The Ehlers-Danlos syndrome(EDS)is a rare inherent connective tissue disorder.The prev-alence of EDS in the population is estimated at one out of ten thousand to one out of a hundred thousand.The vascular EDS(vEDS)are rare among the subtypes but are the worst in prognosis.The article reports a case of vEDS admitted to the hospital.The patient was a young man complaining of a sudden onset of aphasia in right hemiparalysis and severe left abdominal pain for unknown reasons.The diagnosis was made after the genetic testing.The patient suffered from vEDS.Then,the multi-disciplinary team(MDT)made a treatment plan tailored to this young patient.The complexity in classification and delusive presentations of the EDS make the correct diagnosis very challenging.This article hopes to report this case and to share the experiences to the bet-ter understanding of this disease.

Objective:To investigate the effect of dexmedetomidine on the intraoperative and early postoperative complications of patients undergoing orthotopic liver transplantation.Methods:This is a retrospective cohort study. The clinical data of 399 patients who underwent orthotopic liver transplantation at the First Affiliated Hospital of Nanjing Medical University from January 2016 to September 2020 were retrospectively collected. There were 319 males and 80 females, aged (50.9±10.2) years (range: 10 to 73 years). These patients were divided into the control group (369 cases) and the dexmedetomidine group (30 cases) according to whether dexmedetomidine was continuously pumped intravenously during the operation until the operation ended. The 1∶2 propensity score matching was used to match the preoperative and intraoperative conditions of the two groups of patients, and the caliper width was 0.2. Outcome indicators included intraoperative postreperfusion syndrome, acute kidney injury and pulmonary complications within 7 days after surgery, length of hospital stay, time of stay in ICU, duration of assisted mechanical ventilation, rate of reintubation, 6-month and 1-year survival and recurrence-free survival rate after surgery. The independent sample t test, χ2 test, Mann-Whitney U test or Fisher exact test was used to statistically analyze the data of the two groups of patients, respectively. Survival curves of overall survival and disease-free-survival were plotted by Kaplan-Meier method, and the survival rate and recurrence-free survival rate were compared by Log-rank test. Results:A total of 78 patients were included after propensity score matching, including 26 in the dexmedetomidine group and 52 in the control group. The incidence of acute kidney injury in the dexmedetomidine group within 7 days after surgery was 0 (0/26), significantly lower than that of the control group (21.2%,11/52)(corrected χ2=4.776, P=0.029). There were no significant differences in the incidence of intraoperative postreperfusion syndrome and pulmonary complications within 7 days after surgery, length of hospital stay, ICU time, the duration of assisted mechanical ventilation, rate of reintubation, 6-month and 1-year survival, and recurrence-free survival rate after surgery between the two groups (all P>0.05). Conclusion:Continuous infusion of dexmedetomidine via intravenous pump during operation may be beneficial in reducing the incidence of acute kidney injury within 7 days after orthotopic liver transplantation.

