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.

Objective:To investigate the value of systemic inflammatory response syndrome (SIRS), pediatric sequential organ failure assessment (pSOFA) and pediatric critical illness score (PCIS) in predicting mortality of pediatric sepsis in pediatric intensive care units (PICU) from Southwest China.Methods:This was a prospective multicenter observational study. A total of 447 children with sepsis admitted to 12 PICU in Southwest China from April 2022 to March 2023 were enrolled. Based on the prognosis, the patients were divided into survival group and non-survival group. The physiological parameters of SIRS, pSOFA and PCIS were recorded and scored within 24 h after PICU admission. The general clinical data and some laboratory results were recorded. The area under the curve (AUC) of the receiver operating characteristic curve was used to compare the predictive value of SIRS, pSOFA and PCIS in mortality of pediatric sepsis.Results:Amongst 447 children with sepsis, 260 patients were male and 187 patients were female, aged 2.5 (0.8, 7.0) years, 405 patients were in the survival group and 42 patients were in the non-survival group. 418 patients (93.5%) met the criteria of SIRS, and 440 patients (98.4%) met the criteria of pSOFA≥2. There was no significant difference in the number of items meeting the SIRS criteria between the survival group and the non-survival group (3(2, 4) vs. 3(3, 4) points, Z=1.30, P=0.192). The pSOFA score of the non-survival group was significantly higher than that of the survival group (9(6, 12) vs. 4(3, 7) points, Z=6.56, P<0.001), and the PCIS score was significantly lower than that of the survival group (72(68, 81) vs. 82(76, 88) points, Z=5.90, P<0.001). The predictive value of pSOFA (AUC=0.82) and PCIS (AUC=0.78) for sepsis mortality was significantly higher than that of SIRS (AUC=0.56) ( Z=6.59, 4.23, both P<0.001). There was no significant difference between pSOFA and PCIS ( Z=1.35, P=0.176). Platelet count, procalcitonin, lactic acid, albumin, creatinine, total bilirubin, activated partial thromboplastin time, prothrombin time and international normalized ratio were all able to predict mortality of sepsis to a certain degree (AUC=0.64, 0.68, 0.80, 0.64, 0.68, 0.60, 0.77, 0.75, 0.76, all P<0.05). Conclusion:Compared with SIRS, both pSOFA and PCIS had better predictive value in the mortality of pediatric sepsis in PICU.

Objective:To investigate the medication rules of Xin'an medicine for the treatment of melancholia and further analyze the medication ideas of Xin'an physicians in the treatment of melancholia.Methods:The documents of Xin'an physicians treating melancholia in the fifth edition of the Chinese Medical Code and the online database of ancient Chinese medicine were retrieved. Excel was used to extract the prescription information to establish the database. R language was used to analyze the data regarding the medication frequency, nature and taste, association rules, and clustering of the traditional Chinese medicine used in the prescription. Results:A total of 127 effective prescriptions were sorted out, and 177 kinds of Chinese medicines were used with a total medication frequency of 1 031 times. The top three Chinese medicines with the highest frequency of use were Poria cocos (57 times), Licorice (46 times), and Paeonia Lactiflora (40 times). The main nature of herbs was plain and warm nature. The warm herbs were the most frequently used (298 times). The first five flavors of the herbs which were the most used were pungent taste (475 times, 28.70%), bitter taste (459 times, 27.73%), and sweet taste (453 times, 27.37%). The commonly used herbs with confidence coefficient > 0.800 were Licorice + Angelica sinensis, Licorice + Angelica sinensis and Paeonia Lactiflora, Licorice + Bupleurum, Licorice + Atractylodes macrocephala, Cyperus root + Ligusticum Chuanxiong, Angelica sinensis + Atractylodes macrocephala and Licorice, Paeonia Lactiflora + Angelica sinensis and Poria cocos, Licorice + Angelica sinensis and Poria cocos, Licorice + Atractylodes macrocephala and Angelica sinensis, Licorice + Bupleurum and Paeonia Lactiflora, Licorice + Atractylodes macrocephala and Ginseng, Licorice + Ginseng and Angelica sinensis, Cyperus root + Medicated leaven, Ginseng + Astragalus mongholicus, Licorice + Astragalus mongholicus.Conclusion:Xin'an medicine for the treatment of melancholia mainly uses pungent, bitter, sweet, and warm herbs. It can adjust the chill and fever, Yin and Yang of the human body, diminishes the urgency, and regulates the flow of Qi.

