As a neglected tropical disease, schistosomiasis remains a major public health challenge in underdeveloped areas, notably Africa. Currently, the national schistosomiasis control programmes in Africa mainly depend on foreign aids; however, conventional international aid models have multiple limitations. To enhance the effectiveness and sustainability of global schistosomiasis control programmes, this article proposes a trinity collaboration model based on international rules, China’s experiences and local needs, which is explained with China aid project of schistosomiasis control in Zanzibar as an example. Based on the successful experiences from the national schistosomiasis control programme in China, this model emphasizes the compliance with World Health Organization guidelines and fully considers local actual needs to promote the effectiveness and sustainability of the schistosomiasis control programme through integrating international resources and promoting China’s experience to meet local needs. The successful practice of the China aid project of schistosomiasis control in Zanzibar provides strong evidence that the model is of great theoretical significance and practical value to improve the efficiency of multilateral collaboration and promote global health governance.

Our study aims at systematically summarizing and evaluating the applications of digital intelligence technologies in the field of rare disease medical care insurance now and in the future and at constructing a conceptual framework for the digital powered mechanism for the medical care insurance for rare diseases. By using Chinese keywords of " rare disease" " medical insurance"" artificial intelligence"" prediction model"" machine learning"" big data"" algorithm" and their English equivalents, we searched the databases of PubMed, Embase, Web of Science, CNKI, Wanfang, and VIP, collected relevant literature, and decided the criteria of inclusion and exclusion. The finding of our study shows that medical care insurance mechanism of rare disease in China faces significant challenges in drug accessbility and the funding sustainability. Meanwhile, our study shows that the digital intelligence technologies have broad potential in applications-in financing, accessbility, payment, and supervision. Specifically, dynamic simulation models and big data analysis can make precise prediction of the demand for funding of medical care insurance. The machine learning algorithms improve the dynamic evaluation of drug safety and cost-effectiveness. The personalized payment models enhance the efficiency in identifying the cohort with high expenditure so as to alleviate fund expenditure pressures. The intelligent monitoring technologies can accurately detect the abnormal behaviors in funds of medical care insurance. These technologies provide systematic and scientific solutions for improving the medical care mechanism for rare diseases. Even though further investigation is needed, the digital intelligence technologies have shown remarkable potential in enhancing the flexibility, efficiency, and sustainability of the medical care insurance system and a promising future in meeting the needs of patients with rare diseases.

Objective:To explore whether transjugular intrahepatic portosystemic shunt (TIPS) can improve the prognosis of esophagogastric variceal bleeding (EGVB) combined with sarcopenia in cirrhotic patients.Methods:A retrospective cohort study was performed. A total of 464 cases with cirrhotic EGVB who received standard or TIPS treatment between January 2017 and December 2019 were selected. Regular follow-up was performed for the long-term after treatment. The primary outcome was transplantation-free survival. The secondary endpoints were rebleeding and overt hepatic encephalopathy (OHE). The obtained data were statistically analyzed. The t-test and Wilcoxon rank-sum test were used to compare continuous variables between groups. The χ2 test, or Fisher's exact probability test, was used to compare categorical variables between groups. Results:The age of the included patients was 55.27±13.86 years, and 286 cases were male. There were 203 cases of combined sarcopenia and 261 cases of non-combined sarcopenia. The median follow-up period was 43 months. The two groups had no statistically significant difference in follow-up time. There was no statistically significant difference in transplant-free survival between the TIPS group and the standard treatment group in the overall cohort ( HR=1.31, 95% CI: 0.97-1.78, P=0.08). The TIPS patient group with cirrhosis combined with sarcopenia had longer transplant-free survival (median survival: 47.76 vs. 52.45, χ2=4.09; HR=1.55, 95 CI: 1.01~2.38, P=0.04). There was no statistically significant difference in transplant-free survival between the two kinds of treatments for patients without sarcopenia ( HR=1.22, 95% CI: 0.78~1.88, P=0.39). Rebleeding time was prolonged in TIPS patients with or without sarcopenia combination (patients without combined sarcopenia: median rebleeding time: 39.48 vs. 53.61, χ2=18.68; R=2.47, 95 CI: 1.67~3.65, P<0.01; patients with sarcopenia: median rebleeding time: 39.91 vs. 50.68, χ2=12.36; HR=2.20, 95 CI: 1.42~3.40, P<0.01). TIPS patients had an increased 1-year OHE incidence rate compared to the standard treatment group (sarcopenia patients: 6.93% vs. 16.67%, χ2=3.87, P=0.049; patients without sarcopenia combination: 2.19% vs. 9.68%, χ2=8.85, P=0.01). There was no statistically significant difference in the long-term OHE incidence rate between the two kinds of treatment groups ( P>0.05). Conclusion:TIPS can significantly prolong transplant-free survival compared to standard treatment as a secondary prevention of EGVB concomitant with sarcopenia in patients with cirrhosis. However, its advantage is not prominent for patients with cirrhosis in EGVB without sarcopenia.