Objective:To analyze the risk factors and prognosis for early acute kidney injury (AKI) after orthotopic liver transplantation (OLT).Methods:The retrospective study was conduc-ted. The clinicopathological data of 340 pairs of donor and recipients undergoing OLT in The First Affiliated Hospital of Nanjing Medical University from January 2016 to January 2020 were collected. There were 262 males and 78 females of donors. There were 268 males and 72 females of recipients, aged (51±11)years. Of 340 recipients, 217 cases without postoperative early AKI were divided into the non-AKI group and 123 cases with postoperative early AKI were divided into the AKI group. Measure-ment data with normal distribution were represented as Mean± SD, and comparison between groups was conducted using the independent sample t test. Measurement data with skewed distri-bution were represented as M( IQR), and comparison between groups was conducted using the Mann-Whitney U test. Count data were described as absolute numbers or percentages, and comparison between groups was conducted using the chi-square test. Comparison of ordinal data was conducted using the non-parameter test. Multivariate analysis was conducted using the binary Logistic regre-ssion model with forward method. The nomogram predictive model was constructed using the R software with its RMS package (R3.6.1). The efficacy of the predictive model was validated using the area under curve (AUC) of the receiver operating characteristic (ROC) curve, and internal validation of the predictive model was performed using the Bootstrap method. The Kaplan-Meier method was used to draw survival curves, and Log-rank test was used for survival analysis. Results:(1) Com-parison of preoperative clinical characteristics between donors and recipients of the non-AKI group and the AKI group. There was a significant difference in overweight of donors between the non-AKI group and the AKI group ( P<0.05). There were significant differences in preoperative hypertension, viral hepatitis, pathological types, international normalized ratio, fibrinogen levels, platelet (PLT), hemoglobin, and anemia of recipients between the non-AKI group and the AKI group ( P<0.05). (2) Comparison of surgical situations between recipients of the non-AKI group and the AKI group. There were significant differences in intraoperative urine output, volume of intraoperative blood loss, peak serum potassium after reperfusion, massive transfusion, plasma infusion, cryoprecipitate infusion, and aminocaproic acid use of recipients between the non-AKI group and the AKI group ( P<0.05). (3) Influencing factors for postoperative early AKI and construction and evaluation of the nomogram predictive model for postoperative early AKI. Results of multivariate analysis showed that donors of overweight, recipients of preoperative hypertension, recipients of non-viral hepatitis, recipients of preoperative severe PLT reduction, recipients of less intraoperative urine output, recipients of severe post-reperfusion hypotension, recipients of high peak serum potassium after reperfusion, recipients with intraoperative plasma infusion were independent risk factors for postoperative early AKI ( odds ratio=1.982, 3.365, 0.519, 3.615, 0.169, 2.480, 1.500, 1.001, 95% confidence interval as 1.160-3.388, 1.649-6.865, 0.293-0.917, 1.358-9.621, 0.061-0.464, 1.246-4.934, 1.003-2.243, 1.000-1.001, P<0.05). The nomogram predictive model for postoperative early AKI was constructed based on the results of multivariate analysis. Results of ROC curve showed the AUC was 0.769 (95% confidence interval as 0.717-0.820). Results of the calibration curve showed that the predictive results of nomogram predictive model fitted well with the actual situation, with a mean absolute error of 0.016. (4) Comparison of prognosis between recipients of the non-AKI group and the AKI group. There were significant differences in postopera-tive peak creatinine, peak brain natriuretic peptide, duration of intensive care unit stay, mechanical ventilation time, re-intubation of recipients between the non-AKI group and the AKI group ( Z=-4.836, -5.652, -5.861, -6.533, χ2=14.676, P<0.05). All 340 recipients were followed up. For recipients of hepatocellular carcinoma, the 6-month survival rates after surgery were 87.8% and 75.6% of the non-AKI group and the AKI group, respectively, showing a significant difference between them ( χ2=4.010, P<0.05), and the overall survival rates were 46.7% and 56.1% of the non-AKI group and the AKI group, respectively, showing no significant difference between them ( χ2=0.047, P>0.05). For recipients of benign liver disease, the 6-month survival rates after surgery were 89.8% and 78.0% of the non-AKI group and the AKI group, respectively, showing a significant difference between them ( χ2=6.401, P<0.05), and the overall survival rates were 81.4% and 68.0% of the non-AKI group and the AKI group, respectively, showing a significant difference between them ( χ2=4.452, P<0.05). Conclusions:Donors of overweight, recipients of preoperative hypertension, recipients of non-viral hepatitis, recipients of preoperative severe PLT reduction, reci-pients of less intraoperative urine output, recipients of severe post-reperfusion hypotension, recipi-ents of high peak serum potassium after reperfusion, recipients with intraoperative plasma transfu-sion were independent risk factors for postoperative early AKI. Nomogram predictive model has well clinical application value. For recipients of benign liver disease, the 6-month survival rate after surgery and overall survival rate of recipients in the non-AKI group are superior to those of the AKI group.

