This study reports on three patients with Shwachman-Diamond syndrome (SDS) who underwent allogeneic hematopoietic stem cell transplantation (allo-HSCT) at the First Affiliated Hospital of Zhejiang University School of Medicine. Based on relevant literature, the clinical manifestations and genetic mutation characteristics of SDS were summarized, and the efficacy and timing of allo HSCT for such patients were explored. Three SDS patients were all male, with transplant ages of 32, 33, and 32 years old, respectively. All three patients were diagnosed in childhood. Case 1 presented with anemia as the initial clinical manifestation, which gradually progressed to a decrease in whole blood cells; Case 2 and 3 both present with a decrease in whole blood cells as the initial clinical manifestation. Case 1 and 3 have intellectual disabilities, while case 3 presents with pancreatic steatosis and chronic pancreatitis. All three patients have short stature. Three patients all detected heterozygous mutations in the SBDS: c.258+2T>C splice site. The family members of the three patients have no clinical manifestations of SDS. All three patients were treated with a reduced dose pre-treatment regimen (Fludarabine+Busulfan+Me-CCNU+Rabbit Anti-human Thymocyte Globulin). Case 1 and case 2 underwent haploid hematopoietic stem cell transplantation, while case 3 underwent unrelated donor hematopoietic stem cell transplantation. Case 1 was diagnosed with myelodysplastic syndrome transforming into acute myeloid leukemia before transplantation, but experienced early recurrence and death after transplantation; Case 2 is secondary implantation failure, dependent on platelet transfusion; Case 3 was removed from medication maintenance treatment after transplantation, and blood routine monitoring was normal.

ObjectiveTo understand the survival time and possible influencing factors of pneumoconiosis patients in Ninghai County， Zhejiang Province who were followed up from 2019 to 2023， and to provide references for prolonging the survival time of pneumoconiosis patients. MethodsInformation about pneumoconiosis patients was collected from the sub-module Occupational Disease Follow-Up Management and Follow-Up Excel database of the Occupational Disease and Health Hazards Monitoring Information System. Survival analysis was conducted through applying Kaplan-Meier survival curves and Cox regression models. ResultsOf the 366 pneumoconiosis patients， 99.73% were male， 71.04% had stage Ⅰ pneumoconiosis， and the disease type was mainly silicosis. The survival rates at 5， 10， and 20 years after diagnosis of pneumoconiosis were 97%， 88%， and 62%， respectively， with a cumulative survival rate of 42%. The median survival time of patients with pneumoconiosis was 32.11 years. Log-rank test found statistically significant differences in the survival curves among pneumoconiosis patients with different pneumoconiosis stages， age at first diagnosis， and pneumoconiosis complications. Cox regression analysis revealed that the risk of reduced survival time in patients with pneumoconiosis stage Ⅲ was 2.184 times higher than that of patients with stage Ⅰ （P=0.007）. In comparison with the age of first diagnosis at <50 years old group， the risk of reduced survival time was 3.457 and 7.097 times higher， respectively， in the groups of patients who received the diagnosis at 50‒ and ≥60 years （both P<0.001）. The risk of reduced survival time in pneumoconiosis patients with complications was 2.030 times higher than that of patients without complications （P=0.003）. ConclusionSurvival time of pneumoconiosis patients in Ninghai County is affected by the age at first diagnosis， pneumoconiosis stage and pneumoconiosis complications. The survival time can be significantly prolonged by timely detection and diagnosis， as well as active prevention and treatment of pneumoconiosis complications.

