Objectives:To analyze the cardiac involvement of patients with Fabry disease(FD)in Anhui region.Methods:This retrospective analysis included 48 previously and currently diagnosed FD patients(25 males)in Anhui region,overall patient and gender specific cardiac involvement was analyzed.Results:The median age of FD patients is 28.0(19.0,46.0)years.The cardiac manifestations of patients with FD were most commonly characterized by palpitations/arrhythmias(13/42 cases)and exertional dyspnea(11/42 cases),electrocardiographic changes were most commonly characterized by T-wave inversion(22/42 cases),ST-segment depression(16/42 cases),and left ventricular hypervoltage(18/42 cases),cardiac structural and functional changes were most common in papillary muscle hypertrophy(29/36 cases),bilateral sign(22/37 cases)and left ventricular hypertrophy(21/46 cases),as well as reduced left ventricular global longitudinal strain(26/39 cases).Neuropathic pain(28/43 cases)was the most common extracardiac manifestation of FD patients.FD patients of different gender differed in age at diagnosis(P=0.018),alpha galactosidase A activity(P<0.001),globotriaosylsphingosine(lyso-GL3)levels(P<0.001),enzyme replacement therapy rate(P=0.043),dyshidrosis(P<0.01),and the incidence of angiokeratoma(P=0.004).Correlation analysis showed that genotype was not correlated with enzyme activity or Lyso-GL-3 levels,whereas the Sokolow-Lyon index was positively correlated with Lyso-GL-3 levels(ρ=0.423,P=0.008),and the Sokolow-Lyon indices(septal thickness:ρ=0.562,P<0.001;left ventricle posterior wall thickness:ρ=0.569,P<0.001)and QRS duration(septal thickness:ρ=0.543,P<0.001;left ventricle posterior wall thickness:ρ=0.557,P<0.001)were positively correlated with left ventricular wall thickness.Conclusions:Cardiac involvement in patients with FD in the Anhui region is characterised by palpitations or arrhythmias,accompanied by nonspecific electrocardiographic changes.Echocardiography frequently reveals papillary muscle hypertrophy.The manifestation of cardiac involvement in patients of different genders is similar.

Objectives:To analyze the cardiac involvement of patients with Fabry disease(FD)in Anhui region.Methods:This retrospective analysis included 48 previously and currently diagnosed FD patients(25 males)in Anhui region,overall patient and gender specific cardiac involvement was analyzed.Results:The median age of FD patients is 28.0(19.0,46.0)years.The cardiac manifestations of patients with FD were most commonly characterized by palpitations/arrhythmias(13/42 cases)and exertional dyspnea(11/42 cases),electrocardiographic changes were most commonly characterized by T-wave inversion(22/42 cases),ST-segment depression(16/42 cases),and left ventricular hypervoltage(18/42 cases),cardiac structural and functional changes were most common in papillary muscle hypertrophy(29/36 cases),bilateral sign(22/37 cases)and left ventricular hypertrophy(21/46 cases),as well as reduced left ventricular global longitudinal strain(26/39 cases).Neuropathic pain(28/43 cases)was the most common extracardiac manifestation of FD patients.FD patients of different gender differed in age at diagnosis(P=0.018),alpha galactosidase A activity(P<0.001),globotriaosylsphingosine(lyso-GL3)levels(P<0.001),enzyme replacement therapy rate(P=0.043),dyshidrosis(P<0.01),and the incidence of angiokeratoma(P=0.004).Correlation analysis showed that genotype was not correlated with enzyme activity or Lyso-GL-3 levels,whereas the Sokolow-Lyon index was positively correlated with Lyso-GL-3 levels(ρ=0.423,P=0.008),and the Sokolow-Lyon indices(septal thickness:ρ=0.562,P<0.001;left ventricle posterior wall thickness:ρ=0.569,P<0.001)and QRS duration(septal thickness:ρ=0.543,P<0.001;left ventricle posterior wall thickness:ρ=0.557,P<0.001)were positively correlated with left ventricular wall thickness.Conclusions:Cardiac involvement in patients with FD in the Anhui region is characterised by palpitations or arrhythmias,accompanied by nonspecific electrocardiographic changes.Echocardiography frequently reveals papillary muscle hypertrophy.The manifestation of cardiac involvement in patients of different genders is similar.

