Objective To explore the anesthesia management experience in the interventional treatment of pediatric congenital heart diseases (CHD) percutaneously guided by transthoracic echocardiography (TTE) on a mobile operating platform. Methods From March to July 2023, a total of 13 patients from remote areas underwent interventional treatment for CHD on the mobile operating platform of Fuwai Yunnan Cardiovascular Hospital. Patients who received non-tracheal intubation general anesthesia were retrospectively included. Results Eight children who had difficulty cooperating with the surgery (due to young age, emotional tension, crying) received monitored anesthesia care with local anesthesia supplemented by sedative and analgesic drugs while maintaining spontaneous breathing under the monitoring and management of an anesthesiologist (i.e., non-tracheal intubation general anesthesia). Among them, there were 5 males and 3 females, with an age of (6.95±3.29) years and a body weight of (19.50±6.04) kg. Through transthoracic echocardiography, they were diagnosed with atrial septal defect (6 patients), residual shunt after patent ductus arteriosus ligation (1 patient), and severe pulmonary valve stenosis (1 patient). The surgery proceeded smoothly, with satisfactory anesthesia and surgical effects, complete analgesia, and satisfactory postoperative recovery. There was 1 patient of body movement and 1 patient of respiratory depression during the operation, and both patients completed the surgery successfully after treatment. All children had no serious surgery- and anesthesia-related complications. The anesthesia time was 40.5 (34.5, 47.5) min, the surgery time was 39.0 (33.0, 45.5) min, and the recovery time was 43.0 (28.0, 52.5) min Conclusion Interventional surgery for CHD guided by TTE at a mobile platform is a minimally invasive approach without radiation damage. Non-tracheal intubation general anesthesia with spontaneous breathing can be safely and effectively implemented in children who cannot cooperate.

Objective:To analyze the application of Sengstaken-Blakemore tubes combined with endoscopic intervention on the prognosis of patients with acute esophageal and gastric variceal rupture and bleeding due to portal hypertension in liver cirrhosis, and to explore the impact of Sengstaken-Blakemore tubes combined with endoscopic intervention on the prognosis of patients.Methods:A retrospective study included patients with liver cirrhosis who visited the Zhongshan Hospital, Fudan University from January 2023 to July 2024 due to acute upper gastrointestinal bleeding, had no response to drug treatment and unstable hemodynamics, and received the Sengstaken-Blakemore tubes compression hemostasis. According to whether endoscopic intervention was received or not, the patients were divided into the endoscopic intervention group and the non-endoscopic intervention group. The medication and subsequent treatment of the patients were analyzed, and the survival of the patients 28 days after bleeding was followed up. Kaplan-Meier survival analysis and Cox regression analysis were used to evaluate the effect of endoscopic intervention on the 28-day mortality rate of patients.Results:A total of 31 cases successfully had three-lumen two-cystic tubes indwelled. Among them, 8 cases further received endoscopic examination and intervention, and 23 cases did not receive endoscopic examination. During the 28-day follow-up after bleeding, a total of 20 cases died. The Kaplan-Meier survival analysis showed that the 28-day mortality rate in the endoscopic intervention group was significantly lower than that in the non-endoscopic intervention group (25.0% vs 78.3%, P=0.007). Multivariate Cox regression analysis showed that endoscopic intervention was an independent protective factor for 28-day mortality ( HR: 0.14, 95% CI: 0.03-0.63, P=0.01). In addition, the severity of bleeding and prothrombin time were also important factors affecting the prognosis of patients. Conclusions:The Sengstaken-Blakemore tubes, as an important hemostasis method for acute esophageal and gastric variceal rupture and bleeding, can buy time for subsequent endoscopic treatment, but the risk of complications is relatively high. Creating conditions for early endoscopic intervention as early as possible can significantly reduce the mortality rate of patients and improve the prognosis of patients with liver cirrhosis.

