Objective To investigate the spectrum,diagnosis,time of therapy and management of the congeni-tal heart disease(CHD)in patients with Down′s syndrome(DS).Methods A retrospective report was undertaken of 96 cases in children with DS accompanied by CHD in Department of Pediatric Cardiology,Beijing Anzhen Hospital Af-filiated to Capital Medical University.Data were collected and analyzed about their clinical characteristics,and types of cardiovascular abnormalities,and the important laboratory examinations such as echocardiography and catheterization as well as the procedures of diagnosis and treatments were summarized.Then the interventions,complications and prognosis of different patients were estimated.Results (1)Single congenital heart disease was found in 33 cases (34.38%),a-mong which ventricular septal defect was the most common (14 cases,14.58%),followed by atrioventricular septal de-fect and atrial septal defect (equally,7 cases,7.29%).Multi -cardiovascular abnormalities were discovered in 63 ca-ses,and patent ductus arteriosus turned out to be the most common (42 cases,66.67%).(2)Cat-heterization was car-ried out in 18 cases of serious pulmonary arterial hypertension,and 8 cases were proved resistant pulmonary arterial hy-pertension without operation opportunity.The other 8 cases were estimated as high pulmonary arterial hypertension and medical therapy was suggested before reassessment to reduce surgical risks.(3)Operations were undertaken in 61 ca-ses,among which percutaneous interventional occlusion was performed in 7 cases and surgical interventions were per-formed in 54 patients,in which perioperation complications and death were found in 5 cases and 4 cases,respectively. Conclusions Operation interventions are practicable and most cases recovered well with systematic examinations and assessment in patients with DS and cardiovascular malformations.Early diagnosis and timely interventions are highly suggested.Also close attentions should be paid to follow -up and re -estimation after medical therapy.

Background The pathogenesis and development of cataract is associated with oxidative stress-induced apoptosis of human lens epithelial cells(LECs).BH3-only protein is a factor that can initiate apoptosis,and thus the apoptotic process is probably related to the activation of the c-Jun N-terminal kinase(JNK).However,the relationship between oxidative stress-induced apoptosis of human LECs and the JNK pathway remains to be illuminated.Objective This study was to investigate the effects of the JNK/c-Jun pathway and its target gene,Bim (Bcl-2 interacting mediator of cell death)and PU M A(p53 up-regulated modulator of apoptosis),on oxidative stressinduced apoptosis of human LECs.Methods The human LECs cell line(HLEC-B3)was cultured and passaged in DMEM with 10％ fetal bovine serum in vitro.Confluent cells were incubated in 24 well plates and divided into 4 groups.Hydrogen peroxide(H2O2)(50 μmol/L)was used to treat the cells for 4,8 or 12 hours,and cells without H2O2 treatment served as the control group.Apoptosis was detected using Hoechst 33258 staining and quantified by counting the number of cells with pyknotic nuclei.In addition,confluent cells were seeded in 6 well plates,and Western blot and RT-PCR were used to detect the expression of the caspase-3,c-Jun,Bim and PUMA proteins and their mRNA in HLEC-B3,respectively.The JNK/c-Jun pathway inhibitors,CEP11004 or SP600125,were added into cultured media with H2O2,and cells treated with DMSO or H2O2 only served as negative and positive control groups.The expression of the p-JNK,JNK,p-c-Jun,c-Jun,Bim,PUMA proteins was detected by Western blot and apoptosis was assayed using Hoechst 33258 staining.200 pmoL/L of Bim or PUMA small interference RNA(siBim or siPUMA)fragments were transfected into the cells for 24 hours,respectively,and H2O2 was then used to treat the cells for 8 hours.The expression of the Bim and PUMA protein and their mRNA in the cells was detected by Western blot and RT-PCR,respectively.Results After H2O2 treatment in HLEC-B3 cells for 4,8,or 12 hours,the rates of apoptosis were 4.30％±1.15％,27.08％±0.74％ and 46.59％±0.91％,showing a significant difference among them (F=1909.433,P=0.000),and those of the 4,8,12 hour groups were significantly increased in comparison to the control group(P =0.049,0.000,0.000).Compared to untreated cells,the levels of expression of the JNK,Bim,PUMA proteins and their mRNA in HLEC-B3 cells were significantly elevated.After the addition of CEP11004 or SP600125,the expression of these protein and mRNA in HLEC-B3 cells in the presence of H2O2 was significantly weaker than that in the DMSO control group(P =0.000,0.000).After the tranfection of siBim or siPUMA,the apoptosis rates of the H2O2 treated groups were significantly higher than those in the Bim-/-or PIMA-/-group (P＜0.05).Conclusions H2O2 can activate the JNK/c-Jun pathway and up-regulate the expression of its target genes Bim and PUMA in human LECs in a time-dependent manner.Inhibiting the JNK/c-Jun pathway and interfering with the expression of Bim and PUMA can protect human LECs against oxidative stress-induced apoptosis.