Objective:To analyze the correlation between gastric disharmony and daily eating behaviors in college students, and to provide intervention measures for improving gastric disharmony among college students.Methods:From April 10 to 30, 2021, 3 825 college students from five Chinese medicine colleges and universities in China were selected for this study using the multi-level sampling method. The information regarding college student's general situation, daily eating behavior, and stomach disharmony was collected by questionnaire.Results:A total of 3 700 valid questionnaires were collected, with an effective response rate of 96.73%. The average age was (20.0 ± 3.0) years, and 66.41% of college students included in this study were female. Students with different characteristics had different gastric disharmonies and eating behaviors, and gastric disharmony was correlated with eating behavior. Multivariate analysis results showed that snacking ( OR = 1.11, 95% CI: 1.02-1.21), partial eclipse ( OR = 1.12, 95% CI: 1.00-1.26), picky eaters ( OR = 1.23, 95% CI: 1.08-1.41), and supper ( OR = 1.19, 95% CI: 1.04-1.36) were positively correlated with gastric disharmony. Eating regular meals ( OR = 0.86, 95% CI: 0.79-0.94) and 80% fullness ( OR = 0.89, 95% CI: 0.81-0.98) were negatively correlated with gastric disharmony. Conclusion:Poor eating behaviors may be an important factor leading to gastric disharmony.

Objective:To investigate the risk factors of bone cement leakage after percutaneous vertebroplasty (PVP) for osteoporotic vertebral compression fracture (OVCF).Methods:A multi-center, large-sample, case-control study was carried out to analyze the clinical data of 2 273 OVCF patients (2 689 vertebrae) undergone PVP at four hospitals between May 2018 and October 2021, including 994 males and 1 279 females, with the age of 52-91 years [(69.1±3.1)years]. Of all, 581 patients (604 vertebrae) were allocated to leakage group and 1 692 patients (2 085 vertebrae) to no leakage group according to the occurrence of bone cement leakage. The gender, age, fracture sites, vertebral compression degree, endplate integrity of fractured vertebrae, surgical segments, surgical approaches and bone cement injection volume were recorded. Univariate analysis was used to investigate the correlation between those indicators with bone cement leakage. Multivariate Logistic regression analysis was used to identify the independent risk factors for bone cement leakage.Results:Univariate analysis showed that gender, age, fracture sites, vertebral compression degree, bone cement injection volume were related to bone cement leakage after PVP ( P<0.05 or 0.01), but no correlation was found in the endplate integrity of fractured vertebrae, surgical segments and surgical approaches (all P>0.05). Multivariate Logistic regression analysis showed that fracture sites ( OR=1.68, 95% CI 1.11-2.55, P<0.05), vertebral compression degree more than 40% ( OR=1.98, 95% CI 1.29-3.02, P<0.01), bone cement injection volume greater than or equal to 5.5 ml ( OR=1.55, 95% CI 1.07-2.26, P<0.05) were significantly associated with bone cement leakage after PVP. Conclusion:Thoracic vertebral fracture, vertebral compression degree more than 40% and bone cement injection volume greater than or equal to 5.5 ml are independent risk factors for bone cement leakage after PVP in OVCF.

Objective:To investigate the correlation between unhealthy dietary behaviors and traditional Chinese medicine constitution among students from colleges and universities of traditional Chinese medicine.Methods:College students from Shandong University of Traditional Chinese Medicine, Anhui University of Chinese Medicine, Henan University of Chinese Medicine, Shaanxi University of Chinese Medicine and Gansu University of Chinese Medicine were collected during April 10-30, 2021 and grouped taking classes as units. Research subjects were obtained by multistage sampling. Their dietary behaviors, traditional Chinese medicine constitution and demographic characteristics were surveyed using questionnaires. Statistical analysis was performed using SPSS23.0 software.Results:A total of 3825 college students were investigated and 3 700 valid questionnaires (96.7%) were collected. Peaceful constitution was detected in 7.9% of included college students. There was significant difference in peaceful constitution among studies of different sexes and different body types ( P < 0.05). There were significant differences in attention to diet, physical exercise and body weight control, out-of-control diet, excessive eating and drinking, denying body shape, and dissatisfaction with body shape between patients with peaceful and unbalanced constitutions. Multivariate analysis showed that attention to diet ( OR = 1.38, 95% CI: 1.14-1.68), physical exercise and body weight control ( OR = 1.19, 95% CI: 1.03-1.39), dissatisfaction with body shape ( OR = 1.26, 95% CI: 1.10-1.45) were conducive to the development of peaceful constitution. Conclusion:Students from colleges and universities of traditional Chinese medicine have a low proportion of developing peaceful constitution. Dietary behaviors may be an important factor that affects the development of peaceful constitution.