Objective:To analyze the application of Sengstaken-Blakemore tubes combined with endoscopic intervention on the prognosis of patients with acute esophageal and gastric variceal rupture and bleeding due to portal hypertension in liver cirrhosis, and to explore the impact of Sengstaken-Blakemore tubes combined with endoscopic intervention on the prognosis of patients.Methods:A retrospective study included patients with liver cirrhosis who visited the Zhongshan Hospital, Fudan University from January 2023 to July 2024 due to acute upper gastrointestinal bleeding, had no response to drug treatment and unstable hemodynamics, and received the Sengstaken-Blakemore tubes compression hemostasis. According to whether endoscopic intervention was received or not, the patients were divided into the endoscopic intervention group and the non-endoscopic intervention group. The medication and subsequent treatment of the patients were analyzed, and the survival of the patients 28 days after bleeding was followed up. Kaplan-Meier survival analysis and Cox regression analysis were used to evaluate the effect of endoscopic intervention on the 28-day mortality rate of patients.Results:A total of 31 cases successfully had three-lumen two-cystic tubes indwelled. Among them, 8 cases further received endoscopic examination and intervention, and 23 cases did not receive endoscopic examination. During the 28-day follow-up after bleeding, a total of 20 cases died. The Kaplan-Meier survival analysis showed that the 28-day mortality rate in the endoscopic intervention group was significantly lower than that in the non-endoscopic intervention group (25.0% vs 78.3%, P=0.007). Multivariate Cox regression analysis showed that endoscopic intervention was an independent protective factor for 28-day mortality ( HR: 0.14, 95% CI: 0.03-0.63, P=0.01). In addition, the severity of bleeding and prothrombin time were also important factors affecting the prognosis of patients. Conclusions:The Sengstaken-Blakemore tubes, as an important hemostasis method for acute esophageal and gastric variceal rupture and bleeding, can buy time for subsequent endoscopic treatment, but the risk of complications is relatively high. Creating conditions for early endoscopic intervention as early as possible can significantly reduce the mortality rate of patients and improve the prognosis of patients with liver cirrhosis.

Objective:To explore the changes of iron metabolism in patients with hepatitis B cirrhosis and esophageal and gastric varices complicated with portal vein thrombosis.Methods:This study was a cross-sectional study. 253 patients with hepatitis B cirrhosis with esophageal and gastric varices who were hospitalized in the Zhongshan Hospital, Fudan University from January 1, 2020 to December 31, 2021 were included in this study. They were divided into portal vein thrombosis group ( n=57) and non portal vein thrombosis group ( n=196) according to the presence or absence of portal vein thrombosis. The iron metabolism characteristics of the two groups were compared, and subgroups were analyzed according to the presence or absence of ascites, platelet count level, D-dimer level, and Child grade. The factors related to portal vein thrombosis were screened through multivariate logistic regression analysis. Results:The ratio of Child pugh B/C, ascites, D-dimer and platelet count in patients with hepatitis B cirrhosis and esophageal and gastric varices complicated with portal vein thrombosis was higher (all P<0.05). Patients with portal vein thrombosis had higher levels of soluble transferrin receptor [2.4(1.8, 3.6)mg/L vs 1.8(1.3, 2.7)mg/L, P=0.006], and lower levels of ferritin [33.1(18.9, 63.3)ng/ml vs 57.7(19.4, 142.5)ng/ml, P=0.038]. Layered analysis showed that ascites, platelet count levels, D-dimer levels, and Child-pugh grade did not affect the negative correlation trend between ferritin and portal vein thrombosis, and the positive correlation trend between soluble transferrin receptors and portal vein thrombosis. Moreover, soluble transferrin receptors showed a statistically significant positive correlation with portal vein thrombosis in the absence of ascites, low D-dimer levels, and Child-pugh grade A. Multivariate analysis suggested that after weighing Child-pugh grading, platelet count, and D-dimer levels, ferritin ( OR=0.943, 95% CI: 0.904-0.983, P=0.006) and soluble transferrin receptor ( OR=1.034, 95% CI: 0.001-1.067, P=0.044) were independently associated with portal vein thrombosis. Conclusions:In patients with hepatitis B cirrhosis with esophageal and gastric varices, the characteristics of iron metabolism in patients with portal vein thrombosis are different from those in patients without thrombosis, with higher levels of soluble transferrin receptor and lower levels of ferritin.