OBJECTIVETo explore the diagnosis, treatment, and prognosis of prostatic malignant mesenchymal tumors (PMMT).

METHODSWe retrospectively analyzed the clinical and follow-up data about 20 cases of PMMT and reviewed the literature relevant to the diagnosis, treatment, and prognosis of the disease.

RESULTSBased on the results of pathology and immunohistochemistry, the 20 PMMT cases included leiomyosarcoma (n = 7), rhabdomyosarcoma (n = 5), prostatic stromal sarcoma (n = 3), chondrosarcoma (n = 1), and undifferentiated PMMT (n = 4). Twelve of the patients were treated by radical prostatectomy (3 concurrently by sigmoid colostomy and 1 by cystostomy), 2 by pelvic tumor resection following arterial embolization, 1 by total pelvic exenteration, 1 by colostomy with pelvic lymph node biopsy, and 4 by conservative therapy because of metastasis to the lung, pelvis and bone. Of the 20 patients, 9 died of systemic metastasis within 3 months after treatment, 3 died at 6, 7, and 14 months, respectively, 3 survived with tumor for 5, 11, and 12 months, respectively, 2 survived without tumor for 12 and 24 months so far, all subjected to periodic chemotherapy postoperatively, and 3 lost to follow-up.

CONCLUSIONPMMT is a tumor of high malignancy and rapid progression, for which transrectal ultrasound-guided biopsy remains the main diagnostic method. The clinical stage of the tumor is an important factor influencing its prognosis and the survival rate of the patients can be improved by early diagnosis and combined therapy dominated by radical prostatectomy.

Combined Modality Therapy ; methods ; Humans ; Immunohistochemistry ; Male ; Mesenchymoma ; mortality ; pathology ; therapy ; Prognosis ; Prostatectomy ; Prostatic Neoplasms ; mortality ; pathology ; therapy ; Retrospective Studies

Antibodies, Viral ; blood ; China ; epidemiology ; Humans ; Yellow Fever ; epidemiology ; Yellow fever virus ; immunology

OBJECTIVETo identify and characterize mesenchymal stem cells from synovial membrane of temporomandibular joint in vitro.

METHODSSynovial mesenchymal stem cells (SMSCs) were obtained by limited dilution method and expanded in 25 ml flasks. Methyl thiazolyl tetrazolium (MTT) method was used to determine the cell growth cycles. The expressions of vimentin and keratin were respectively detected with immunocytochemistry, while the expressions of CD8, CD34, CD44, vascular cell adhesion molecule-1 (VCAM-1) and intercellular adhesion molecule-1 (ICAM-1) were determined by flow cytometry.

RESULTSPure mesenchymal stem cells were of spindle shape and uniform in size, which were intensively positive in vimentin, but negative in keratin. The expression of CD44, VCAM-1 and ICAM-1 were also verified by flow cytometry.

CONCLUSIONSMesenchymal stem cells could be purified from adult synovial membrane of temporomandibular joint.

Cell Differentiation ; Cell Separation ; methods ; Cells, Cultured ; Flow Cytometry ; Humans ; Mesenchymal Stromal Cells ; cytology ; Synovial Membrane ; cytology ; Temporomandibular Joint