Objective:To summarize the clinical manifestations and diagnostic methods of adult neuronal intranuclear inclusion disease (NIID), improve understanding of the disease, and avoid misdiagnosis.Methods:Clinical data of 8 adult NIID patients in the Hunan region were collected, and their clinical manifestations, cranial imaging, genetic testing, skin biopsy, and other characteristics were analyzed.Results:Among the 8 patients, 4 were males and 4 were females; The initial symptoms of 2 patients were dizziness, 2 were mental abnormalities, 2 were stroke like attacks, 1 was urinary incontinence, and 1 was limb tremor; Six patients experienced slow progression of the disease, while two patients experienced sudden progression after several years of slow progression; The GGC repeat amplification mutation in the 5′untranslated region of the NOTCH2NLC gene, as well as the lace like sign in the brain cortex medullary junction on diffusion-weighted imaging (DWI) and the presence of eosinophilic transparent inclusion bodies in the nucleus on skin biopsy, were helpful in diagnosing NIID.Conclusions:The clinical manifestations of NIID are highly heterogeneous, and some patients have rare initial clinical symptoms, which are prone to misdiagnosis and missed diagnosis. It is necessary to combine imaging, genetic testing, and skin biopsy to confirm the diagnosis; Some patients may experience sudden progression and poor prognosis after years of slow progression.

Anti γ-aminobutyric acid B receptor encephalitis is a type of autoimmune encephalitis characterized by the production of self specific antibodies in cerebrospinal fluid and/or serum, with seizures, memory loss, and consciousness disorders as the main clinical manifestations. This type of encephalitis caused by autoantibodies has the same pathological characteristics as other peripheral encephalitis. There are many different etiologies and pathophysiological processes that lead to the occurrence of limbic encephalitis, and it is necessary to understand their heterogeneity in order to find effective treatment methods. This article will systematically review the epidemiology, pathogenesis, clinical characteristics, diagnosis, treatment, and prognosis of anti γ-aminobutyric acid B receptor encephalitis, aiming to enhance clinical doctors' understanding of this disease and provide reference for clinical decision-making.

