Objective:To analyze the clinical characteristics and prognosis of patients with myelodysplastic syndrome (MDS) with a bone marrow nucleated erythroid cell proportion of greater than or equal to 50% (MDS-E) .Methods:The clinical characteristics and prognostic factors of patients with MDS-E were retrospectively analyzed by collecting the case data of 1 436 newly treated patients with MDS diagnosed in the Institute of Hematology and Blood Diseases Hospital, Chinese Academy of Medical Sciences from May 2014 to June 2023.Results:A total of 1 436 newly diagnosed patients with complete data were included in the study, of which 337 (23.5%) patients with MDS-E had a younger age of onset and lower neutrophil and platelet counts compared with those in patients with an erythroid cell proportion of less than 50% (MDS-NE) (all P<0.05). The proportion of MDS cases with ring sideroblasts (MDS-RS) was higher in the MDS-E group than in the MDS-NE group, and multi-hit TP53 mutations were more enriched in the MDS-E group than in the MDS-NE group (all P<0.05). Among patients with MDS-RS, the frequency of complex karyotypes and the TP53 mutation rate were significantly lower in the MDS-E group than in the MDS-NE group (0 vs 11.9%, P=0.048 and 2.4% vs 15.1%, P=0.053, respectively). Among patients with TP53 mutations, the frequencies of complex karyotypes and multi-hit TP53 mutations were significantly higher in the MDS-E group than in the MDS-NE group (87.5% vs 64.6%, P=0.003 and 84.0% vs 54.2%, P<0.001, respectively). Survival analysis of patients with MDS-RS found that the overall survival (OS) in the MDS-E group was better than that in the MDS-NE group [not reached vs 63 (95% CI 53.3-72.7) months, P=0.029]. Among patients with TP53 mutations and excess blasts, the OS in the MDS-E group was worse than that in the MDS-NE group [6 (95% CI 2.2-9.8) months vs 12 (95% CI 8.9-15.1) months, P=0.022]. Multivariate analysis showed that age of ≥65 years ( HR=2.47, 95% CI 1.43-4.26, P=0.001), mean corpuscular volume (MCV) of ≤100 fl ( HR=2.62, 95% CI 1.54-4.47, P<0.001), and TP53 mutation ( HR=2.31, 95% CI 1.29-4.12, P=0.005) were poor prognostic factors independent of the Revised International Prognostic Scoring System (IPSS-R) prognosis stratification in patients with MDS-E. Conclusion:Among patients with MDS-RS, MDS-E was strongly associated with a lower proportion of complex karyotypes and TP53 mutations, and the OS in the MDS-E group was longer than that in the MDS-NE group. Among patients with TP53 mutations, MDS-E was strongly associated with complex karyotypes and multi-hit TP53 mutations, and among TP53-mutated patients with excess blasts, the OS in the MDS-E group was shorter than that in the MDS-NE group. Age of ≥65 years, MCV of ≤100 fl, and TP53 mutation were independent adverse prognostic factors affecting OS in patients with MDS-E.

【Objective】 To investigate the main causes of blood donor deferral in domestic blood center. 【Methods】 The causes of donor deferral were classified into 12 categories as previous medical history, drug use, alcohol consumption, menstrual period, underweight, abnormal blood pressure, abnormal body temperature, abnormal hemoglobin (Hb), lipemic blood, positive hepatitis B surface antigen (HBsAg), elevated alanine aminotransferase (ALT) and others according to the comparison indicators of Asia-Pacific Blood Network (APBN) and the national standard Blood Donor Health Examination Requirements. The relevant data of the top 3 causes of donor deferral, voluntarily reported by the members of Practice Comparison Working Group of China’s Mainland Blood Collection and Supply Institutions from 2014 to 2019, were collected and a histogram was generated. 【Results】 The median donor deferral rate of 20 domestic blood centers from 2014 to 2019 was 12.14%, with the lowest at 0.18% and highest at 32.32%, respectively. The top three causes for donor deferral were elevated ALT, abnormal Hb and abnormal blood pressure in year 2014, 2015, 2018 and 2019; elevated ALT, lipemic blood and abnormal blood pressure in 2016; elevated ALT, abnormal Hb, and lipemic blood in 2017. 【Conclusion】 The main causes of donor deferral were elevated ALT, abnormal Hb, abnormal blood pressure and lipemic blood.

