ObjectiveTo explore the role of structural MRI in the diagnosis of spinocerebellar ataxia type 3 （SCA3） and further evaluate its correlation with disease severity and disease duration. MethodsWe prospectively enrolled 81 genetically diagnosed SCA3 patients ［59 symptomatic （sym-SCA3） and 22 pre-symptomatic （pre-SCA3）］ and 35 age- and sex-matched healthy controls （HCs）. MRI structural images （3D T1 MPRAGE） and clinical data of all subjects were collected. Three observers with different radiological experience measured the width of the superior， middle and inferior cerebellar peduncle （SCP， MCP and ICP）， the anterior-posterior diameters of the pons and spinal cord at the levels of the foramen magnum and upper edge of the 3rd-5th cervical vertebra. One observer performed the measurements again 2 months later to assess for the intra- and inter-observer reliability， respectively. One-way ANOVA， rank-sum test， ROC curve and Random Forest were used to evaluate the diagnostic value of the above metrics for SCA3， and the correlation between the metrics and clinical variables was analyzed. ResultsNot depending on the radiological experience， the metrics based on morphological MRI showed high intra- and inter-observer reliability， among which bilateral superior and middle cerebellar peduncles performed best. The diameters of bilateral SCP， MCP， ICP， pons and spinal cord （except spinal cord at the level of the upper edge of the 5th cervical vertebra） decreased successively in HCs， pre-SCA3 and sym-SCA3 with a statistical difference （P<0.017）. ROC analysis revealed that the left MCP had the highest diagnostic value for pre-SCA3 （AUC=0.911）， with sensitivity， specificity and a cut-off value of 85.7%， 95.5% and 10.15 mm， respectively. In contrast， the right SCP had the highest diagnostic value for sym-SCA3 （AUC=0.999）， with sensitivity， specificity and a cut-off value of 100%， 98.3% and 2.62 mm， respectively. The Random Forest model based on the above metrics also had high diagnostic efficiency （AUC= 0.970， specificity=93.1%）， and the left MCP contributed the most. Correlation analysis showed that the above metrics had a significantly or moderately negative correlation with the Scale for the Assessment and Rating of Ataxia （SARA） and disease duration （P<0.05）. ConclusionNot depending on radiological experience， measurements of brain structure based on morphological MRI are reliable， which can help diagnose SCA3 and predict disease severity and duration. The left MCP and the right SCP perform best for predicting pre-SCA3 and sym-SCA3， respectively. Therefore， the structural MRI is recommended for assisting the clinical diagnosis of SCA3.

Objective: To systematically evaluate the impact of psychological intervention on nursing staff' compassion fatigue. Methods: From March to May 2020, PubMed, Cochrane Library, EMbase, Web of Science, CNKI, VIP, Wanfang and other databases were electronically searched to collect randomized controlled trials (RCTs) on the influence of psychological intervention on nursing staff' compassion fatigue with the main search terms including compassion fatigue, nurs*, psychological intervention, mental intervention, RCT and so on from inception to March 31, 2020. Screened literature, extracted data and assessed the risk of bias of included studies. The Stata 16.0 software was used to calculate the pooled effectiveness of psychological intervention on nursing staff' compassion fatigue. Results: All 13 RCTs were enrolled, including 940 nursing staff. Meta-analysis results demonstrated that the psychological intervention group was superior to the control group in the improvement of the compassion fatigue score (SMD=-0.96, 95%CI: -1.17-0.74, P=0.001) , compassion satisfaction score (SMD=0.61, 95%CI: 0.45-0.77, P=0.002) , burnout score (SMD=-0.46, 95%CI: -0.62-0.29, P=0.006) , secondary trauma score (SMD=-0.40, 95%CI: -0.68-0.12, P=0.020) , and the difference was statistically significant. Subgroup analysis found that the psychological intervention group was more effective than the control group in improving compassion satisfaction score, reducing burnout score and secondary trauma score, and the differences were statistically significant (P<0.05) in different intervention time (<8 weeks and ≥8 weeks) and intervention methods. Conclusion: The psychological intervention can improve the level of compassion satisfaction, and reduce the compassion fatigue among nursing staff, and have a certain preventive effect on compassion fatigue.
Burnout, Professional/prevention & control* ; Compassion Fatigue/prevention & control* ; Empathy ; Humans ; Nursing Staff ; Psychosocial Intervention ; Quality of Life