Objectives:To compare the diagnostic efficacy of non-ST-segment elevation myocardial infarction (NSTEMI) and the predictive value for major adverse cardiovascular events (MACE) of the 0/3-hour algorithm for high-sensitivity cardiac troponin (hs-cTn) recommended by the 2015 European Society of Cardiology (ESC) guidelines for the management of non-ST-segment elevation acute coronary syndrome (NSTE-ACS) and the 2021 "Chinese Expert Consensus on Laboratory Testing and Clinical Application of Cardiac Troponin" in suspected acute coronary syndrome (ACS) patients in the Chinese population. Methods:This is a multicenter prospective observational study,including 1527 patients with suspected ACS from three clinical centers from January 2017 to September 2020.Plasma hs-cTnI levels were measured using the ARCHITECT assay at the time of presentation and 3 hours later in patients with suspected ACS (test determination).Clinical judgment (independent clinical judgment by cardiac experts,independent of the test results) was used as the gold standard to compare the sensitivity,specificity,and consistency of the two diagnostic algorithms,and to analyze their predictive value for MACE at 30 days and 180 days.MACE in this study was defined as a composite event of cardiovascular death,myocardial infarction,and unplanned coronary revascularization. Results:According to clinical judgment,there were 400 patients with NSTEMI and 1127 patients without NSTEMI.The 0/3-hour algorithm recommended by the 2021 Chinese Expert Consensus showed higher sensitivity in diagnosing NSTEMI than the 2015 ESC guidelines (91.50％[95％ CI:88.32％-94.04％]vs.87.75％[95％ CI:84.13％-90.80％]),but slightly lower specificity (93.88％[95％ CI:92.32％-95.21％]vs.95.56％[95％ CI:94.19％-96.69％]),with both differences being statistically significant (both P<0.001).In the follow-up at 30 days and 180 days,the incidence of MACE in patients diagnosed with NSTEMI by both algorithms was higher than in those without NSTEMI (P<0.001).The incidence of MACE at 30 days and 180 days for the group excluded from the diagnosis of NSTEMI by 2015 ESC guidelines was 0.19％ and 1.120％,respectively,and for the NSTEMI group was 2.89％ and 3.68％,respectively;for the group excluded from NSTEMI by the 2021 Chinese Expert Consensus,the incidence was 0.096％ and 0.770％,respectively,and for the NSTEMI group was 2.91％ and 4.36％,respectively.Cox analysis showed that the HR ratio for MACE at 180 days in the NSTEMI group diagnosed by both algorithms was 3.418 and 5.892,respectively,significantly higher than the group excluded from NSTEMI. Conclusions:The 0/3-hour algorithm recommended by the 2021 Chinese Expert Consensus has superior diagnostic sensitivity compared to the 2015 ESC NSTE-ACS guidelines,at the cost of slightly lower specificity.Both algorithms can effectively predict MACE within 180 days,but based on the data from this study,the algorithm recommended by the 2021 Chinese Expert Consensus is more sensitive in predicting the risk of MACE,and patients excluded from the diagnosis of NSTEMI by this method have a lower incidence of MACE,suggesting that its application in clinical practice may be more helpful in terms of long-term safe management of patients.

Objective:To construct a Bayesian network risk prediction model for delirium during recovery from general anesthesia. To explore the network relationship between awakening delirium of general anesthesia and its related factors, and to reflect the influence intensity of each factor on awakening delirium of general anesthesia through network reasoning.Methods:This is a cross-sectional study. From February to May 2022, the Chinese version of the four rapid delirium diagnosis protocols for general anesthesia patients admitted to the department of Anesthesia, the First Hospital of Shanxi Medical University were adopted as research subjects through convenience sampling method to carry out the delirium screening program during awakening, and general information and blood sample laboratory test results of the subjects were collected. The single factor analysis was used to screen the correlative factors of awakening delirium and a Bayesian network model based on the maximum minimum climb method (MMHC) was constructed.Results:A total of 480 patients were included in the study, and the delirium rate during the recovery period of general anesthesia was 12.9%(62/480). The Bayesian network of awakening delirium consisted of 11 nodes and 18 directed edges. The Bayesian network showed that age, sodium, cerebral infarction and hypoproteinemia were the direct factors related to awakening delirium, while ASA grade, hematocele and hemoglobin were the indirect factors related to awakening delirium. The area under its ROC curve was 0.80(0.78-0.83).Conclusions:Bayesian networks can well reveal the complex network connections between awakening delirium and its related factors, and then prevent and control awakening delirium accordingly.