Objective:To explore the predictive value of multimodal ultrasound (MMU) for upper urinary tract damage (UUTD) in children with neurogenic bladder (NB).Methods:This was a case-series study.From January 2022 to December 2023, 87 children with NB admitted to the First Affiliated Hospital of Zhengzhou University were examined by MMU.During the filling of bladder, vesical volume (VV), bladder wall thickness (BWT), shear wave velocity (SWV) of the anterior wall, resistance index (RI), and vascularization index (VI) were measured.After the emptying of bladder, VV and anterior wall SWV were measured, and ultrasound bladder compliance (△C) was calculated.The anterior posterior diameter (APD) of the renal pelvis and ureteral diameter (UD) were also measured.According to the upper/lower urinary tract dysfunction classification criteria, NB children were divided into a UUTD group and a non-UUTD (NUUTD) group.The differences in clinical data and related examinations between the 2 groups were analyzed to screen out independent risk factors, and an early warning model was established based on these factors.The prediction efficiency of the model and the urodynamic study (UDS) for UUTD was compared.Results:(1) There were 47 children in the UUTD group and 40 children in the NUUTD group.There was no significant difference in gender, age and body mass index between the 2 groups (all P>0.05).(2) In the UUTD group, the total glomerular filtration rate (tGFR) was (70.45±16.17) mL/min, the incidence of hydronephrosis was 38.30%, and the incidence of ureteral dilatation was 23.40%.No morphological changes were found in the imaging examination of the urinary system in the NUUTD group, and its tGFR was (100.55±16.27) mL/min.There was a significant difference in tGFR between the 2 groups ( P<0.05).(3) The filling VV, emptying VV, mean BWT, filling SWV, emptying SWV, VI, mean RI, △C, maximum cystometric capacity (MCC), maximum detrusor pressure during filling (Pdet.max), bladder compliance (BC), and detrusor leak point pressure (DLPP) in the NUUTD group were (218.43±87.53) mL, (14.62±6.14) mL, (3.08±0.65) mm, (2.64±0.54) m/s, (1.88±0.41) m/s, (6.20±1.04)%, 0.68±0.04, (147.58±49.18) mm 2·s, (309.50±66.54) mL, (59.83±19.79) cmH 2O(1 cmH 2O=0.098 kPa), (25.80±10.34) mL/cmH 2O, and (34.00±6.16) cmH 2O, respectively.Compared with the NUUTD group, the UUTD group showed decreased filling VV [(167.21±85.63) mL], △C [(78.49±31.86) mm 2·s], VI [(5.01±0.81) %], MCC [(255.32±75.10) mL], and BC [(12.57±6.44) mL/cmH 2O], and increased emptying VV [(19.50±7.65) mL], mean BWT [(4.02±0.82) mm], filling SWV [(3.99±1.07) m/s], emptying SWV [(2.15±0.35) m/s], mean RI (0.70±0.08), Pdet.max [(75.94±26.23) cmH 2O], and DLPP [(48.13±12.61) cmH 2O] (all P<0.05).(4) The decreased BC ( OR=0.841, 95% CI: 0.562-1.256, P=0.045), △C ( OR=0.427, 95% CI: 0.202-0.904, P=0.026) and VI ( OR=0.461, 95% CI: 0.091-2.325, P=0.010) and the increased DLPP ( OR=1.139, 95% CI: 0.894-1.451, P=0.040), filling SWV ( OR=1.895, 95% CI: 1.082-3.321, P=0.007) and mean BWT ( OR=1.191, 95% CI: 0.850-1.669, P=0.025) were independent risk factors for UUTD.Among MMU parameters, filling SWV had the highest prediction efficiency for UUTD, with a threshold of 3.33 m/s, sensitivity of 72.34% and specificity of 92.50%. Conclusions:MMU can well predict the occurrence of UUTD in children with NB, and filling SWV has the highest prediction efficiency.

[Objective] To evaluate the efficacy and safety of zero-pressure percutaneous nephrolithotomy (PCNL) combined with hands-free pneumatic lithotripsy in the one-stage treatment of calculous pyonephrosis in the non-acute infection phase. [Methods] Clinical data of 15 patients' treated during Feb.2022 and Dec.2023 were retrospectively analyzed.Double-sheath negative pressure PCNL was adopted, with zero-pressure gravity perfusion where the perfusion height was at the level of the patients' kidney, not exceeding 20 cm above the kidney.Pneumatic lithotripsy was performed without the need to hold the device with hand. [Results] The median stone diameter was 28 (range 22—40) mm, with a median stone density of 1028 (range 765—1305) Hu.All operations were successful.The median operation time was 55 (range 35—90) min.Postoperative low fever (Clavien grade Ⅰ) occurred in 1 case, and high fever (Clavien grade Ⅱ) occurred in 1 case, with no complications of Clavien grade Ⅲ or above.The initial stone-free rate was 73.3%, and the stone-free rate after one month was 93.3%. [Conclusion] For patients with calculous pyonephrosis in the non-acute infection phase, one-stage treatment using zero-pressure PCNL combined with hands-free pneumatic lithotripsy demonstrates good efficacy and safety.