Objective To explore the causal relationship between rheumatoid arthritis(RA)and iron deficiency a-nemia(IDA)in European population by two-sample Mendelian randomization analysis.Methods The single nu-cleotide polymorphisms(SNPs)of RA and IDA were analyzed using public genome-wide association studies(GWAS).The inverse variance weighting method(IVW)was used as the main analysis method to evaluate the causal effect of RA on IDA.MR-Egger method,weighted median method(WM),weighted model method and simple model method were used as regression supplements to evaluate the robustness of sensitivity analysis results.The het-erogeneity function was used to calculate the P-value to test the heterogeneity,and the intercept term intercept was used to test the level pleiotropy.Results In the FINNGEN database at the genome-wide level,strong-related SNPs that removed linkage disequilibrium and met the P＜5.0 × 10-8 by Mendelian randomization analysis were select-ed.After integrating exposure and outcome data,31 SNPs were obtained as the final effective instrumental variables.IVW showed that RA was a risk factor for IDA(the risk of IDA in RA patients was 1.064 times higher than that in non-RA patients,OR=1.064,95％CI:1.028-1.103).The weighted median method and MR-Egger method re-sults supported the positive correlation between RA and IDA.The intercept value was close to 0,indicating that there was no horizontal pleiotropy between exposure and outcome.The heterogeneity function's P＜0.05 indicated that there was heterogeneity between exposure and outcome,but the random effect model test showed P＜0.05,indi-cating that even if there was heterogeneity in causality,the overall trend was stable.Conclusion RA is a risk factor for IDA,and there is a positive correlation between RA and IDA.

Objective To investigate the risk factors for unplanned readmission within 30 days after discharge in cirrhotic patients with esophagogastric variceal bleeding undergoing transjugular intrahepatic portosystemic shunt(TIPS),and to construct a nomogram predictive model.Methods A total of 241 cirrhotic patients who underwent TIPS due to esophagogastric variceal bleeding in Affiliated Drum Tower Hospital of Nanjing University Medical School from January 2020 to June 2023 were enrolled as subjects,and unplanned readmission within 30 days was analyzed.According to the presence or absence of unplanned readmission,they were divided into readmission group with 36 patients and non-readmission group with 198 patients,and related clinical data were collected from all patients.The independent-samples t test was used for comparison of normally distributed continuous data between two groups,and the Mann-Whitney U test was used for comparison of non-normally distributed continuous data between two groups;the chi-square test was used for comparison of categorical data between two groups.A logistic regression analysis was used to identify independent risk factors for unplanned readmission.A nomogram prediction model was constructed,and the receiver operating characteristic(ROC)curve was plotted to assess its discriminatory ability for unplanned readmission;the calibration curve was plotted to evaluate the consistency of the nomogram model in predicting unplanned readmission;the ResourceSelection package of R language was used for the Hosmer-Lemeshow goodness-of-fit test to evaluate the degree of fitting of the mode;the decision curve analysis was used to investigate the practicality of the model.Results Age(odds ratio[OR]=2.664,95%confidence interval[CI]:1.139-6.233,P<0.05),CTP score(OR=1.655,95%CI:1.098-2.495,P<0.05),and blood ammonia(OR=1.032,95%CI:1.016-1.048,P<0.05)were independent risk factors for unplanned readmission within 30 days after discharge in the patients undergoing TIPS.The multivariate analysis showed that for the nomogram predictive model constructed in this study,repeated sampling for 1 000 times using the Bootstrap method was performed for internal validation,and the area under the ROC curve was 0.773,which was significantly higher than that of age(0.582),CTP score(0.675),and blood ammonia(0.641).The calibration curve showed good consistency between the probability of unplanned readmission predicted by the nomogram model and the actual probability,and the Hosmer-Lemeshow goodness-of-fit test showed good degree of fitting(c2=5.647 3,P=0.686 7).Conclusion Age,CTP score,and blood ammonia are independent risk factors for unplanned readmission within 30 days after TIPS,and the nomogram prediction model constructed based on these factors can help to predict the risk of unplanned readmission in TIPS patients and provide an accurate decision-making basis for early prevention.