Objective:To investigate the diagnosis method of Gilbert syndrome (GS) and the relationship between UGT1A1 gene polymorphism distribution with serum bilirubin.Methods:Clinical data of 115 GS cases diagnosed in our hospital from January 2013 to November 2018 were retrospectively analyzed. Chi-square test, Fisher’s exact probability method, t-test, and non-parametric test were used for data analysis.Results:115 cases with GS had an average age of (36.89 ± 12.77) years and an average serum total bilirubin level of (44.01 ± 18.78) μmol/L.UGT1A1*28/*28 (21, 18.3%), UGT1A1*1/*28 (17, 14.8%), and UGT1A1*1/*6 (17, 14.8%) were the most common single-site mutations. UGT1A1*1/*28 + *1/*6 (26, 22.6%), UGT1A1*28/*28 + *1/*27 (5, 4.3%) and UGT1A1*1/*28 + *1/*6 + *1/* 27 (5, 4.3%) were the most common multiple-site mutations. Among 110 cases with Gilbert syndrome combined with non-hemolytic diseases, pairwise comparisons showed that the total bilirubin levels of patients with UGT1A1*28/*28 mutations were significantly higher than UGT1A1*6/*6 and UGT1A1*1/*28 + *1/*6 mutation ( P < 0.05). Additionally, with the increase of UGT1A1*28 distribution, the serum total bilirubin level had gradually increased ( P = 0.028), but UGT1A1*6 was opposite ( P = 0.021). There were no significant differences in gene distribution and bilirubin level between GS group (67 cases) and GS combined with viral hepatitis group (32 cases) ( P > 0.05). Conclusion:UGT1A1 gene sequencing detection is a simple, safe, specific and sensitive effective method to assist GS diagnosis. It can reduce the misdiagnosis and mistreatment of clinical jaundice, thus reducing the patients’ psychological burden and saving the limited medical resources. It is worthy of clinical application.

Objective:To investigate the expression and role of chemokine CCL21 and its receptor CCR7 in ischemic brain white matter lesion (WML).Methods:Permanent bilateral common carotid artery occlusion (BCCAo) was used to prepare an ischemic WML model in C57Bl/6 mice. Evans blue perfusion staining was used to assess the permeability of the blood-brain barrier. Immunofluorescence staining was used to detect the expression of CCL21 and its receptor CCR7 in ischemic WML tissues. Cerebral microvascular endothelial cells were subjected to oxygen glucose deprivation (OGD) to prepare ischemic WML model in vitro. The expression level of CCL21 protein after OGD 6 hours and reoxygenation 24 hours was detected by Western blot analysis. Primary cultured oligodendrocyte precursor cells (OPCs) were treated with recombinant CCL21, and then inflammatory reaction was induced by lipopolysaccharide. The mRNA expression level of myelin basic protein (MBP), a marker of mature oligodendrocytes, was detected by quantitative polymerase chain reaction. Results:Compared with the sham operation group, the exudation of Evans blue stain in the brain tissue was significantly increased after BCCAo ( P<0.05), the expression level of CCL21 in cerebral vascular endothelial cells and CCR7 in OPCs was significantly down-regulated ( P<0.05). After cerebral microvascular endothelial cells were subjected to OGD, the expression level of CCL21 protein were significantly down-regulated compared with the control group ( P<0.05). After OPCs were treated with recombinant CCL21, the expression level of MBP mRNA was significantly up-regulated ( P<0.05). Conclusions:The expression of CCL21/CCR7 was significantly down-regulated after WML. The application of recombinant CCL21 may promote the differentiation and maturation of OPCs cells, suggesting that it may play an important role in ischemic WML.

The clinical data of 69 patients with non-HIV-related cryptococcal meningitis admitted in the Second Hospital of Hebei Medical University from January 2013 to May 2019 were analyzed retrospectively.The main presentations of 69 patients are headache,fever,nausea,vomiting, visual impairment, hearing damage.Among them, 36 cases (52%) had underlying diseases, 30 cases (43%) were misdiagnosed, and 38 cases (55%) were complicated with high intracranial pressure. Cerebrospinal fluid examination showed that leukocytes increased in 47 cases, protein increased in 55 cases, chloride decreased in 41 cases, glucose decreased in 34 cases. The imaging findings were cerebral ischemia, hydrocephalus, meningeal or cerebral parenchyma enhancement. During the induction period, 63 cases were treated with combined antifungal drugs and 6 cases were treated with single antifungal drugs. The clinical symptoms were improved in 54 cases, 9 cases were discharged automatically and 6 cases died. The clinical manifestations, routine and biochemical examination of cerebrospinal fluid, imaging findings are not specific for patients with non-HIV-related cryptococcal meningitis.So early and multiple lumbar puncture should be performed to find etiological evidence to reduce misdiagnosis. The combination of antifungal drugs during the induction period is safe and effective.