Background Pneumoconiosis is one of the most common and widespread prescribed occupational diseases in China, and there is no cure for it at present. The Healthy China 2030 underlines that the prevention and treatment of pneumoconiosis are the critical element of promoting occupational health. Objective To understand the basic characteristics, social security, and death causes of pneumoconiosis cases in Ninghai County, and to provide a basis for scientific prevention and treatment of pneumoconiosis. Methods Information on pneumoconiosis cases in Ninghai County from 1974 to 2019 was obtained by consulting occupational disease and occupational health information monitoring systems, occupational disease diagnosis institutions, and employers, which may retain original data on occupational pneumoconiosis diagnosis, as well as specialized epidemiological survey projects on pneumoconiosis. Telephone or face-to-face follow-up visits were conducted based on relevant information to complete or verify relevant information such as length of service with dust exposure, type, stage, and diagnosis date of pneumoconiosis. ANOVA, chi-square test, and rank-sum test were used to analyze age of diagnosis and age of death, entitlement to social security and mortality rate of cases/length of service. Results As of the end of 2019, a total of 420 cases of pneumoconiosis were diagnosed among workers exposed to dust in Ninghai County, with 409 cases (97.38%) followed up and 11 cases (2.62%) lost to follow-up. Since the first case of pneumoconiosis was reported in 1974, 39 cases, 278 cases, and 101 cases of pneumoconiosis were reported before 2000, from 2000 to 2009, and from 2010 to 2019, respectively. Two other cases of pneumoconiosis were diagnosed with an unknown year, one as a dead case and one as a lost case. There were 294 surviving pneumoconiosis cases, who mainly lived in Sangzhou Town. The mean age of diagnosis was (58.68±15.37) years old, and the median length of service with exposure to dust was 8.0 (4.0, 15.7) years. The age of diagnosing stage III pneumoconiosis was less than that of stage I and stage II, and the difference between the age of diagnosing stage III and stage II pneumoconiosis was significant (P <0.05). There were 231 cases (78.57%) aged ≥ 60 years, while there were 74 cases aged ≥ 80 years (25.17%). There were 160 cases (54.42%) whose length of service was < 10 years. There were 12.59% and 7.82% of the total cases compensated by work injury insurance and civil compensation by employers, respectively. From the perspective of diagnosis period, the proportion of patients who collected work-related injury insurance and civil compensation from employers in the 2010—2019 year group was higher than that in the <2000 year group or the 2000—2009 year group (P<0.01). There were 115 fatal pneumoconiosis cases, and the fatality rates of stage I, II , and III pneumoconiosis were 23.86%, 18.18%, and 50.75%, respectively. The fatality rate of stage III pneumoconiosis was higher than that of stage I or II (P <0.05). The age of diagnosis and age of death of stage III pneumoconiosis cases were lower than those of stage I and II (P <0.05). The cause of death was definite in 65 patients, including 25 cases (38.46%) of respiratory diseases (except lung cancer) and 28 cases (43.07%) of tumors, and lung cancer accounted for 1/2 of the pneumoconiosis cases that died due to tumors. Conclusion The surviving pneumoconiosis cases in Ninghai are mainly older than 60 years old and largely live in Sangzhou Town. Constructing local rehabilitation stations should be taken as an opportunity to actively prevent and treat pneumoconiosis complications, further improving the quality of life of pneumoconiosis patients.

