Objective To analyze the variations of glycerol-3-phosphate dehydrogenase 1 like gene (GPD1-L) and address the association with sudden m anhood death syndrom e (SMDS). Methods The genom ic DNA was extracted from blood sam ples of the SMDS group and the norm alcontrolgroup.The exons, exon-in-tron boundaries and 3′-U TRs of coding region of GPD1-L w ere PCRam plified and DNAsequenced di-rectly to confirm the types of variations. The genotype frequency and allele frequency w ere analyzed statistically. Results There w ere tw ovariants in the SMDS group, c.465C>Tand c.*18G>T, the latter existed certain degree difference of genotype distribution and allele frequency betw een the SMDS group and the control group, but there was no statistically significant (P>0.05). Conclusion The relation be-tw een gene m utation of GPD1-L and the occurrence of Chinese SMDS deserves a further research.

BACKGROUND:Human mesenchymal stem cells derived from Wharton's jelly(WJCs)display the characteristics of MSCs as defined by the International Society for Cellular Therapy.They can be differentiated into bone,cartilage,adipose,muscle,and neural cells.They can also support the expansion of other stem cells,be weli-tolerated by the immune system,and have the ability to home to tumors.OBJECTIVE:To investigate biological changes of WJCs and brain tumor stem cells(BTSCs)co-cultured in vitro.METHODS:WJCs cultured by situ cultivation and BTSCs used enzyme digestion way respectively,and gathering the 3rd passage of WJCs though subculturing as well as BTSCs.Two kinds of cells co-cultured in 24-well plates in serum-free medium (SFM)without any growth factor.3 and 7 days after co-cultured respectively,CD133 expression of suspension cells in the 24-well plates were identified by flow cytometry,and immunofluorescence was performed for Nestin and glial fibrillary acidic protein (GFAP)expression of adherent cells.Co-culture supernatant(CCS)re-suspended 3~(rd) passage of BTSCs and cultured into 96-well plates at day 3,which were used to determine the difference in cell growth curve in both groups using a microplate reader.RESULTS AND CONCLUSION:With the cocultivation days increasing,the phenomenon that tumor sphere cells began to be decomposed,adherent and differentiated observed by an inverted microscope.BTSCs in the co-cultured group expressed GFAP and Nestin when adherent and differentiated.The higher degree of malignant brain tumor tissue used in culturing BTSCs was,the higher expression of CD133 in BTSCs was.CD 133~+ in BTSCs declined when co-cultured with WJCs.Growth curve of brain tumor stem cells cultured in CCS compared with in SFM at day 3,which indicates that the proliferation of BTSCs inhibited obviously.Results indicated that CD 133~+ expression and proliferative capacity of BTSCs went down and BTSCs underwent differentiation during the co-culture in vitro.

BACKGROUND:Bone marrow mesenchymal stem cells have low immunogenicity and immunomodulatory effect,but there are seldom reports concerning the immunomodulatory effect of Wharton's jelly-derived mesenchymal stem cells of human umbilical cord and its mechanims.OBJECTIVE:To investigate the immunomodulatory effects and mechanisms of Wharton's jelly-derived mesenchymal stem cells of human umbilical cord on varient peripheral blood T lymphocytes.METHODS:Mesenchymal stem cells were isolateded from Wharton's jelly of human umbilical cord by tissue culture.T lymphocytes from human peripheral blood were stimulated by phytohemagglutinin and co-cultured with umbilical cord Wharton's jelly-derived mesenchymal stem cells and umbilical cord Wharton's jelly-derived mesenchymal stem cells supernatant respectively to measure A value following 72 hours of coculture using multifunctional microplate reader.Expression of cytokines including transforming growth factor-beta 1(TGF-β1)and interferon-y(IFN-γ)was evaluated by enzyme-labeled immunosorbent assay.RESULTS AND CONCLUSION:Wharton's jelly-derived mesenchymal stem cells could inhibite the proliferation of T lymphocytes induced by phytohemagglutinin.The proliferation inhibition rate was 56%(P＜0.01).Wharton's jelly-derived mesenchymal stem cells supernatant also had inhibitory effects on proliferation of T lymphocytes induced by phytohemagglutinin,in a dose-dependent fashion.The proliferation inhibition rates were 8.3% and 27% respectively in the 50% Wharton's jelly-derived mesenchymal stem cells supernatant and 100% Wharton's jelly-derived mesenchymal stem cells supematant groups(P＜0.05).Wharton's jelly-derived mesenchymal stem cells significantly decreased γ-interferon secrted from T-lymphocytes(P＜0.05).The secretion of TGF-β1 was lower in the coculture of Wharton's jelly-derived mesenchymal stem cells and T lymphocytes group than Wharton's jelly-derived mesenchymal stem cells alone group(P＜0.05).These indicated that Wharton's jelly-derived mesenchymal stem cells and Wharton's jelly-derived mesenchymal stem cells supernatant have inhibitory effects on proliferation of T lymphocytes induced by phytohemagglutinin.The mechanims may be associated with cell contant and inhibition of v-interferon secrted from T-lymphocytes.