Objective: To investigate the causes of disease among patients with liver disease hospitalized in Department of Infectious Diseases in our hospital and the changes in such causes within the past 20 years. Methods: A retrospective analysis was performed for the clinical data of 7570 patients who were admitted to our hospital from January 1995 to December 2015. The chi-square test was used for the statistical analysis of constituent ratio. Results: Of all 7570 patients with liver disease, 4930 (65.13%) had viral hepatitis, 332 (4.39%) had immune disease, 215 (2.84%) had drug-induced liver injury, 192 (2.54%) had fatty liver disease, 88 (1.16%) had schistosome-induced liver disease, 160 (2.11%) had inherited metabolic diseases, and 20 (0.13%) had vascular disease; 689 (9.1%) still had no clear cause of disease at discharge. The proportion of patients with viral hepatitis was 77.61% in the first 10 years and 59.19% in the last 10 years (P < 0.01). As for liver disease caused by hepatotropic virus, there were significant increases in the proportion of patients with hepatitis C or hepatitis E from the first to the last 10 years (hepatitis C: 2.24% vs 15.56%, P < 0.01; hepatitis E: 18.61% vs 23.07%, P < 0.05), while there were significant reductions in the proportion of patients with hepatitis B (68.14% vs 60.01%, P < 0.05) or hepatitis A (10.7% vs 1.36%, P < 0.05). The proportion of patients with immune diseases was 0.82% in the first 10 years and 6.08% in the last 10 years (P < 0.01). There were also certain changes in the proportion of patients with liver disease caused by other reasons. Conclusion There is a large proportion of patients with viral hepatitis among patients with liver disease hospitalized in Department of Infectious Diseases in a large general hospital, especially hepatitis B and E caused by hepatotropic virus. There is a certain change in the epidemiology of liver disease within the past 20 years, with a reduction in the proportion of patients with viral hepatitis and an increase in the proportion of patients with non-infectious liver diseases. There is a large proportion of patients with unknown causes of liver disease.

Purpose To analyze the hemodynamic characteristics of brain stem in patients with basilar artery hypoplasia (BAH) by magnetic resonance perfusion-weighted imaging (PWI).Materials and Methods According to the inclusion and exclusion criteria,51 patients with BAH were selected as the BAH group,and 79 patients without BAH were selected as the non BAH group.All patients were examined by MRI,3D-TOF and PWI,and magnetic resonance angiography was acquired after the three examinations.The regional cerebral blood flow (rCBF),regional cerebral blood volume (rCBV),regional mean transit time (rMTT) and time to peak (TTP) values of pontine area were measured.Results The rCBF value of the BAH group [(17.10±6.52) ml/(100 g · min)] was significantly lower than that of the non BAH group [(29.06± 13.32) ml/(100 g · min)] (P＜0.05);the rCBV value of the BAH group [(1.41 ±0.26) ml] was significantly lower than that of the non BAH group [(2.62± 0.82) ml] (P＜0.05);the TTP value of the BAH group [(6.14± 1.31) s] was significantly higher than that of the non BAH group [(5.39 ± 1.08) s] (P＜0.05);the rMTT value of the BAH group [(20.78±3.48) s] was significantly higher than that of the non BAH group [(19.01 ±2.39) s] (P＜0.05).TTP was the most sensitive index of cerebral perfusion injury,and the incidence of TTP extension was 41.18％ in the BAH group.Conclusion PWI can detect the abnormal cerebral hemodynamics in patients with BAH,which provides the basis for the timely treatment and prevention of irreversible injury in the ischemic area of the brain.

Objective To investigate the role of S100B protein in the pathogenesis of patients with Japanese encephalitis (JE).Methods A total of 45 patients were enrolled in the Second Hospital of Hebei Medical University from August 2013 to October 2013,who were diagnosed as JE on the basis of clinical features and positive IgM antibodies against JE virus measured by enzyme-linked immunosorbent assay (ELISA) from the Center of Disease Control of Shijiazhuang.The JE patients were divided into initial phase group,acute phase group and convalescence group based on the course,mild JE group,moderate JE group and severe JE group based on the severity,MRI-no-lesion group and MRI lesions group based on the imaging findings of JE.Twelve cases with no evidence of infection in central nervous system in the meantime were chosen as control.The S100B protein was measured by ELISA.Results The content of S100B protein in cerebrospinal fluid was as follows:522.76 (393.35,620.37) pg/ml in mild JE group (acute phase group:609.77 (549.27,779.71) pg/ml,convalescence group:420.48 (344.36,453.19) pg/ml),792.09 (705.47,1 108.96) pg/ml in moderate JE group (acute phase group:770.19 (646.31,1 069.54) pg/ml,convalescence group:803.45 (602.90,1 396.84) pg/ml),and 1 021.94 (680.84,1 302.15) pg/ml in severe JE group (acute phase group:981.82 (680.84,1 826.28) pg/ml,convalescence group:989.00 (553.62,1 207.67) pg/ml).The S100B protein content was 561.52 (454.36,814.56) pg/ml,803.45 (602.90,1 104.01) pg/ml,762.22 (594.95,1 044.97) pg/ml,581.76 (442.51,1 069.10) pg/ml in MRI-no-lesion group,MRI lesions group,total acute phase group and total convalescence group,respectively.While in control group,the S100B protein content was 266.71 (205.72,390.05) pg/ml.The contents of S100B protein in moderate JE group,severe JE group,total acute phase group,total convalescence group,MRI-no-lesion group,MRI lesions group were higher than that in control group (H =4.864,5.497,5.075,3.918,2.971,4.981,P =0.000,0.000,0.000,0.000,0.009,0.000).The contents of S100B protein in mild JE group was lower than that in moderate JE group and severe JE group (H =-2.786,-3.514,P =0.032,0.003).Conclusions The level of S100B protein in cerebrospinal fluid is related with the severity,duration and imaging presentation of JE patients.The dynamic monitoring of S100B protein levels is of great significance for assessment of the patients' condition and curative effect.