Objective To explore the effects of anticoagulation treatment to postoperative bleeding events in liver cirrhosis patients with gastric varices and portal vein thrombosis.Methods Patients diagnosed with portal vein thrombosis and treated with endoscopic cyanoacrylate injection at Zhongshan Hospital,Fudan University due to gastric variceal bleeding from January 2023 to December 2023 were included.Clinical data of patients were collected,and patients were divided into anticoagulant group and non-anticoagulant group based on whether anticoagulant treatment was performed within 48 h after treatment.Re-bleeding in patients was evaluated in 6 weeks of follow-up.Cox regression was used for univariate and multivariate analysis of re-bleeding within 6 weeks after treatment.Results A total of 160 patients were included,of whom 65 patients received anticoagulation treatment within 48 h after endoscopic cyanoacrylate injection.There were no statistically significant differences in gender,etiology of liver cirrhosis,dosage of cyanoacrylate and sclerosing agents,and Child-Pugh grading between the two groups.There was no statistically significant difference in re-bleeding rate within 6 weeks after treatment between the two groups(1.54％vs 1.05％,P=0.795).Multivariate Cox regression analysis showed that the large amount of cyanoacrylate was a risk factor for re-bleeding within 6 weeks after endoscopic treatment(HR=5.862,P=0.015).Conclusions For patients with liver cirrhosis,gastric varices,and portal vein thrombosis,who receive endoscopic cyanoacrylate injection,early anticoagulation does not increase the risk of re-bleeding after treatment,while a large amount of cyanoacrylate injection may be a risk factor for re-bleeding.However,sample should be increased to verify.

Objective:To investigate correlation between neutrophil extracellular traps(NETs) formation and T cell subsets in mice with experimental autoimmune thyroiditis(EAT) and the impact of active vitamin D intervention.Methods:Six-week-old female BALB/c mice were randomly divided into Control group, EAT group and 1, 25 dihydroxy vitamin D 3[1, 25(OH) 2D 3] treatment group(VitD group; n=6/group). HE staining was used to observe thyroid pathology. Plasma thyroglobulin antibody(TGAb), thyroid peroxidase antibody(TPOAb), and 1, 25(OH) 2D 3 were measured by ELISA. Peripheral NETs formation, Th1, Th2, and Th17 cell ratio from spleen were measured by flow cytometry. Correlation between NETs formation rate and Th1, Th2, and Th17 cell ratio was analyzed. Results:Compared with Control group, mice in EAT group had significantly increased thyroid inflammation scores, thyroiditis morbidity, TPOAb, TGAb levels, NETs formation rate, Th2(CD4 + IL-4 + or CD4 + IL-13 + )and Th17 cell proportions( P were <0.001, 0.002, 0.007, <0.001, <0.001, 0.003, 0.001, and 0.002, respectively), and significant decreased 1, 25(OH) 2D 3, Th1 cell proportions, Th1/Th2(CD4 + IL-4 + ), Th1/Th2(CD4 + IL-13 + ), and Th1/Th17 ratios( P were 0.010, 0.018, 0.010, 0.005, and 0.007, respectively). Compared with the EAT group, the VitD group had lower thyroid inflammation scores, TPOAb, TGAb levels, NETs formation rate, Th2(CD4 + IL-4 + or CD4 + IL-13 + ) and Th17 cell proportions( P were 0.044, 0.007, <0.001, 0.001, 0.014, 0.008, and 0.001, respectively), and significant higher Th1 cell ratio, Th1/Th2(CD4 + IL-13 + ) and Th1/Th17 ratio( P were 0.011, 0.009, and 0.003, respectively). The Th1/Th2(CD4 + IL-4 + ) was not significantly increased in VitD group compared with EAT group( P=0.174). NETs formation rate was positively correlated with Th2(CD4 + IL-4 + or CD4 + IL-13 + ) and Th17 cell proportion( r were 0.65, 0.59, and 0.61; and P were 0.004, 0.010, and 0.007, respectively), but not with Th1 cell proportion( r=-0.47, P=0.051). Conclusion:EAT mice were more prone to NETs formation. Active vitamin D may relieve immune imbalance with increased Th2 and Th17 cell ratio and decreased Th1 cell ratio by reducing the formation of NETs in EAT mice. Vitamin D played the protective role in thyroid by reducing thyroid pathological damage and thyroid autoantibody levels, and relived overall lymphocyte imbalance.