Objective:To investigate the role of heparin-binding protein (HBP) as a predictor of early bacterial infections in patients with traumatic intracerebral hemorrhage.Methods:Patients with traumatic intracerebral hemorrhage admitted to the Emergency Department of the First Hospital of Shanxi Medical University from September 2021 to June 2022 were collected prospectively. Patients with bacterial infection diagnosed by pathogenic examination were classified as the infected group, and those with negative pathogenic examination were classified as the non-infected group. Peripheral blood HBP counts were measured within 48 h of admission, and general information and relevant laboratory tests were collected. The differences of the indicators between the two groups were compared, the receiver operating characteristic (ROC) curve was drawn, the predictive value of the indicators for patients with co-infection was assessed, and the valuable predictors were screened out using multivariate logistic regression analysis.Results:Eighty-five patients [44 males and 41 females, aged (55.09±1.18) years] , were included in the study. Among the patients included in the study, 39 patients had bacterial infection and 46 were non-infected. Patients in the infected group were older , and had more surgeries, higher respiratory rate and injury severity score, and higher levels of HBP [(33.00±3.49) ng/mL vs. (16.27±1.61) ng/mL, P<0.001], leukocytes, and neutrophils [(15.32±3.19) ×10 9/L vs. (6.69±0.57) ×10 9/L, P=0.005] than in the non-infected group, while the Glasgow Coma Scale [(8.72±0.63) vs. (11.37±0.48), P=0.001] was lower than that in the non-infected group, with statistically significant differences (all P<0.05). There was no significant differences in lymphocytes, red blood cells, platelets, calcium, procalcitonin and coagulation indexes between the two groups (all P>0.05). Logistic regression analysis showed that neutrophils ( OR=1.252, 95% CI: 1.075-1.457, P=0.004) and HBP ( OR=1.081, 95% CI: 1.025-1.141, P=0.004) were independent risk factors for infection in patients with traumatic cerebral hemorrhage. The area under ROC curve for HBP of diagnosing early co-infection in patients with traumatic intracerebral hemorrhage was 0.82 (95% CI: 0.71-0.88), the sensitivity was 92.31%, and the specificity was 52.17%. Conclusions:HBP is a valuable predictor of early traumatic intracerebral hemorrhage complicated with bacterial infection in the emergency department, and has a good supplementary value to the existing test indicators.

Objective:To investigate the indication, effectiveness, tolerance, and safety of levosimendan in patients with acute heart failure (AHF) in 20 hospitals in Beijing, China.Methods:This prospective, observational, and multicenter study consecutively enrolled AHF patients who were treated with levosimendan at 20 hospitals in Beijing from April 2020 to March 2022. Baseline demographics, laboratory parameters, clinical presentation, concomitant diseases and medications were collected. After initiation of levosimendan, levosimendan administration, laboratory parameter pre- and post-administration, symptoms improvement, and adverse events were also collected.Results:Totally 800 AHF patients were included, 67% of whom were male, aged (65 ±17) years, 50% of whom had ischemic heart disease, and the left ventricular ejection fraction (LVEF) was (36±11)%. The dose of levosimendan was (11.84 ±2.11) mg and the mean infusion time was (1 450±307) min. Dyspnea was improved in 83.4% of AHF patients at 24 h after treatment. The level of B-type natriuretic peptide (BNP) significantly decreased from 689 (406-1509) pg/mL to 410 (156-697) pg/mL in all patients at 24-72 h after treatment ( P<0.001), and the level of N-terminal pro-brain natriuretic peptide (NT-pro BNP) decreased from 6910 (3 715-13 914) pg/mL to 2 851 (1 288-6 191) pg/mL ( P<0.001). Meanwhile, LVEF level also improved significantly [(40±11)% vs. (36±11)%, P<0.001]. During levosimendan administration, adverse events occurred in 74 (9.3%) patients, including hypotension (5.9%), arrhythmia (1.9%), and other symptoms (1.1%). Among them, 7 patients ( 2 patients with hypotension and 5 patients with ventricular tachycardia) interrupted levosimendan administration. Conclusions:The use of levosimendan is safe, and can improve symptoms reduce BNP or NT-pro BNP levels and increase LVEF level in AHF patients.