Objective To evaluate left ventriclular systolic function in patients with obstructive hypertrophic cardiomyopathy (HOCM) after modified Morrow surgery using two-dimensional speckle tracking imaging (2D-STI).Methods Twenty three HOCM patients were recruited in this study.Echocardiographic data from HOCM patients during pre-operation,1-month and 3-month post-operation were analyzed by Qlab software to compare the variation in systolic function indicators 1-month and 3-month post-operation including global and 16 segmental longitudinal strain,circumferential strain and conventional echocardiographic parameters of left ventricle.Results Compared with preoperative data of HOCM patients,postoperative LVOT diameter and pressure gradient,left atrial diameter and volume index were significantly decreased(P<0.05),but there was no significant difference in left ventricular ejection fraction(P<0.05).Compared with the preoperative case,global and segmental longitudinal strain showed significant reduction after 1 week and gradually recovered after 3 months,without significant variation.The longitudinal strain of the anteroseptum reduced significantly and the longitudinal strain of the free wall increased after 3 months,however,the circumferential strain reduced significantly.The circumferential strain of basal and middle segment after 3 months had improved significantly than those of postoperative 1 week.The circumferential strain of surgical site is no obvious change and the strain of free wall was improved after 3 months.Conclusions 2D-STI can effectively evaluate global and regional systolic function of left ventricle for HOCM patients after modified Morrow surgery.

Objective: To evaluate the predicting value of the 2014 European Society of Cardiology (ESC) guidelines risk prediction model for sudden cardiac death (HCM Risk-SCD) in Chinese patients with hypertrophic cardiomyopathy (HCM), and to explore the predictors of adverse cardiovascular events in Chinese HCM patients. Methods: The study population consisted of a consecutive 207 HCM patients admitted in our center from October 2014 to October 2016. All patients were followed up to March 2017. The 5-year SCD probability of each patient was estimated using HCM Risk-SCD model based on electrocardiogram, echocardiography and cardiac magnetic resonance (CMR) examination results. The primary, second, and composite endpoints were recorded. The primary endpoint included SCD and appropriate ICD therapy, identical to the HCM Risk-SCD endpoint. The second endpoint included acute myocardial infarction, hospitalization for heart failure, thrombus embolism and end-stage HCM. The composite endpoint was either the primary or the second endpoint. Patients were divided into the 3 categories according to 5-year SCD probability assessed by HCM Risk-SCD model: low risk group<4%,intermediate risk group ≥4% to<6%, and high risk group≥6%. Results: (1) Prevalence of endpoints: All 207 HCM patients completed the follow-up (350 (230, 547) days). During follow-up, 8 (3.86%) patients reached the primary endpoints (3 cases of SCD, 3 cases of survival after defibrillation, and 2 cases of appropriate ICD discharge); 21 (10.14%) patients reached the second endpoints (1 case of acute myocardial infarction, 16 cases of heart failure hospitalization, 2 cases of thromboembolism, and 2 cases of end-stage HCM). (2) Predicting value of HCM Risk-SCD model: Patients with primary endpoints had higher prevalence of syncope and intermediate-high risk of 5-year SCD, as compared to those without primary endpoints (both P<0.05). (3) Predicting value of HCM Risk-SCD model: The low risk group included 122 patients (59%), the intermediate risk group 42 (20%), and the high risk group 43 (21%). There was a clear trend towards to higher heart rate, higher values of PTF_V1 and plane QRS-T angle, higher left ventricular mass index (LVMI), elevated maximal left ventricular outflow tract pressure gradient (LVOT-PGmax), enlarged left atrial dimension(LAD) and volume index (LAVI), reduced systolic mitral annular velocity (s'), and higher late gadolinium enhancement (LGE) volume and mass in patients with high risk of 5-year of SCD, as compared to those with low-intermediate risk (all P<0.05). Moreover, 5-year SCD probability was positively correlated with heart rate, plane QRS-T angle, LVMI, LAVI, LGE%, and negatively correlated with s'(r=0.161, P=0.019; r=0.669, P=0.001; r=0.206, P=0.004; r=0.284, P=0.000; r=0.351, P=0.000; r= -0.245, P=0.001; respectively). (4) LAD, LAVI, e' and s' were independent predictors for poor outcomes. HCM patients with LAD≥39 mm, LAVI≥49.6 ml/m², e'≤6.5 cm/s and s'≤6.6 cm/s were more likely to have adverse cardiovascular events (AUC 0.702, 95%CI 0.604-0.799, P=0.001; AUC 0.700, 95%CI 0.567-0.833, P=0.001; AUC 0.716, 95%CI 0.616-0.817, P=0.000; AUC 0.764, 95%CI 0.676-0.853, P=0.000,respectively). Conclusions The HCM Risk-SCD model is of value in predicting SCD for Chinese HCM patients. The plane QRS-T angle and LGE% are the best predictors of 5-year SCD risk in Chinese HCM patients. Moreover, conventional echocardiographic parameters, including LAD, LAVI, e' and s', are useful to predict adverse cardiovascular events among Chinese HCM patients.