Objective To evaluate the curative value of the tauroursodeoxycholic acid (TUDCA) and Danning table (DN) in the prevention of bile duct stones recurrence after ERCP. Methods 210 patients with Choledocholithiasisby ERCP were randomly divided into 3 groups. The patients in the control group were not given any medicine. The patients in the TUDCA group took TUDCA every night. The patients in the DN group took Danning tablets of three times a day. The course of medication and followed up for the patients was 24 months after the operation. All the patients would be examined regularly by Biliary color doppler ultrasound and MRCP. The recurrence rates of Choledocholithiasis, cholesterol saturation index and serum lipid were recorded and compared statistically between the three groups. Results In total, 190 patients completed the treatment and follow-up according to the experimental design. The recurrence rates of the control, TUDCA and DN group were 17.46%(11/63), 6.34% (4/63) and 10.94% (7/64). The recurrence rates in the TUDCA and DN group were significantly lower than those the control group (P < 0.05).The recurrence rate in the TUDCA group was significantly lower than that in the DN group; On the tenth days after the operation, the CSI in the TUDCA were significantly lower than those the control group and the DN group (P < 0.05). There was no statistically significant difference between the DN group and the control group (P > 0.05); To the patients without recurrence, the level of TC, LDL in the TUDCA group were significantly lower than those the control group and and the DN group (P < 0.05). The level of HDL in the TUDCA group were significantly higher than those the control group and and the DN group (P < 0.05). There was no statistically significant difference on the level of HDL between the DN groupand the control group (P > 0.05). Conclusion The application of TUDCA and DN for patients with Choledocholithiasis after ERCP can effectively reduce the recurrence rates, the curative effect of TUDCA more than DN.

Objective To systematically evaluate the differences in preoperative planning for left atrial appendage closure(LAAC)using cardiac CT angiography(CCTA)and transesophageal echocardiography(TEE).Methods Relevant studies comparing CCTA and TEE for preoperative assessment of LAAC published from the inception of each database to July 18,2023,were searched in PubMed,EMBASE,the Cochrane Library,CNKI,Wanfang,and CBM database.Meta-analysis was performed using RevMan 5.4 and Stata 15.1.Results A total of 21 studies with 2 137 patients were included.Compared to TEE,preoperative CCTA measurement of the left atrial appendage(LAA)showed a larger maximum diameter(MD 2.27 mm,95％CI 1.70-2.83,P＜0.001)and higher accuracy in predicting occlusion device(RR 1.67,95％CI 1.39-2.01,P＜0.001).Subgroup analysis based on whether 3D reconstruction was performed and occlusion device type showed similar results.There was no statistically significant difference in the surgical success rate(RR 1.03,95％CI 0.98-1.08,P=0.230)between the two imaging methods.Additionally,preoperative 3D reconstruction based on CCTA resulted in fewer occlusion devices used(MID-0.29,95％CI-0.53--0.04,P=0.020),less contrast agent usage(MD-4.48 ml,95％CI-7.40—-1.56,P=0.003),shorter operative time(MD-14.98 min,95％CI-17.79—-12.17,P＜0.001),and a lower rate of postoperative residual leakage(RR 0.59,95％CI 0.36-0.99,P=0.040).Conclusions Compared to TEE,preoperative CCTA for LAAC improves the accuracy of occlusion device prediction.3D reconstruction based on CCTA reduces the occlusion devices used and the usage of contrast agents,shortens the operative time,improves surgical efficiency,and is associated with a lower rate of postoperative residual leakage.Therefore,CCTA has more advantages in preoperative planning for LAAC.

Objective: To evaluate the prognostic value of kinetic changes in minimal residual disease (MRD) status, as well as its relationship with risk stratification, therapeutic response and treatment in patients with newly-diagnosed multiple myeloma (MM) . Methods: A total of 135 patients with newly-diagnosed MM were screened, and 105 patients who achieved VGPR or more as the best responses were included into this study. The MRD status was determined by multiparameter flow cytometry (MFC) at multiple intervals after two cycles of treatment until clinical relapse, death, or last follow-up. The statistical methods included Kaplan-Meier analysis, Cox regression, etc. Results: ①In all 135 patients, 57.8% (78/135) patients achieved MRD negativity (MRD(-)) after treatment. In 105 patients who achieved VGPR and thus included in this study, the MRD(-) rate was 72.4% (76/105) , with a median interval of 3 months from starting treatment to achievement of MRD(-) status. ②The 2-year PFS rate of patients with MRD(-) status was significantly higher than that of MRD(+) status (62.2% vs 41.3%, P=0.001) , while MRD persistence (MRD(+)) was an independent factor for poor prognosis (multivariate analysis for PFS: P=0.044, HR=3.039, 95%CI 1.029-8.974) . ③Loss of MRD(-) status (i.e., MRD reappearance) showed inferior outcomes compared with MRD sustained negative ones, the PFS was 18 months versus not reach (P<0.001) and the OS was not reach for both (P=0.002) . ④The 2-year PFS and OS rates of patients with duration of MRD(-)status≥12 months were significantly higher than those of the control group (PFS: 77.7% vs 36.7%, P<0.001; OS: 96.4% vs 57.9%, P<0.001 respectively) . Duration of MRD(-) status was associated with a marked reduction in risk of relapse or death (univariate analysis for PFS: P<0.001, HR=0.865, 95%CI 0.815-0.918; for OS: P=0.001, HR=0.850, 95%CI 0.741-0.915 respectively) . ⑤Moreover, even in patients carrying high-risk cytogenetic abnormalities (CA) or ineligible for ASCT, MRD negativity remained its prognostic value to predict PFS (high-risk CA medianPFS: not reach vs 19 months, P=0.006; ineligible for ASCT medianPFS: not reach vs 25 months, P=0.052 respectively) . ⑥Last, treatment with the bortezomib-based regimens contributed to prolonged MRD(-) duration (median MRD(-) duratio: 25 months vs 10 months, P=0.034) . Conclusion: Our findings supported MRD(+) status as an independent poor prognostic factor in MM patients, which implicated that duration of MRD(-) status also played a significant role in evaluation of prognosis, while loss of MRD(-)status might serve as an early biomarker for relapse. Therefore, monitoring of MRD kinetics might more precisely predict prognosis, as well as guide treatment decision, especially for when to start retreatment in relapsed patients.
Bortezomib/therapeutic use* ; Humans ; Multiple Myeloma/therapy* ; Neoplasm Recurrence, Local ; Neoplasm, Residual/diagnosis* ; Prognosis ; Risk Assessment ; Treatment Outcome