Objective : To analyze the length of hospital stay of patients with heart failure in a multicenter cohort in order to explore the influencing factors of length of stay and provide data support for further intervention. Methods: A total of 2 794 patients enrolled in the multicenter prospective heart failure cohort were divided into two groups: long hospital stay group (≥9 days) and short hospital stay group ( < 9 days) . The general data of the two groups were compared, and the factors with statistical difference in univariate analysis were included in Logistic multifactor regression analysis to explore the difference in length of hospital stay between the two groups. According to left ventricular ejection fraction(LVEF), patients were divided into heart failure with preserved reduced ejection fraction (HFpEF)group, heart failure with mildly reduced ejection fraction(HFmrEF)group and heart failure with reduced ejection fraction(HFrEF) group, and Logistic multifactor regression analysis was performed to find influencing factors. Results : Logistic multifactor regression analysis showed that LVEF, pneumonia, N⁃terminal pro⁃B⁃type natriuretic peptide(NT⁃proBNP), serum sodium, cardiac resynchronization therapy( CRT) or implantable cardioverter defibrillator(ICD)implantation, β blockers, aldosterone receptor antagonists, positive inotropic drugs and vasodilators were all factors influencing the hospitalization of HF patients. In the HFpEF, HFmrEF, and HFrEF groupsCRT/ICD implantation, positive inotonic drugs, and vasodilator use were suggested to be common factors affecting length of hospital stay in all three groups. Conclusion LVEF, pneumonia, NT⁃proBNP, serum sodium, CRT or ICD implantation, β blockers, aldosterone receptor antagonists, positive inotropic drugs and vasodilators are the influencing factors of hospitalization time in HF patients.

Objective:To investigate the effect of JAK2 V617F gene mutation status and burden on BCR-ABL-negative myeloproliferative neoplasms (MPN).Methods:Clinical data of 199 patients with MPN in Cangzhou Central Hospital in Hebei Province from September 2015 to January 2020 were retrospectively analyzed, and the correlations of JAK2 V617F gene mutation burden with clinicopathological features and prognostic score of MPN patients were analyzed.Results:Of the 199 BCR-ABL-negative MPN patients, 138 patients (69.4%) were positive for JAK2 V617F mutation; of these patients, 64 (88.9%) of 72 patients with polycythemia vera (PV) were mutation-positive, 54 (53.5%) of 101 patients with essential thrombocytosis (ET) were positive, 20 (80.0%) of 25 patients with myelofibrosis (MF) were mutation-positive, and 1 patient with hypereosinophilic syndrome (HES) was mutation-positive. About 55.1% (76/138) of patients had a high JAK2 V617F mutation burden. The mutation burden in PV group was the highest, MF was the second, and ET was the lowest; the mutation burdens in these three groups were (73.9±18.3)%, (59.9±25.2)% and (25.0±16.5)%, respectively. Mutation burden was positively correlated with the white blood cell counts of patients with PV, ET and MF ( r values were 0.626, 0.675 and 0.796, all P < 0.01). JAK2 V617F mutation burden was positively correlated with the prognostic scores of patients with PV and ET ( r values were 0.296 and 0.404, both P < 0.05). Conclusion:The JAK2 V617F mutation burden of BCR-ABL-negative MPN patients is related to clinicopathological factors, and the prognosis of patients with high JAK2 V617F mutation burden is poor.

[Abstract] Objective: To investigate the effect of apatinib (APA) combined with cisplatin (DDP) on the proliferation, invasion and migration capacity of gastric carcinoma (GC) cells and its molecular mechanism. Methods: Cancer and para-cancerous tissue samples resected from 50 GC patients, who were surgically treated in Wuwei People's Hospital from January 2016 to June 2019, were collected for this study; in addition, GC cell lines MGC803 and SGC7901 were also collected. qPCR was used to detect the HMGA2 expression in tissues and mRNA expressions of molecules related to cell proliferation, migration and invasion in GC cell lines. MGC803 and SGC7901 cells were transfected with pcHMGA2 by liposome transfection technology. After treatment with DDP and APA at different concentrations, the cells were divided into NC, pcHMGA2, pcHMGA2+DDP and pcHMGA2+DDP+APA groups. Protein expression of HMGA2 in GC cells was detected by Western blotting, and proliferation, migration and invasion of the cells were detected by MTT and Transwell assay, respectively. Results: The mRNA expression of HMGA2 in GC tissues was higher than that in para-cancerous tissues (P<0.05), and the survival rate of GC patients in the high expression group was significantly reduced (P<0.01). DDP significantly inhibited the proliferation, invasion and migration of MGC803 and SGC7901 cells (all P<0.01); the proliferation, invasion and migration of MGC803 and SGC7901 cells in DDP+APA group significantly decreased (all P<0.01) as compared with DDP group; APA significantly enhanced the inhibitory effect of DDP on HMGA2 expression in GC cells (P<0.01); APA enhanced the anticancer activity of DDP against GC by down-regulating HMGA2 expression. Conclusion: APA promotes the anticancer activity of DDP against GC, and its molecular mechanism is the promotion of the inhibitory effect of DDP on HMGA2 expression.