BACKGROUND:Anatomic medul ary locking (AML) femoral prosthesis is circular cylinder and has satisfactory efficacy. However, some scholars found the complications such as thigh pain, loss of bone at the proximal end of the femur, and wearing-related osteolysis. F2L femoral prosthesis is cone-shaped and also has satisfactory efficacy, but the thigh pain incidence is relatively low. ＜br> OBJECTIVE:To compare the intermediate-long term results of AML versus F2L in total hip arthroplasty. ＜br> METHODS:Between November 1997 and January 2005, we retrospectively reviewed 60 patients (66 hips) undergoing total hip arthroplasty using biological femoral prosthesis. At fol ow-up examination, 58 hips in 52 patients were available for clinical and roentgenographic review. 26 AML devices were placed in 24 patients, and 32 F2L devices were placed in 28 patients. The AML group were reviewed with an average of 12.7 years fol ow-up (range 10 years and 3 months to 15 years and 5 months), while the F2L group were reviewed with an average of 9.5 years fol ow-up (range 8 years and 3 months to 11 years and 1 month). The clinical results were evaluated with Harris methods and X-ray examination. Kaplan-Meier analysis was performed to evaluate the survival of femoral component. End point was radiographical loosening or revision of the femoral component for any reason. ＜br> RESULTS AND CONCLUSION:There were no significant difference between AML and F2L about Harris score in the latest fol ow-up (P>0.05). After surgery, the incidence of thigh pain was significantly lower in F2L group than that in AML group (P＜0.05). In AMKL group, the stress-shielding 1 level was observed in 21 hips (81%), and 2 level in five hips (19%);in F2L group, the stress shielding 0 level was observed in 20 hips (62%) and 1 level in 12 hips (38%). There were significant differences between the two groups (P＜0.05). The stress shielding showed significant differences between the two groups (P＜0.05). The incidence of osteolysis in F2L group was significantly lower than that in AML group (P＜0.05). Kaplan-Meier analysis showed that, the survival rate of both AML and F2L components were 1.0 (95%confidence interval:0.98-1.00). Experimental findings indicate that, both AML and F2L femoral prosthesis have a satisfactory long-term efficacy after total hip arthroplasty, and the incidence of thigh pain and osteolysis is significantly lower in F2L group.

The noninvasive measurement of liver stiffness (LS) was evaluated by transient elastography (FibroScan) and the possible influencing factors from the patients' clinical situations including age, gender, liver inflammation represented by alanine transaminase (ALT) and total billirubin (TBIL) level, HBV replication (HBV DNA loads), portal vein pressure (portal vessel diameter, PVD), splenic thickness (SPT) and body mass index (BMI) were analyzed in patients with chronic hepatitis B (CHB). A total of 466 patients including 31 patients with acute-on-chronic liver failure (ACLF), and 435 patients with chronic hepatitis B (CHB) among which 82 patients were diagnosed with liver cirrhosis (LC) by clinical manifestations and liver B-type ultrasonic inspection were enrolled at Tongji Hospital from April to December 2009. LS was measured by a FibroScan device (EchoSens, France). Simultaneously, ALT and TBIL levels, HBV DNA loads, PVD, SPT and BMI in all patients were also tested. Forty-one healthy volunteers served as controls. The values of LS were correlated positively with ages of CHB patients and significantly higher in males than in females. In patients with BMI>28 kg/m(2) (obesity) and abnormal levels of ALT and TBIL, LS values were significantly increased as compared with those having normal levels of ALT and TBIL. The patients with ACLF had the highest LS value. Furthermore, LS values in the patients with LC were significantly higher than those in patients without LC. It is concluded that noninvasive measurement of liver fibrosis by FibroScan provides an alternative method to evaluate liver fibrosis of patients with CHB. In order to properly illustrate the stiffness value taken by transient elastography, patients' gender should be taken into consideration and it is also suggested to avoid possible influencing factors including liver inflammation (high levels of ALT and TBIL) and obesity (high BMI).