Objective To explore the changes of left ventricular torsion function in patients with latent obstructive hypertrophic cardiomyopathy ( HCM ) ,and provide quantitative informations for clinical evaluation of cardiac function . Methods A total of 49 consecutive patients with HCM without left ventricular outflow tract obstruction at rest were enrolled . All subjects underwent exercise stress echocardiography . After exercise left ventricular outflow tract pressure gradient ( LVO T‐PG ) ≥30 mm Hg was positive for exercise stress test ( latent obstruction) ,w hile LVO T‐PG＜ 30 mm Hg was negative for exercise stress test ( non‐obstruction) . An ultrasound system obtained two‐dimensional ultrasound images of resting and moving peaks . The global longitudinal strain ( GLS ) ,global circumferential strain ( GCS ) , global radial strain ( GRS) of the left ventricle 16 segments and left ventricular rotation ,twist were analysis using off‐line EchoPAC software . T he differences of the above parameters were compared between the two groups . Results T here were no significant differences in GLS ,GRS ,GCS and Rotation‐B between the two groups in resting and peak period of exercise ( all P ＞ 0 .05 ) ,GRS in both groups were significantly increased compared with that before exercise ( all P ＜ 0 .05 ) . Compared with the negative exercise stress group ,the left ventricular twist and Rotation‐A were significantly increased in resting and peak period of exercise in the positive exercise stress test group( all P ＜0 .05) . Compared with before exercise ,Rotation‐A and left ventricular twist were significantly decreased in the positive exercise stress test group ( all P ＜0 .05) ,while no significantly difference was found in the negative exercise stress group ( all P ＞ 0 .05 ) . Conclusions Left ventricular torsion function is significantly changed in rest and after exercise in latent obstructive HCM patients ,providing valuable quantitative information for clinical comprehensive evaluation of cardiac function .

Objective To assess whether strain parameters derived from three-dimensional speckle tracking imaging ( 3D-STI) could identify improvement of cardiac function and predict response of patients with immunoglobulin light-chain cardiac amyloidosis ( AL-CA) following chemotherapy . Methods Totally 13 patients with AL-CA [aged (58 .5 ± 8 .9)years;69％ males] were treated with melphalan or bortezomib-based regimens and by regular chemotherapy for 6 months .The clinical data was collected . Maximal left ventricular wall thickness (MLVWT) ,left ventricular mass index (LVMI) ,left ventricular ejection fraction ( LVEF) ,systolic mitral annular velocity ( s′) ,early diastolic mitral annular velocity echocardiography ( e′) , 3D-STI global longitudinal ,circumferential ,radial and area strain (GLS ,GCS ,GRS ,and GAS) ,the standard deviation of time to peak longitudinal strain among 16 left ventricular segments ( TS-SD_GLS ) were obtained by conventional echocardiography and 3D-STI . In addition ,serologic biomarkers including N-terminal pro-brain natriuretic peptide ( NT-proBNP) and free light chains ( FLC) were acquired at baseline and 6 months after chemotherapy . These patients were divided into two groups according to difference of FLC :complete response (CR) group and Non-CR group . The clinical data ,cardiac ultrasound parameters and serological parameters were compared between groups ,each group parameters at baseline and 6 months after chemotherapy were compared within the group.Results ①There were no significant differencein conventional echocardiographic parameters ,GCS ,GRS and GAS ,as well as TS-SD_GLS in either group between before and after chemotherapy . But GLS was improved only in CR group ( P = 0 .036) ,and its improvement was correlated with the decrease in NT-proBNP ( r = -0 .738 , P = 0 .037) . ② In baseline evaluation ,patients in Non-CR group had increased LVMI ,deteriorated e′ and GLS ,and longer Ts-SD as compared to those in CR group ( all P < 0 .05 ) . ③ ROC analysis revealed that these parameters had discriminative ability to forecast those with better therapeutic effectiveness ,esecially for AL-CA patients with baseline LVMI<96 .55 g/m2 ,e′>4 .7 cm/s ,absolute value of GLS>16 .6％ ,and TS-SD_GLS<35 .2 ms ,which may have better hematologic response to chemotherapy . Conclusions GLS can identify early improvement of cardiac function in AL-CA patients after chemotherapy . Moreover ,LVMI ,e′,GLS and TS-SD_GLS are sensitive measurements of pre-treatment ventricular impairment ,and may predict better response to chemotherapy .