Objective To investigate the causal relationship between rheumatoid arthritis (RA) and pulmonary hypertension (PH) by the Mendelian randomization design method.Methods The data on single nucleotide polymorphisms (SNPs) of exposure and outcome were obtained by using publicly available ge-nome-wide association studies and the summary analysis was conducted;the inverse variance-weighted (IVW) method as the primary analysis method was used to assess the causal effect of exposure factors (RA) on the outcomes (PH);the MR-Egger regression method,weighted median method (WM),weighted model and sim-ple model were used as supplementary regression explanations to conduct the sensitivity analysis for evalua-ting the robustness of results;the "heterogeneity" function was used to calculate the "P value" for testing the heterogeneity,and the "horizontal pleiotropy" function was used to calculate the "P value" to test the level pleiotropy.Results In the "FINNGEN data" included in the GWAS database,the SNPs had the strong corre-lation after removing the linkage imbalance by Mendelian random analysis and satisfying "P<5×10-8" were selected,the effective instrumental variables were obtained by integrating the exposure and outcome.The IVW results showed that RA was a risk factor for PH (OR=1.295,95％CI:1.053-1.593,P=0.014)."heteroge-neity" function test showed that the results had no heterogeneity (P=0.221);" horizontal pleiotropy" func-tion test showed that the results had no horizontal pleiotropy (P=0.877),and the total results were steady and reliable.Conclusion RA is a risk factor for PH,and RA is positively associated with PH.

Objective To investigate the accuracy of liver stiffness (LS) as a noninvasive index in predicting hepatic venous pressure gradient (HVPG) in patients with decompensated liver cirrhosis and the value of LS in the diagnosis of decompensated liver cirrhosis. Methods A retrospective analysis was performed for the clinical data of 88 patients with decompensated cirrhosis due to viral hepatitis or decompensated alcoholic cirrhosis who received both HVPG measurement and LS measurement by acoustic radiation force impulse (ARFI) in Department of Gastroenterology, Nanjing Drum Tower Hospital, from April 2013 to June 2021, and according to HVPG, the patients were divided into serious portal hypertension (SPH) (HVPG≥20 mmHg) group with 24 patients and non-SPH (HVPG < 20 mmHg) group with 64 patients. The two groups were compared in terms of LS, spleen stiffness, portal vein velocity, and related biochemical parameters. The t -test or the Mann-Whitney U rank sum test was used for comparison of continuous data between two groups, and the chi-square test was used for comparison of categorical data between two groups. A Pearson correlation analysis was used to investigate the correlation of different noninvasive indices with HVPG, and a Logistic regression analysis was used to investigate the association of different noninvasive indices with the risk of SPH. Receiver operating characteristic (ROC) curves were plotted for different noninvasive indices in predicting HVPG≥20 mmHg, and the area under the ROC curve (AUC), sensitivity, specificity, maximum Youden index, and corresponding cut-off value were calculated to investigate the value of each index in predicting SPH. Results Among the 88 patients, 76 had decompensated cirrhosis due to viral hepatitis and 12 had decompensated alcoholic cirrhosis. There were no significant differences between the SPH group and the non-SPH group in age, sex, white blood cell count, hemoglobin, platelet count, prothrombin time, alanine aminotransferase, aspartate aminotransferase, albumin, serum sodium, creatinine, Child-Pugh class, and spleen stiffness, while there was a significant difference in LS between the two groups ( t =-3.970, P < 0.01). The correlation analysis showed that HVPG was positively correlated with LS ( r =0.458, P < 0.001). The Logistic regression analysis showed that LS was a risk factor for SPH (odds ratio=3.941, 95% confidence interval: 1.245-12.476, P =0.020). The ROC curve analysis showed that LS had an AUC of 0.751 in predicting the onset of SPH, with a sensitivity of 54.17% and a specificity of 90.63% at the optimal cut-off value of 2.295 m/s. Conclusion In patients with decompensated cirrhosis, LS measured by ARFI is correlated with HVPG and has a certain value in the non-invasive diagnosis of decompensated cirrhosis with HVPG≥20 mmHg.