Objective: To systematically evaluate the correlation between professional quality of life and social support of Chinese nurses based on Pearson and Spearman correlation coefficients. Methods: In databases including PubMed, Cochrane Library, CINAHL, Medline, CBM, CNKI、Wanfang, and other databases were searched by computer for the literatures on correlation between Chinese nurses' professional quality of life and social support from January 2005 to July 2020. The Chinese and English search terms are "nurse" "professional quality of life" "empathy satisfaction" "empathy fatigue" "professional quality of life" "ProQOL" "comparison satisfaction" "comparison fatigue" "social support" "competent social support" "SSRS" "PSSS", etc. Literatures were screened according to the inclusion and exclusion criteria. After evaluating quality and extracting data, meta-analysis was conducted using RevMan 5.3 software. Results: A total of 12 studies were included. The meta analysis showed that nurses' compassion satisfaction, burnout, secondary traumatic stress were related to social support, summary r were 0.35, -0.26 and -0.23 respectively. The correlation between compassion satisfaction and social support were increased with sample, the south was higher than the north, and comprehensive departments were higher than other departments (P<0.05) . The correlation between burnout and social support were increased with time and sample, and the south was higher than the north, oncology was higher than others, non-random sampling was higher than random sampling, using ProQOL and Perceived Social Support Scale (PSSS) was higher than Professional Quality of Life Scale (ProQOL) and Social Support Racting Scale (SSRS) (P<0.05) . The correlation coefficient between secondary traumatic stress and social support in oncology was higher than others, random sampling was higher than non-random sampling, using ProQOL and PSSS was higher than ProQOL and SSRS (P<0.05) . Conclusion: There is a positive and weak correlation between compassion satisfaction and social support, and a negative and weak correlation between burnout and secondary traumatic stress and social support. There are differences in different time, research design, region and department.
Burnout, Professional ; China ; Cross-Sectional Studies ; Humans ; Job Satisfaction ; Nurses ; Quality of Life ; Social Support ; Surveys and Questionnaires

AIM: To observe the safety and efficacy of stab incision glaucoma surgery(SIGS)in the treatment of adult glaucoma.METHODS: A series of retrospective case studies were carried out from June 2018 to November 2020, the clinical data of 55 cases with 70 eyes of glaucoma treated with SIGS in our hospital were collected. Following up at 6mo after operation, the intraocular pressure(IOP), bleb and postoperative complications were observed.RESULTS: Among the included patients, 30 eyes were performed SIGS alonely, 40 eyes were performed SIGS combined with phacoemulsification. Among them, the operation of 33 eyes(47%)was completely successful, the operation of 28 eyes(40%)was partially successful, and the operation of 9 eyes(13%)were failed. The mean preoperative IOP under medication was 31.82±13.16mmHg, and at 1wk, 1, 3 and 6mo after operation, the mean IOP(14.97±5.25, 17.94±5.24, 18.43±4.74, 17.37±3.36)mmHg were all significantly lower than before operation, and the number of IOP-lowering drugs used at 6mo after operation [0(0, 1)] was significantly lower than before operation [3(2, 3)](P<0.001). At 6mo after operation, the filtering blebs' shape of the patients: 30 eyes(43%)of type I(functional bleb), 31 eyes(44%)of type Ⅱ(functional bleb), 7 eyes(10%)of type Ⅲ(flat bleb)and 2 eyes(3%)of type IV(encapsulated vesicular bleb)were included. During the follow-up period, 2 eyes had hyphema in anterior chamber, 4 eyes had inflammatory reaction in anterior chamber, 3 eyes had low IOP, shallow anterior chamber and excessive filtration, 1 eye had malignant glaucoma, 1 eye had endophthalmitis, 1 eye had choroidal detachment, 1 eye had choroidal detachment, and 9 eyes had scarring of filtering blebs.CONCLUSION: SIGS is effective in the treatment of primary open angle glaucoma, primary angle-closure glaucoma and some secondary glaucoma without serious complications.

Platelet-derived growth factor receptor beta (PDGFRB) rearrangements play an important role in the pathogenesis of eosinophilia-associated myeloid/lymphoid neoplasms. Up to now, more than 70 PDGFRB fusions have been identified. Here, a novel PDGFRB fusion gene CSNK2A1-PDGFRB has been identified in myeloproliferative neoplasm (MPN) with eosinophilia by RNA-sequencing, which has been verified by reverse transcription polymerase chain reaction and Sanger sequencing. The new PDGFRB fusion partner gene CSNK2A1 encoded one of the two catalytic subunit of casein kinase II (CK2). To our knowledge, this is the first report on the involvement of CSNK2A1 in fusion genes, especially fusion with another kinase PDGFRB in MPN. In addition, the CSNK2A1-PDGFRB fusion retained the entire kinase domain of PDGFRB and response to imatinib at low concentration. The patient with CSNK2A1-PDGFRB was sensitive to imatinib treatment and acquired sustained complete remission.