The diagnosis and treatment resources for rare diseases in China are highly imbalanced. The basic diagnosis and treatment capabilities are weak, the diagnosis period for patients is long, and the rates of missed diagnosis and misdiagnosis are relatively high. The establishment of a hierarchical diagnosis and treatment system is the inevitable approach to enhancing the diagnosis and treatment standards of rare diseases. Currently, the implementation of the domestic hierarchical diagnosis and treatment system for rare diseases still confronts numerous challenges, such as ambiguous referral standards and processes of primary medical institutions, and ineffective information interaction among institutions at all levels. Thus, it is essential to facilitate high-level information construction for the hierarchical diagnosis and treatment of rare diseases. This paper explores the process of constructing a multidisciplinary joint remote diagnosis and treatment platform and a health management platform through informatization, with the hope of establishing two closed loops of digital diagnosis and treatment services and health follow-up management for patients with rare diseases, as well as achieving timely diagnosis and lifelong health management for patients. It integrates and optimizes auxiliary diagnostic tools, promotes the rapid dissemination of rare disease diagnosis and treatment experiences to the grassroots, enhances the information construction level of the hierarchical diagnosis and treatment system, and endeavors to address the practical predicament of weak diagnosis and treatment capabilities of rare diseases in grassroots medical institutions. Additionally, this paper proposes an essential approach for multi-dimensional independent innovation to guide the popularization of efficient and high-quality rare disease diagnosis and treatment services. By encompassing innovating the rare disease diagnosis and treatment collaboration network and multidisciplinary diagnosis and treatment model, facilitating the application of the latest biomedical and informatics technologies to the grassroots, and constructing a national intelligent data platform for rare disease innovation, a new model for rare disease services with Chinese characteristics will be established. This will significantly enhance the medical treatment level of rare diseases in China and strive for more benefits for patients.

Objective:To investigate the effect of dexmedetomidine on the intraoperative and early postoperative complications of patients undergoing orthotopic liver transplantation.Methods:This is a retrospective cohort study. The clinical data of 399 patients who underwent orthotopic liver transplantation at the First Affiliated Hospital of Nanjing Medical University from January 2016 to September 2020 were retrospectively collected. There were 319 males and 80 females, aged (50.9±10.2) years (range: 10 to 73 years). These patients were divided into the control group (369 cases) and the dexmedetomidine group (30 cases) according to whether dexmedetomidine was continuously pumped intravenously during the operation until the operation ended. The 1∶2 propensity score matching was used to match the preoperative and intraoperative conditions of the two groups of patients, and the caliper width was 0.2. Outcome indicators included intraoperative postreperfusion syndrome, acute kidney injury and pulmonary complications within 7 days after surgery, length of hospital stay, time of stay in ICU, duration of assisted mechanical ventilation, rate of reintubation, 6-month and 1-year survival and recurrence-free survival rate after surgery. The independent sample t test, χ2 test, Mann-Whitney U test or Fisher exact test was used to statistically analyze the data of the two groups of patients, respectively. Survival curves of overall survival and disease-free-survival were plotted by Kaplan-Meier method, and the survival rate and recurrence-free survival rate were compared by Log-rank test. Results:A total of 78 patients were included after propensity score matching, including 26 in the dexmedetomidine group and 52 in the control group. The incidence of acute kidney injury in the dexmedetomidine group within 7 days after surgery was 0 (0/26), significantly lower than that of the control group (21.2%,11/52)(corrected χ2=4.776, P=0.029). There were no significant differences in the incidence of intraoperative postreperfusion syndrome and pulmonary complications within 7 days after surgery, length of hospital stay, ICU time, the duration of assisted mechanical ventilation, rate of reintubation, 6-month and 1-year survival, and recurrence-free survival rate after surgery between the two groups (all P>0.05). Conclusion:Continuous infusion of dexmedetomidine via intravenous pump during operation may be beneficial in reducing the incidence of acute kidney injury within 7 days after orthotopic liver transplantation.