Objective:To investigate how gender differences between the donor and the recipient affect the effectiveness of antithymocyte globulin (ATG) and pure peripheral blood stem cell (PBSC) hematopoietic stem cell transplantation (haplo-HSCT) in the treatment of malignant hematological diseases.Methods:From February 2015 to September 2020, 648 hematological malignancies patients underwent myeloablative condition regimen haplo-HSCT treatment at the Bone Marrow Transplant Center of the First Affiliated Hospital of Zhejiang University. The median age was 32 (14-62) years, with 363 males (56.0% ) and 285 females (44.0% ) present. 242 cases of acute lymphoblastic leukemia (ALL) (37.3% ) , 293 cases of acute myeloid leukemia (AML) (45.2% ) , 56 cases of myelodysplastic syndrome (MDS) (8.7% ) , 27 cases of non-Hodgkin's lymphoma (NHL) (4.2% ) , and 30 cases of other hematological malignancies (4.6% ) .Results:① The 3-year overall survival (OS) , DFS, the incidence of Ⅱ-Ⅳ grade acute graft-versus-host disease (aGVHD) , the incidence of Ⅲ-Ⅳ grade aGVHD, the 3-year incidence of moderate & severe chronic GVHD (cGVHD) , severe cGVHD, the 3-year incidence of relapse, and NRM of the whole group were (73.10±1.90) % , (70.80±1.90) % , (33.96±1.87) % , (13.08±1.33) % , (35.10±2.14) % , (10.66±1.38) % , (19.43±1.67) % , and (9.80±1.24) % , respectively. ②There was no statistically significant difference between the donor-recipient gender match and donor-recipient gender mismatch groups in the 28-day cumulative neutrophil engraftment rate, 28-day cumulative platelet engraftment rate, the incidence of Ⅱ-Ⅳ grade aGVHD, the incidence of Ⅲ-Ⅳ grade aGVHD, 3-year OS, 3-year DFS, the cumulative incidence of relapse, NRM, and incidence of moderate & severe cGVHD, severe cGVHD. ③The 28-day cumulative neutrophil engraftment rate did not differ statistically between the male-female, female-female, male-male, and female-male groups ( P=0.148) . The incidence of Ⅱ-Ⅳ grade aGVHD, the incidence of Ⅲ-Ⅳ grade aGVHD, 3-year OS, 3-year DFS, cumulative relapse rate, and NRM, and the incidence of cGVHD were not statistically different among the four groups ( P>0.05) . The 28-day cumulative platelet engraftment rate of the female-male group was significantly lower than male-female group, and the female-female group [ (91.45±2.63) % vs. (94.77±1.75) % , P=0.004; (91.45±2.63) % vs. (95.54±2.05) % , P=0.005]. No significant difference existed in the 28-day cumulative platelet engraftment rate between the female-male group and the male-male group [ (91.45±2.63) % vs. (95.08±1.41) % , P=0.284]. ④Among patients ≤35 years old, the 3-year incidence of severe cGVHD patients receiving sister donors and sibling donors were (26.71±5.90) % and (10.33±4.43) % , respectively ( P=0.054) . Patients accepting daughter donors and son donors had a 3-year incidence of moderate and severe cGVHD that was 40.07% vs. 27.41% , respectively, among those over 35 (40.07±6.65) % vs. (27.41±4.54) % ( P=0.084) . ⑤Female donors to male recipients had a significantly lower 28-day cumulative platelet engraftment rate compared to the other groups [ (91.45±2.63) % vs. (95.08±0.95) % , P=0.037]. ⑥ Female donors to male recipients had a significantly lower 28-day cumulative platelet engraftment rate than the other groups in the ATG-Fresenius (ATG-F) 10 mg/kg group [ (89.29±4.29) % vs. (94.49±1.45) % , P=0.037]. But when compared to the other groups in the Rabbit Antihuman Thymocyte Immunoglobulin (rATG-T) 6 mg/kg group, the 28-day cumulative platelet implantation rate between female donors and male recipients was not significantly different [ (93.44±3.38) % vs. (95.62±1.26) % , P=0.404]. Conclusion:The main clinical outcomes of patients with malignant blood diseases following transplantation are unaffected by the gender combination of the donor and patient in the haplo-HSCT mode based on ATG and PBSC sources. Female donors to male recipients have a lower 28-day cumulative platelet engraftment rate and longer platelet engraftment times.