Platelet-derived growth factor receptor beta (PDGFRB) rearrangements play an important role in the pathogenesis of eosinophilia-associated myeloid/lymphoid neoplasms. Up to now, more than 70 PDGFRB fusions have been identified. Here, a novel PDGFRB fusion gene CSNK2A1-PDGFRB has been identified in myeloproliferative neoplasm (MPN) with eosinophilia by RNA-sequencing, which has been verified by reverse transcription polymerase chain reaction and Sanger sequencing. The new PDGFRB fusion partner gene CSNK2A1 encoded one of the two catalytic subunit of casein kinase II (CK2). To our knowledge, this is the first report on the involvement of CSNK2A1 in fusion genes, especially fusion with another kinase PDGFRB in MPN. In addition, the CSNK2A1-PDGFRB fusion retained the entire kinase domain of PDGFRB and response to imatinib at low concentration. The patient with CSNK2A1-PDGFRB was sensitive to imatinib treatment and acquired sustained complete remission.

Female ; Humans ; Middle Aged ; Pemphigus ; diagnosis ; drug therapy ; Pemphigus, Benign Familial ; diagnosis ; drug therapy ; Tacrolimus ; therapeutic use

PURPOSE: The purpose of this study was to investigate the function of Zinc finger protein 488 (ZNF488) in nasopharyngeal carcinoma (NPC). MATERIALS AND METHODS: The endogenous expression of ZNF488 in NPC tissues, normal nasopharyngeal epithelium tissues and NPC cell lines were detected by quantitative reverse transcription polymerase chain reaction. ZNF488 over-expressing and knock-down NPC cell line models were established through retroviral vector pMSCV mediated over-expression and small interfering RNA (siRNA) mediated knock-down. The invasion and migration capacities were evaluated by wound healing and transwell invasion assays in ZNF488 over-expressing and control cell lines. Soft-agar colony formation and a xenograft experiment were performed to study tumorigenic ability in vitro and in vivo. Immunofluorescence and western blotting analysis were used to examine protein changes followed by ZNF488 over-expression. Microarray analysis was performed to explore gene expression profilings, while luciferase reporter assay to evaluate the transcriptive activity of Tcf/Lef. RESULTS: ZNF488 was over-expressed in NPC tissues compared with normal tissues, especially higher in 5-8F and S18, which are well-established high metastatic NPC clones. Functional studies indicate that over-expression of ZNF488 provokes invasion, whereas knock-down of ZNF488 alleviates invasive capability. Moreover, over-expression of ZNF488 promotes NPC tumor growth both in vitro and in vivo. Our data further show that over-expression of ZNF488 induces epithelial mesenchymal transition (EMT) by activating the WNT/beta-catenin signaling pathway. CONCLUSION: Our data strongly suggest that ZNF488 acts as an oncogene, promoting invasion and tumorigenesis by activating the Wnt/beta-catenin pathway to induce EMT in NPC.
Blotting, Western ; Carcinogenesis* ; Cell Line ; Clone Cells ; Epithelial-Mesenchymal Transition* ; Epithelium ; Fluorescent Antibody Technique ; Gene Expression Profiling ; Heterografts ; Luciferases ; Microarray Analysis ; Oncogenes ; Polymerase Chain Reaction ; Reverse Transcription ; RNA, Small Interfering ; Wnt Signaling Pathway* ; Wound Healing ; Zidovudine ; Zinc Fingers

OBJECTIVETo investigate the effect of xy2004, a centchroman derivative, on the proliferation of MCF-7 cells and the mechanisms.

METHODSThe effects of xy2004 on MCF-7 cell proliferation and apoptosis were evaluated with MTT assay and flow cytometry, respectively. The expressions of the apoptosis-related proteins were examined with Western blotting. Competitive estrogen-receptor binding assay was used to investigate the affinity of xy2004 to estrogen receptors (ER).

RESULTSxy2004 induced proliferation of MCF-7 cells at low concentrations but inhibited cell proliferation at high concentrations. The application of tamoxifen inhibited xy2004-induced proliferation of MCF-7 cells. The relative binding affinity of xy9906 to ERα and ERβ, presented as the IC50 value, was 7.38 × 10⁻³ mol/L and 4.12 × 10⁻⁷ mol/L, respectively. Treatment of MCF-7 cells with high-concentration xy2004 reduced the cellular expression of Bcl-2 protein and increased Bax protein expression.

CONCLUSIONAt low concentrations, xy2004 directly stimulates the proliferation of MCF -7 cells through ligand-receptor binding, and at high concentrations, it inhibits the cell proliferation by regulating the expression levels of the apoptosis-related proteins.

Apoptosis ; Breast Neoplasms ; pathology ; Cell Proliferation ; Centchroman ; pharmacology ; Estrogen Receptor alpha ; metabolism ; Estrogen Receptor beta ; metabolism ; Humans ; MCF-7 Cells ; drug effects ; Proto-Oncogene Proteins c-bcl-2 ; metabolism ; Tamoxifen ; bcl-2-Associated X Protein ; metabolism