The aim of this study is to establish a simple and accurate method for vaccine immune e-valuation of porcine pseudorabies virus.In this research,PRV-gD recombinant protein was ex-pressed from mammalian cell HEK-293F as coating antigen,and then the reaction conditions of gD-iELISA were optimized according to checkerboard titration method.The gD-iELISA was used to detect the antibody levels of 211 clinical pig serum samples and the consistency with the neu-tralizing antibody levels wasanalyzed.The results showed that the antigen coating concentration was 0.90 mg/L;the serum to be detected was diluted 1∶100 and incubated at 37 ℃ for 30 min;goat anti-pig IgG-HRP antibody was diluted 1∶55 000 and incubated at 37 ℃ for 30 min;TMB sub-strate was developed at 37 ℃ for 20 min.The method could detect 1∶6 400 diluted PRV positive serum.The results of CSFV,PRRSV,PCV-2,PEDV and FMDV positive sera were all negative by gD-iELISA,and there was no cross-reaction between the method and the above positive sera.The coincidence rate of gD-iELISA and commercial kits was 95.26％,and the intra-and inter-batch co-efficients of variation were both less than 10％.Correlation analysis showed that the correlation coefficient(r)between gD antibody level and neutralizing antibody level was significantly greater than that of gB antibody level,and the gD antibody level had a good linear relationship with the neutralizing antibody level.The results indicated that gD-iELISA was more suitable for vaccine im-mune evaluation of PRV than gB-iELISA.Therefore,the method will have a good prospect of ap-plication in the immunization control of the PRV.

Objectives:To investigate the clinical feasibility of three-dimensional(3D)high-resolution late gadolinium enhancement(LGE)MRI in accessing left atrial myocardial fibrosis in patients with atrial fibrillation(AF). Methods:A total of 34 AF patients referred for hybrid surgical ablation were retrospectively enrolled in this study.3D-LGE-MRI images were acquired by Siemens 3.0 T machine and analyzed by ADAS post-processing software by two experienced radiologists to obtain parameters such as the area and the area percentage of LGE.Regional analysis was performed by one of the two radiologists at ten left atrial segments.The Kappa test was used to assess the agreement for scoring image quality,and the interclass correlation coefficient(ICC)was used to evaluate the interobserver agreement of LGE parameters.The parameters of left atrial morphology,area(and area percentage)of LGE,and location of LGE were compared between patients with persistent AF and paroxysmal AF. Results:Images of all 34 patients were considered to have diagnostic value.The scores of the overall image quality and the clarity of the left atrial wall evaluated by two radiologists were(2.88±0.64)points and(3.26±0.75)points(radiologist 1),(2.97±0.58)points and(3.24±0.70)points(radiologist 2),respectively.The corresponding Kappa values were 0.724 and 0.859.Both the area and the area percentage of LGE showed good consistency among observers,and the ICCs were 0.969 and 0.950,respectively.The difference in the area of LGE and the area percentage of LGE between patients with paroxysmal and persistent atrial fibrillation was similar(both P＞0.05).Compared with patients with paroxysmal AF,patients with persistent AF had a higher Utah stage and more severe myocardial fibrosis in the right inferior pulmonary vein antrum and the left atrial septum(all P＜0.05). Conclusions:3D high-resolution LGE-MRI provides a non-invasive way to visualize and quantify left atrial myocardial fibrosis.The extent of left atrial fibrosis in patients with persistent AF is more severe than that in patients with paroxysmal AF,with a preferential distribution in the right inferior pulmonary vein antrum and the left atrial septum.