Objective:This multi-centre study was conducted to assess the efficacy of various preoperative/pre-transfusion screening methods for blood transmitted disease.Methods:From July 2021 to December 2021, plasma samples of patients admitted to 10 hospitals were collected for screening preoperative/pre-transfusion blood transmitted disease. Nucleic acid detection technology was used to detect hepatitis B virus (HBV) DNA, hepatitis C virus (HCV) RNA and human immunodeficiency virus (HIV)(1+2) RNA, and the results were compared with the immuno-serological methods. χ 2 test and Kappa test were used to analyze the efficacy of these two methods. Results:A total of 8 655 valid specimens were collected from 10 hospitals. There was a statistically significant difference in the positive detection rate of HCV between the two methods ( P<0.001). There was no significant difference in the positive detection rate of HBV and HIV assessed by the two methods ( P>0.05), but the number of positive cases detected by HBV DNA and HIV RNA (218 and 4 cases) was significantly higher than the corresponding serological results (216 and 2 cases). At the same time, there were HBV, HCV and HIV immuno-serological omissions by the immuno-serological methods, among which 28 cases were HBsAg negative and HBV DNA positive, 2 cases were HCV antibody negative and HCV RNA positive, and 2 cases were HIV antigen/antibody negative and HIV RNA positive. In addition, in the 66 samples with inconsistent results from the two detection methods, 83.3% (55/66), 68.2% (45/66), 63.6% (42/66) and 62.1% (41/66) of patients aged was>45 years, tumor, surgery and male, respectively. Conclusions:Compared with immuno-serological tests, nucleic acid tests have the advantage in terms of sensitivity on detecting HBV, HCV and HIV infection and could reduce missed detection. The risk of transmission can be reduced by adding HBV, HCV, and HIV nucleic acid tests to preoperative/pre-transfusion immuno-serological tests screening for patients over 45 years of age and tumor patients.

OBJECTIVE: To report on a rare case of Neurofibromatosis type 2 (NF2) manifesting as oculomotor nerve palsy and explore its genetic basis. METHODS: A patient with NF2 who had presented at Beijing Ditan Hospital Affiliated to Capital Medical University on July 10, 2021 was selected as the study subject. Cranial and spinal cord magnetic resonance imaging (MRI) was carried out on the patient and his parents. Peripheral blood samples were collected and subjected to whole exome sequencing. Candidate variant was verified by Sanger sequencing. RESULTS: MRI revealed bilateral vestibular Schwannomas, bilateral cavernous sinus meningiomas, popliteal neurogenic tumors, and multiple subcutaneous nodules in the patient. DNA sequencing revealed that he has harbored a de novo nonsense variant of the NF2 gene, namely c.757A>T, which has replaced a codon (AAG) encoding lysine (K) at position 253 with a stop codon (TAG). This has resulted in removal of the Merlin protein encoded by the NF2 gene from position 253 onwards. The variant was not found in public databases. Bioinformatic analysis suggested that the corresponding amino acid is highly conserved. Based on the guidelines from the American College of Medical Genetics and Genomics (ACMG), the variant was rated as pathogenic (PVS1+PS2+PM2_Supporting+PP3+PP4). CONCLUSION The heterozygous nonsense variant c.757A>T (p.K253*) of the NF2 gene probably underlay the disease in this patient with an early onset, atypical but severe phenotype.
Male ; Humans ; Neurofibromatosis 2/genetics* ; Genes, Neurofibromatosis 2 ; Oculomotor Nerve Diseases/genetics* ; Computational Biology ; Genomics ; Mutation