OBJECTIVE： To investigate the effects of new mode of medical orders evaluation according to disease classification. METHODS： Totally 16 kinds of common diseases were selected from each department of our hospital. Each 10 electronic medical records of each disease were randomly selected from 160 patients in the hospital information system from Jan. 1 to Dec. 31， 2016. The rationality of 10 720 medical orders were evaluated retrospectively during hospitalization. Statistical analysis was made on the relevant indicators such as unsuitable usage and dosage， the proportion of auxiliary drugs and unsuitable indications in medical orders of irrational drug use， and a new mode of prescription comment was established according to the classification of diseases. 9 862 medical orders of same disease types were collected after the implementation （from Apr.-May， 2017） of new mode and intervene to evaluate the irrational rate of them so as to evaluate the effects of new mode. RESULTS： In the samples in 2016， a total of 751 medical orders of irrational drug use were evaluated， the most of them were unsuitable usage and dosage （414 items， 55.12％）， especially in the intracerebral hemorrhage diseases （69 items， 16.67％）； under this kind of disease， most of irrational drug use was excessive dosage of single dosing （31 items， 44.92％）. The top three disease types in the list of the ratio of adjuvant drug cost in total drug cost were surgery for renal tumors （39.43％）， acute cerebral infarction （non-surgery） （37.03％） and intervertebral disc herniation surgery （35.26％）. 70 medical orders of irrational drug use were unsuitable indications （9.32％）. After the application of evaluation mode according to classification of disease types and intervene， the irrational rate of medical orders decreased from 7.00％ （751/10 720） before evaluation to 2.95％ （291/9 862）after evaluation， decreasing by 57.86％. CONCLUSIONS： The application of evaluation mode according to classification of disease types can achieve good results in controlling the irrational rate of medical orders and contribute to rational drug use in clinic.

OBJECTIVE:To explore the medication rules of TCM complex formulas for anti-experimental liver injury. METH-ODS:Usingexperimental liver injuryTCM complex preparationsprotection effectas themes,related literatures about medi-cation rules of TCM complex formulas for anti-experimental liver injury in CJFT,Wanfang database and VIP database were re-trieved,and general information,distribution,use frequency and co-occurrence frequency of single herbal medicine were descrip-tively analyzed. K-means clustering model and Apriori in SPSS Modeler were adopted for cluster analysis(clustering side and im-portance)and correlation analysis in medicines with top 10 use frequency. RESULTS:498 related literatures were retrieved,and 70 valid literatures were involved totally,including 51 TCM complex formulas and 111 Chinese herbal medicines. It was mainly drug for clearing heat,restoring vital energy and blood circulation. Drugs for clearing heat were most widely used(34 varieties),drugs for restoring vital energy showed the highest use frequency,with 63 times(25.82%);Prunella vulgaris had the highest frequency in single herbal medicines(accounting for 25.49%);and P. vulgaris-Salvia miltiorrhiza had the highest co-occurrence frequency in drug pairs. 5 clustering sides were obtained in clustering analysis,which were P. vulgaris-S. miltiorrhiza-Angelica sinensis- Glycyr-rhiza uralensis,Astragalus membranaceus-A. sinensis-Hedyotis diffusa,Artemisia scoparia-Curcuma wenyujin-Plygonum cuspida-tum,P. vulgaris-S. miltiorrhiza-A. scoparia,Schisandra chinensis-G. uralensis. Importance prediction showed,S. chinensis was the most important and P. cuspidatum was the least important. 13 drug pair rules were obtained from correlation analysis,and both sup-port and confidence degrees were low. CONCLUSIONS:The TCM complex formulas for anti-experimental liver injury are mainly based on drugs for clearing heat and restoring vital energy,while the compatibility is varied,and correlation degree is relatively low.