Objective:To assess the feasibility, efficacy, and therapeutic advantages of radioactive 125I seed implantation for the treatment of abdominal metastases. Methods:The clinical data of eight patients with abdominal metastases treated with radioactive 125I seed implantation were retrospectively analyzed. Results:Of the three pelvic metastasis cases, two patients with grade II pain had postoperative pain relief, and one patient with grade III pain had a gradual decrease in pain to grade I one week after surgery. One patient with lower extremity edema had complete disappearance of edema one month after surgery. The lumbar back pain disappeared after ureteral stent implantation in one patient with ureteral compression. In three patients with perineal drop sensation, the discomfort disappeared at the 2-month postoperative follow-up. In two patients with abdominal wall metastases, the pain disappeared one day after surgery, and the metastatic lesions gradually decreased in size. Postoperative re-evaluation of patients with multiple peripancreatic and retroperitoneal lymph node metastases showed the disappearance of lymph node enlargement.Conclusion:Radioactive 125I seed implantation can effectively improve the life quality of patients with abdominal metastases.

Objective To explore and construct an indicator system for performance appraisal in operating room nurses of cancer hospitals,in order to provide references for performance management.Methods From May to December 2022,the four-dimensional model of individual performance was used as the theoretical framework,and the research was carried out by literature research method,focus group interview method,Delphi expert consultation method and analytic hierarchy process,to establish the content of the performance appraisal index system of operating room nurses in cancer hospitals and the weight of each index.Results After 2 rounds of expert correspondence,29 questionnaires were sent out and 27 returned in the first round,with an expert positive coefficient of 93.10％;27 questionnaires were sent out and 27 returned in the second round,with an expert positive coefficient of 100％;expert authority coefficient of the first round was 0.941 and the second round was 0.937.In the first round,the variation coefficient of each index was 0-0.249,and the coordination coefficient was 0.201-0.352;in the second round,the variation coefficient of each index was 0-0.204,and the coordination coefficient was 0.275-0.407.Finally,a performance appraisal index system of operating room nurses in cancer hospitals was formed,which consisted of 4 first-level indicators,including task performance,interpersonal performance,adaptive performance and effort performance,11 second-level indicators and 54 third-level indicators.Conclusion The indicator system for performance appraisal in operating room nurses of cancer hospitals constructed in this study is reliable and scientific,specialized and applicable,and it can provide references for performance management.

Objective:To investigate the effectiveness, safety, and advantages of modified radical neck dissection by gasless unilateral axillary approach (GUA-MRND) in the surgical management of selected patients with papillary thyroid cancer.Methods:We retrospectively analyzed patients with papillary thyroid cancer who underwent GUA-MRND (endoscopic group, n=16) versus unilateral open modified radical neck dissection (MRND) (open group, n=32) during the period from Jan. 2019 to Jun. 2021, including the differences in surgical efficiency, complication rate, and incisional satisfaction.Results:Compared MRND with GUA-MRND, the patients were younger ( P<0.05) , operative time and postoperative drainage anterior ( P<0.01) were slightly inferior in the latter, but it had obvious advantages in cervical swallowing discomfort and incision satisfaction evaluation ( P<0.05) . There was no significant difference in the incidence of temporary recurrent laryngeal nerve injury, intraoperative and postoperative bleeding, hematoma, infection, lymphatic or chylous leakage and supraclavicular numbness after surgery ( P>0.05) . The number of dissected lymph nodes in area II in the GUA-MRND was lower ( P<0.05) , but it was significantly higher ( P<0.01) in area III. And the average regional cleaning efficiency in the GUA-MRND was level Ⅲ (35.5%) , level Ⅵ (28.59%) , level Ⅳ (23.21%) , level Ⅱ (7.18%) and level Ⅴ (7.12%) , suggested that GUA-MRND had higher efficacy for level III, level Ⅵ and Level IV. Conclusion:GUA-MRND is safe, effective, and has high cosmetic satisfaction in the treatment of selected patients with lateral cervical lymph node metastases from papillary thyroid cancer.