained return of spontaneous circulation (ROSC) in the field, while 912 (25.9%) survived to hospital discharge. On the other hand, 2489(25.2%) of the OHCA with non-shock-able arrest rhythms obtained ROSC in the field, while 325 (3.3%) of them survived to hospital discharge. Multivariable regression analysis revealed those factors which indicated likelihood of shock-able rhythms recorded were: age(OR: 0.991,95%CI 0.988-0.993), male gender (OR1.887,95%CI1.718-2.073), pre-hospital Emergency Medical System(EMS) response time(OR0.950,95%CI 0.931-0.970), cardiac arrest witnessed by EMS providers(OR1.709,95%CI 1.423-2.054) or bystander(OR3.199,95%CI 2.921-3.504) and those with bystanders CPR (OR1.357,95%CI 1.239-1.487),public place of cardiac arrest (OR2.705,95%CI 2.437-3.003). Conclusions Some demographics and pre-hospital characteristics were proved associated with likelihood of shock-able arrest rhythms first recorded in OHCA victims, which might help develop corresponding strategies to deliver prompt shock on initially shock-able patients and improve their outcomes.

Objective: To investigate the incidence and clinical characteristics of urothelial carcinoma (UC) accompanied with multiple primary carcinoma (MPC). Methods: The clinical data of 121 UC patients with MPC in Peking University Third Hospital from January 2010 to May 2018 were retrospectly analyzed. Results: UC patients with MPC accounted for 9.74% (121/1 242) of all the UC patients. The ratio of male to female patients was 2.10∶1 in the total MPC patients, but it was 1∶1 in the upper urinary tract MPC subgroup. The MPC patients were more common in elderly people, whose medium age was 68 (32-93) years old. Of all the location (131 person-time) of other tumors besides UC, the digestive system tumors occurred most frequently, accounting for 41.98% (55/131), followed by the urinary and male reproductive system tumors (20.61%, 27/131) and the female reproductive system (12.21%, 16/131). The proportion of the digestive system tumors (47.37%, 9/19) was the highest in the upper urinary tract MPC, with a total number of the other primary cancer of 19 person-time. However, the proportion of the urinary and male reproductive system tumors (37.14%, 13/35) was higher in the synchronous MPC group, with a total number of the other primary cancer of 35 person-time. Some patients had a history of radiotherapy and/or chemotherapy before UC was diagnosed. We also observed 2 cases of genetically confirmed Lynch syndrome. The median overall survival (mOS) of UC patients with MPC was 132 months, and the mOS of patients with UC as the first malignancy (including synchronous MPC and UC as the first malignancy in metachronous MPC) was 120 months. The mOS of the synchronous MPC group was 84 months, which was significantly shorter than 178 months of metachronous MPC group (χ² =14.029, P<0.001). Conclusions The incidence of UC accompanied with MPC is not low, and the most common sites of MPC are the digestive system and reproductive system. Therefore, screening for MPC in UC patients, especially those with personal or family history of tumors, as well as elderly patients, may help early diagnosis and treatment of MPC patients and improve their prognoses.

Objective To investigate the incidence and clinical characteristics of urothelial carcinoma (UC) accompanied with multiple primary carcinoma (MPC). Methods The clinical data of 121 UC patients with MPC in Peking University Third Hospital from January 2010 to May 2018 were retrospectly analyzed. Results UC patients with MPC accounted for 9.74%( 121／1 242) of all the UC patients. The ratio of male to female patients was 2.10 ∶ 1 in the total MPC patients, but it was 1 ∶ 1 in the upper urinary tract MPC subgroup. The MPC patients were more common in elderly people, whose medium age was 68 (32?93) years old. Of all the location (131 person?time) of other tumors besides UC, the digestive system tumors occurred most frequently, accounting for 41.98%( 55／131), followed by the urinary and male reproductive system tumors (20.61%, 27／131) and the female reproductive system ( 12.21%, 16／131). The proportion of the digestive system tumors (47.37%, 9／19) was the highest in the upper urinary tract MPC, with a total number of the other primary cancer of 19 person?time. However, the proportion of the urinary and male reproductive system tumors (37.14%, 13／35) was higher in the synchronous MPC group, with a total number of the other primary cancer of 35 person?time.Some patients had a history of radiotherapy and／or chemotherapy before UC was diagnosed. We also observed 2 cases of genetically confirmed Lynch syndrome. The median overall survival (mOS) of UC patients with MPC was 132 months, and the mOS of patients with UC as the first malignancy ( including synchronous MPC and UC as the first malignancy in metachronous MPC) was 120 months. The mOS of the synchronous MPC group was 84 months, which was significantly shorter than 178 months of metachronous MPC group (χ2＝14.029, P＜0.001). Conclusions The incidence of UC accompanied with MPC is not low, and the most common sites of MPC are the digestive system and reproductive system. Therefore, screening for MPC in UC patients, especially those with personal or family history of tumors, as well as elderly patients, may help early diagnosis and treatment of MPC patients and improve their prognoses.