Objective:To explore the value of three-dimensional speckle tracking imaging (3D-STI) in the diagnosis of immunoglobulin light-chain cardiac amyloidosis(AL-CA) patients with normal left ventricular ejection fraction (LVEF).Methods:A total of 92 consecutive patients diagnosed with systemic immunoglobulin light chain amyloidosis(sAL) and with normal LVEF from October 2014 to January 2018 in Xijing Hospital were enrolled.Based on the diagnostic criteria of cardiac involvement, the patients were divided into AL-CA group (52 cases) and immunoglobulin light chain amyloidosis (AL) group (40 cases). The clinical data and serological markers of the patients were collected, the conventional echocardiography and full-volume three dimensional dynamic images were acquired, left ventricular global longitudinal strain (GLS), global radial strain (GRS), global circumferential strain (GCS), and global area strain (GAS) were analyzed using off-line TomTec software. The differences between the two groups were compared.Results:Compared with the AL group, the NT-proBNP of AL-CA group was significantly higher ( P<0.05) and there were no significant differences of the other serological indexes between the two groups(all P>0.05). Compared with the AL group, the maximal left ventricular wall thickness, left ventricular mass index, left atrial volume index, and E/e′ in the AL-CA group were significantly increased (all P<0.05). There were no significant differences of other conventional echocardiographic measurements between the two groups(all P>0.05). Compared with the AL group, GLS, GAS, and GRS were significantly lower in AL-CA group (all P<0.05); but there was no significant difference of GCS between the two groups( P>0.05). The ROC curve analysis showed that the cut-off values discriminating cardiac involvement were 16.09% for GLS, 36.54% for GAS and 31.90% for GRS. Conclusions:3D-STI measurements of left ventricular myocardial mechanics could detect cardiac involvement in patients with sAL amyloidosis, and provides a new method for diagnosis of AL-CA.

Objective:To evaluate the left ventricular myocardial strain in patients with light chain cardiac amyloidosis (AL-CA) with normal left ventricular ejection fraction (LVEF) by three-dimensional speckle tracking imaging(3D-STI), and to explore the clinical value of 3D-STI in predicting the prognosis of AL-CA patients with normal LVEF.Methods:A total of 80 patients with AL-CA and LVEF≥50% were retrospectively analyzed in the Xijing Hospital of Air Force Military Medical University from October 2014 to May 2020.According to whether the patients had endpoint events, they were divided into endpoint event group and non-endpoint event group. The clinical data, conventional echocardiographic parameters, 3D-STI related parameters and follow-up results were collected. Cox regression proportional hazards model was used to analyze the survival status of AL-CA patients with univariate and multivariate regression analyses, in order to find the relevant indicators of conventional echocardiography and 3D-STI to predict adverse events.Results:All patients were followed up for 20(7.3, 40.8) months. At the end of follow-up, 25 patients had all-cause deaths. Compared with the non-endpoint group, the endpoint event group had significantly increased left ventricular end diastolic maximum wall thickness (MLVWT), peak early diastolic flow velocity/peak early diastolic velocity at mitral annulus(E/e′) (all P<0.05), and decreased LVEF, left ventricular global longitudinal strain (GLS) and basal segment longitudinal strain (LS) (all P<0.05). Multivariate cox regression analysis after adjusting for age and gender showed that basal segment LS ( HR=0.812, 95% CI=0.675-0.976, P=0.026) was an independent predictor of end-point events in patients with AL-CA. Kaplan-Meier survival curve showed that AL-CA patients with basal segment LS≤13.07% were more likely to have endpoint events. Conclusions:Basal segment LS can be used as a predictor of endpoint events in patients with AL-CA.