Objective:To explore the role of serum pyrrole-protein-adduct (PPA) in evaluating the severity and predicting the anticoagulant efficacy in patients with pyrrolidine alkaloid-related hepatic sinusoidal obstruction syndrome (PA-HSOS).Methods:From April 2018 to December 2019, the data of 48 patients with PA-HSOS admitted and treated at Drum Tower Hospital, Affiliated Medical College of Nangjing University were collected, which included PPA level, portal vein velocity (PVV), ascites grading, PA-HSOS severity grading (according to the new severity grading criteria for suspected hepatic sinusoidal obstruction syndrome in adults by the European Society of Blood and Bone Marrow Transplantation and adjusted) and the outcome of anticoagulation. Patients with acute onset (onset of symptoms within 1 month after consuming pyrrolizidine alkaloid-containing plants) were taken as research subjects. The combination of PPA with PVV or with ascites classification of PA-HSOS severity assessment model was fitted by logistic regression, and the logit values of 2 combination models were calculated, the formula was logit 1=0.034×PPA(nmol/L)+ 0.055×PVV(cm/s)-3.287, logit 2=0.039×PPA(nmol/L)-2.712×ascites grade 2 (Yes=1, No=0)-0.388×ascites grade 3 (Yes=1, No=0)-0.899. The patients received initial anticoagulation therapy at Drum Tower Hospital, Affiliated Medical College of Nanjing University were selected as research subjects. The anticoagulant efficacy prediction model of combination of PPA with serum creatinine (SCR) and with hepatic venous pressure gradient (HVPG) was fitted by logistic regression, and the logit value was calculated, the formula was logit 3=0.013×PPA(nmol/L)+ 0.064×SCR (mol/L)+ 0.542×HVPG (mmHg, 1 mmHg=0.133 kPa)-16.005. The predictive value of PPA in evaluating the severity of PA-HSOS and anticoagulant efficacy was evaluated. Receiver operating characteristic curve analysis was performed for statistical analysis. Results:The serum PPA level of 48 patients was 10.81 nmol/L (3.91 nmol/L, 32.04 nmol/L). Among them, 33 cases (68.8%) were mild PA-HSOS, 3 cases (6.2%) were moderate PA-HSOS, no severe PA-HSOS case and 12 cases (25.0%) were very severe PA-HSOS. Among 23 patients received initial anticoagulant therapy at Drum Tower Hospital, Affiliated Medical College of Nanjing University and with complete data, 8 patients responded and survived, and 15 patients did not respond (5 patients died, 1 patient relieved after continue anticoagulant therapy, and 9 patients survived after switching to anticoagulant therapy and transjugular intrahepatic portosystemic shunt (TIPS) treatment). One patient without initial anticoagulant therapy, survived after TIPS treatment because of the progress of the disease. Area under the curve (AUC) of PPA to assess the severity of acute onset PA-HSOS was 0.75, 95% confidence interval ( CI) was 0.52 to 0.98 ( P=0.047). When PPA≥45.519 nmol/L, the specificity and sensitivity in evaluating severe and very severe PA-HSOS was 100.0% and 57.1%, respectively. AUC of combination of PPA and PVV to assess the severity of PA-HSOS was 0.77, 95% CI was 0.55 to 1.00 ( P=0.032). When the logit of combination model≥0.180, the specificity and sensitivity in evaluating severe and very severe PA-HSOS was 71.4% and 81.8%, respectively. AUC of combination of PPA and ascites grade (grade 1, 2 or 3) to assess the severity of PA-HSOS was 0.85, 95% CI was 0.63 to 1.00 ( P=0.005). When the logit of combination model≥0.347, the specificity and sensitivity in evaluating severe and very severe PA-HSOS was 85.7% and 92.0%, respectively. AUC of combination of PPA, SCR and HVPG to predict anticoagulation efficacy was 0.85, 95% CI was 0.69 to 1.00 ( P=0.009). When the logit≥0.393, the specificity and sensitivity in predicting anticoagulation efficacy was 62.5% and 91.7%, respectively. Conclusions:PPA can be used to assess the severity of acute onset PA-HSOS patients, and combined with ascites grading can significantly improve its efficiency. PPA combined with SCR and HVPG can better predict anticoagulant efficacy.