OBJECTIVETo summarize and analyze the effects of treatment and prognosis of infants with endocardial fibroelastosis (EFE) in different states of the illness undergone relevant therapies, and to understand the roles of different treatments for improving the prognosis of the disease.

METHODSData of 75 cases with EFE admitted into Anzhen Hospital Affiliated to Capital Medical University from August 1984 to June 2006 were analyzed retrospectively.

RESULTS(1) Of the 75 cases with EFE (40 males and 35 females), with the onset age ranged from 20-days to two years and eight months, 69 cases were treated normally and followed up in the Outpatient Department of the Hospital after discharge, the follow-up rate was 92%, with the follow-up span from six months to 23 years (5.7 years in average). During the follow-up, six cases (8.7%) died. (2) The total curative rate of EFE patients was 46.4% (32/69), while the improvement rate was 40.6% (28/69), the total rate of the cure and improvement was 87%. (3) The average value of ejection fraction (EF) of left ventricle of all the patients returned to normal two years after treatment (EF value was 55.86 ± 2.85), the percentage of patients with normal left ventricle EF at 1 year, 3 years, 5 years and 10 years after treatment was 42.6% (26/61), 64.4% (29/45), 70.7% (29/41) and 84.6% (22/26), respectively. The average value of cardiothoracic (C/T) ratio became normal three years after treatment through X ray examination (0.50 ± 0.01), however the average value of the LVDD had not been returned to normal 3 years after treatment. At 1 year, 3 years, 5 years and 10 years after treatment, the proportion of patients with normal LVDD was 0% (0/61), 13.3% (6/45), 53.7% (22/41) and 84.6% (22/26), respectively. (4) The average value of EF became normal one year after treatment in the glucocorticoid group (EF value 58.44 ± 5.10) in 37 cases scored < 22 at the first visit, while the average value of C/T normalized two years after treatment (0.50 ± 0.00); The average value of EF became normal three years after treatment in the glucocorticoid plus cyclophosphamide group (EF 57.33 ± 3.43) in 29 cases scored < 22 at the first visit, however the average value of the C/T and the LVDD did not return to normal 3 years after treatment. (5) Use of IVIG reduced the percentage of patients who received cyclophos-phamide. (6) The recovery of intimal thickness was slow in EFE patients, the span was four years on the average (1 - 8 years), the percentage of patients whose endocardium became normal 1 year, 3 years, 5 years and 10 years after treatment was 9.85% (6/61), 22.2% (10/45), 51.2% (21/41), 100% (29/29).

CONCLUSIONThe long-term continuous normal treatment of patients with EFE showed good therapeutic effects. For severe and refractory cases, immunotherapy must be strengthened and maintained for longer time. For those who clinically recovered, the quantity of activity should be restricted after the treatment is discontinued, and the re-examination should be done timely for further management.

Child, Preschool ; Endocardial Fibroelastosis ; therapy ; Female ; Follow-Up Studies ; Humans ; Infant ; Infant, Newborn ; Male ; Patient Care ; Prognosis ; Retrospective Studies ; Treatment Outcome