Objective:To analyze the clinicopathological characteristics and intrahepatic immune cells infiltration condition after Kasai biliary atresia surgery.Methods:Data of 28 cases who underwent liver transplantation in the liver transplantation center of our hospital from June 2017 to March 2019 were enrolled. Of which, 20 cases were in the biliary atresia group (divided into two subgroups: 10 cases without Kasai surgery and 10 cases after Kasai surgery, and latter subsided cholestasis) and 8 cases in the control group. Clinical and pathological morphological characteristics of the groups were compared. Liver tissue sections were stained with immunohistochemistry and CD3, CD4, CD8, CD20, Foxp3, and interleukin-17A were quantitatively analyzed. Kruskal-Wallis test was used to measure the above indicators, and rank-sum test or Fisher's exact test was used to compare the count data.Results:The degree of clinical and pathological cholestasis in the biliary atresia group after Kasai surgery was significantly lower than that of the group without Kasai surgery, and the degree of liver fibrosis was also significantly reduced ( P < 0.05), but there was no statistically significant difference in the degree of inflammation in the portal vein area between the two groups ( P > 0.05). There was statistically significant difference in the types of immune cells infiltrated in the liver ( P < 0.05). Compared with the group without Kasai surgery, the infiltration of CD3, CD8, IL-17A and Foxp3 positive cells in the portal vein area after Kasai surgery group ( P < 0.05) was significantly reduced, but there was no statistically significant difference in the proportion of Foxp3/CD4 positive cells between the two groups ( P > 0.05), which continued to be lower than that of the control group ( P < 0.05). Compared with the non-Kasai surgery group, the proportion of Foxp3/IL-17A and Foxp3/CD8 positive cells in the portal vein area did not increase significantly after Kasai surgery group ( P > 0.05), and remained lower than the control group. However, the proportion of Foxp3/IL-17A and Foxp3/CD8 positive cells was significantly reduced ( P ??< 0.05). Conclusion:Intrahepatic inflammatory cell infiltration and regulatory/effector T lymphocyte proportion dysregulation exist in patients with subsided cholestasis after Kasai biliary atresia surgery, which may be an important factor to promote the disease progression.

Objective: To study the effect of WT1 expression on the prognosis of allogeneic hematopoietic stem cell transplantation (allo-HSCT) in acute leukemia (AL) and its significance as molecular marker to dynamically monitor minimal residual disease (MRD) . Methods: Retrospectively analyzed those AL patients who underwent allo-HSCT in the First Hospital Affiliated to Zhejiang University School of Medicine during Jan 2016 to Dec 2017, a total number of 314 cases, 163 males and 151 females, median age was 30 (9-64) years old. Comparing the difference of WT1 expression at diagnosed, pre-HSCT and after HSCT. Using the receiver operating characteristic (ROC) curve to determine the WT1 threshold at different time so as to predict relapse. The threshold of WT1 expression before transplantation was 1.010%, within 3 months after HSCT was 0.079% and 6 months after HSCT was 0.375%. According to these thresholds, WT1 positive patients were divided into low expression groups and high expression groups. Analyzed the relationship between overall survival (OS) , disease-free survival (DFS) , cumulative incidence of relapse (CIR) and WT1 expression. Results: The OS and DFS of high expression group pre-HSCT were lower than low expression group [69.2% (9/13) vs 89.1% (57/64) , χ²=4.086, P=0.043; 53.8% (7/13) vs 87.5% (56/64) , χ²=9.766, P=0.002], CIR was higher than low expression group [30.8% (4/13) vs 7.8% (5/64) , P=0.017]. There was no significant difference of OS and DFS between high expression and low expression group of 3 months after HSCT (P=0.558, P=0.269) . The OS and DFS of high expression group of 6 months after transplantation were both lower than low expression group (P=0.049, P=0.035) . Multivariate analysis showed that WT1>0.375% when 6 months after transplantation was the only independent prognostic factor for shorter DFS (P=0.022) . There was no statistically significant difference in CIR between the high-expression group and the low-expression group 3 months after transplantation and 6 months after transplantation (P=0.114, P=0.306) . Conclusion High expression of WT1 before and after HSCT was an adverse prognosis factor. It is of clinical practical value to use WT1 as a transplant recommendation index for patients with acute leukemia and as a marker to monitor MRD dynamically.

Objective To explore the MRI findings of placental protusion sign in predicting postpartum hemorrhage in patients with placenta previa.Methods Totally 354 placenta previa patients with whole clinical data underwent MR scaning 2 weeks before operation.Association of postpartum hemorrhage and placental protusion sign was analyzed.Results Among 354 patients with placental previa,the age of the pregnant women (x2 =4.34,P=0.04),gestational age at delivery (x2 =5.19,P=0.02) and the number of cesarean sections (x2 =44.85,P＜0.01) had associated with postpartum hemorrhage.Eight cases had placental protusion sign in MRI,while 6 cases occurred postpartum hemorrhage.The incidence of postpartum hemorrhage was 75.00％ (6/8) and 12.72％ (44/346) in patients with placenta accreta and with placental abruption,respectively (x2 =20.14,P＜0.01).The sensitivity,specificity,odds ratio (95％ confidence interval) and positive likelihood ratio of predicting postpartum hemorrhage was 12.00％ (6/50),99.34％ (302/304),20.59 (4.03,105.23) and 15.68,respectively.Conclusion MRI placental protrusion sign has important clinical reference value in predicting postpartum hemorrhage.