Objective To investigate the significance of CD44v6 expression in acute leukemia( AL) and it's relation with the prognosis of AL. Methods Sixty AL patients were treated by enzyme linked immunosorbent assay( ELISA)as initial treatment group. Fourty-seven cases were remission as remission group, and 20 cases with no-remission group. Meanwhile,45 healthy people were served as the control group. The level of CD44v6 was measured by ELISA. Results The serum CD44v6 in initial treatment patients,remission group, no-remission group and control group were( 179. 34 ± 39. 41 )μg/L,( 190. 61 ± 28. 05 )μg/L,( 106. 72 ± 26. 38)μg/L and(98. 31 ± 21. 78)μg/L respectively,and the difference was significant( F =56. 303,P﹤0. 01),and the CD44v6 of initial treatment group and remission group were higher than that of no-remission group and control group(P﹤0. 05). The leukocyte levels was positive related to CD44v6 levels in 60 patients(r=0. 826,P﹤0. 01),and it was also related to disease stage,extramedullary infiltration(( r=0. 485,0. 512;P﹤0. 01). Conclusion The level of CD44v6 is closely related with the occurrence and development of acute leukemia. The assay of CD44v6 in serum of AL patients is helpful in diagnosing and predicting the risk of metastasis and prognosis in AL.

Background and purpose: Itch protein is an established regulator of T cell immune response thresholds, belong to a class of E3 ubiquitin-transferring enzymes, widely involve in the ubiquitination of several key signaling molecules, such as ZAP70, P85, VAV, PLC-γ, PKC-θ, etc, plays a critical role in tumor induced immu-nosuppression. Itch ligase activity regulate T-cell anergy and development of regulatory T cells in the periphery by modulating key components of T-cell receptor and transforming growth factor-βsignaling. Therefore, manipulation of Itch activities may provide the opportunities to develop future therapies for immune disorders such as autoimmunity and cancer. speciifc small interfering RNA(siRNA) was utilized to silence the expression of Itch gene of T-lymphocytes and investigate the cytotoxicity activity of transfected T lymphocytes against MFC stomach neoplasms cells in vitro. Methods:T lymphocytes were isolated from the spleen of 615 mice and transfected by speciifc siRNA to silence the expression of Itch gene, The expression of Itch protein were examined by Western bolt in each group;72 hours after transfection, The secretion level of IL-2, INF-γwere measured by enzyme-linked immunosorbent assay (ELISA). At the end, the cytotoxicity activity changes against MFC stomach neoplasms cells was compared between transfected T lym-phocytes, negative control and blank control in vitro. Results:Compared with control group, the expression rate of Itch protein of transfected T-lymphocytes was decreased to 16%after transfection 48 hours;72 hours after transfection, the secretion level of IL-2 in transfection group, negative control and blank control respectively were (1 891.96±141.91)pg/mL, (1 241.69±91.67)pg/mL and (1 175.03±89.14)pg/mL (P<0.001), the secretion level of INF-γin transfection group, negative control and blank control respectively were (958.33±75.46)pg/mL, (683.33±66.67)pg/mL and (691.72±68.72) pg/mL (P<0.05). Transfected T lymphocyte also showed more efifcient killing ability against MFC stomach neoplasms cells than negative control and blank control in vitro, the highest killing rate has reached (54.18±2.96)%. Conclusion:Silencing Itch gene can signiifcantly promoted the secretion level of IL-2, INF-γof mice T lymphocyte, enhanced the cytotoxicity activity of T lymphocyte against MFC stomach neoplasms cells in vitro.

Objective To investigate the effect of idarubiein to imensify the MAC (IMAC) as preparative regimen for autologous peripheral blood stem cell transplantation(APBSCT)in acute myeioid leukemia.Methods Fourteen patients with acute myeloid leukemia who underwent APBSCT were analysed. IMAC was used as preparative regimens.Results All patients were engrafted successfully.The disease-free survival could be Ben in 8 cases(57.1%),the median disease-free survival duration were 26(8-72)months and no treatment-related mortality was present.Conclusion This study suggested that addition of Idarubicin to the MAC preparative regimens Was safe,effective and feasible for patients with acute myeloid leukemia,and may improve disease-free survival and overall survival.