Objective:To investigate the effect of improved epidural catheter labor analgesia on the maternal and infant outcome of re-pregnant parturients with scar uterus.Method:A total of 100 cases of re-pregnant women with scarred uterus admitted to Shexian Hospital from January 2019 to January 2020 were selected. According to the random number table, they were divided into the observation group (50 cases) and the control group (50 cases). Parturients in the observation group received a modified epidural catheter for labor analgesia, and parturients in the control group received conventional epidural analgesia. The complications of epidural vascular injury, difficulty in catheterization, multiple catheterization, loss of catheter resistance, urinary retention, intrauterine distress, neonatal asphyxia, postpartum hemorrhage, Apgar score, maternal and neonatal outcomes were compared between the two groups.Results:The rate of epidural vascular injury, difficulty in catheter placement, multiple catheter placement and disappearance of catheter resistancein the observation group were lower than those in the control group: 2.0%(1/50) vs. 14.0%(7/50), 2.0%(1/50) vs. 16.0%(8/50), 0 vs. 8.0% (4/50), 8.0% (4/50) vs. 24.0% (12/50), χ2 = 4.89, 5.98, 4.17, 4.76, P<0.05. The rate of urinary retention, intrauterine distress and neonatal asphyxia in the observation group were lower than those in the control group: 4.0%(2/50) vs. 16.0%(8/50), 2.0%(1/50) vs. 14.0%(7/50), 0 vs. 8.0%(4/50), χ2 = 4.00, 4.89, 4.17, P<0.05. Conclusions:Improved epidural catheter delivery analgesia for patients with scarred uterus can achieve significant results, effectively reduce the incidence of vascular injury, and improve the maternal and infant outcome.

In order to investigate the apoptosis triggered by porcine circovirus type 2 (PCV2) in lymphocytes and the underlying mechanism, the levels of apoptosis and the expression levels of miRNA were examined by flow cytometry, Western blotting and real-time PCR (qPCR). The mimics or inhibitors of miR-125a-5p, an apoptosis-related miRNA, were transfected into PK-15 cells, and the apoptosis rate was examined upon overexpression or inhibition of mir-125a-5p. The target gene of mir-125a-5p was predicted by bioinformatics method, and the regulation of mir-125a-5p on the target gene was analyzed by luciferase reporter assay. The expressions of Bcl-2, Bax, cytochrome C and caspase-3 were detected by Western blotting. The results showed that exosomes secreted by PK-15 cells infected with PCV2 significantly increased the lymphocyte apoptosis rate, which was dose-dependent in certain concentration range. The expression of miR-125a-5p was dramatically increased. The apoptosis rate was increased significantly in the cells transfected with miR-125a-5p. It was predicted that there were binding sites of miR-125a-5p at Bcl-2 3'UTR by TargetScan. The luciferase activity of wild-type pmir-Bcl-2 3'UTR was inhibited significantly by miR-125a-5p mimics, but that of mutant pmir-Bcl-2 3'UTR was not changed. By Western blotting, Bcl-2 was reduced significantly, while Bax, cytochrome C and caspase-3 increased significantly, and the ratio of Bcl-2/Bax was significantly decreased. These results showed that PCV2 up-regulated the expression of miR-125a-5p through exosomes, then inhibited the expression of Bcl-2 at both mRNA and protein level, activated mitochondrial apoptosis pathway and induced apoptosis in lymphocytes.
3' Untranslated Regions ; Animals ; Apoptosis/genetics* ; Caspase 3/genetics* ; Cell Proliferation/genetics* ; Circovirus/genetics* ; Cytochromes c/genetics* ; Luciferases/genetics* ; Lymphocytes/metabolism* ; MicroRNAs/metabolism* ; Swine ; bcl-2-Associated X Protein/genetics*

Hospital culture plays an important role in the orderly operation of large shelter hospitals as well as epidemic prevention and control.From April to May 2022, the shelter hospital of the National Convention and Exhibition Center(Shanghai) had created the large shelter hospital culture co-built by doctors and patients with a greater sense of belonging by taking measures such as joint party building between doctors and patients, giving play to the vanguard force of party members, carrying out various forms of cultural, sports and science popularization activities, encouraging enthusiastic patients to participate in activity planning, focusing on key groups, formulating shelter " residents convention", and so on. These measures ultimately formed cultural adaptation, cultural synchronization and cultural shaping, which were conducive to enhancing the empathy of doctors and patients, improving the effectiveness of medical implementation, and promoting the standardization of shelter management system. This harmonious, warm and autonomous culture co-built by doctors and patients effectively ensures the safe and orderly operation of the shelter hospital, and provides reference for the construction of the cultural system of large shelter hospitals in China.