Objective: To analyze the clinical features and prognostic factors of desmoplastic small round cell tumor (DSRCT). Methods: Clinical datum of 140 patients with DSRCT published from Nov. 2003 to Jul. 2012 were collected and studied retrospectively by searching Medline and Embase databases. The observation indicators were progression-free survival (PFS) or overall survival (OS). Survival rates were calculated using the Kaplan-Meier method and compared between groups using a log-rank test. Multivariate analysis was performed using the Cox model to determine the prognostic factors. Results: Patient median age was (23.2±12.7) years (range 4-74 years, the ratio of male and female was 3.12: 1). Frequent symptoms were abdominal pain (35.7%) and evidence of a palpable mass (20.0%). 106 cases tumors were in the abdominal or pelvic cavity, the remaining were extra-abdominal tumors. The frequency of patients receiving conventional chemotherapy, cytoreductive surgery, neoadjuvant chemotherapy, adjuvant chemotherapy or first-line chemotherapy was 76.4%, 52.1%, 17.1%, 47.9% and 38.6%, respectively. Some patients received adjuvant radiotherapy (17.1%), hyperthermic intraperitoneal chemotherapy (4.1%) and bone marrow transplantation (7.3%). By univariate analysis, male gender, absence of metastasis, effective cytoreductive surgery, chemotherapy and multimodal therapy were significant prognostic factors for prolonged OS (all P<0.05). Primary tumor site, extraabdominal tumors, absence of metastasis and effective cytoreductive surgery were associated with improved PFS (all P<0.05). Cox regression analysis showed effective cytoreductive surgery and chemotherapy were independent prognostic factors. Conclusion: Multimodal therapeutics that clear tumors by surgery, adjuvant therapy are favorable prognostic factors for improved survival level in DSRCT patients.

Objective To investigate the value of high-dose dobutamine stress echocardiography combined with two-dimensional strain imaging in early diagnosis of coronary artery disease. Methods Highdose dobutamine stress echocardiography was performed to 28 patients with suspected coronary artery disease. All wall movements were observed during resting condition and at all stress levels,respectively;the peak systolic longitudinal strain in each endomyocardial segment of left ventricular was measured; the sensitivity and specificity between visual method and two-dimensional strain imaging in diagnosing myocardial ischemia with high-dose dobutamine stress echocardiography were compared. The average peak systolic longitudinal strain was calculated against control group, coronary artery disease group during ischemia segments and non-ischemia segments, and a comparison was made inside each group as well as against the other groups. The area under receiver operating characteristic curve of the peak systolic longitudinal strain was used to predict the sensitivity and the specificity of myocardial ischemia. Results With dobutamine dose of 40 μg·kg-1 · min-1 ,wall motion abnormalities were diagnosed in 6 patients (20 segments) through visual method, myocardial ischemia was found in 15 patients (148 segments) through computing the peak systolic longitudinal strain. Inside the coronary artery disease group during ischemic　segments,the majority of peak systolic longitudinal strain was significantly reduced ( P＜0.05) compared to the non-ischemic segments and the control group. In diagnosing myocardial ischemia in high-dose dobutamine stress echocardiography, the sensitivity of visual method and two-dimensional strain imaging were 35.3％ and 88.2％(P＜0.01), specificity 100％ and 100％(P＞0.05), and accuracy 60.7％ and 92.8％ (P＜0.01). The cutoff value of the peak systolic longitudinal strain was less than or equal to 14.9％, its sensitivity and specificity in predicting myocardial ischemia were 83.3％ and 91.7％,respectively. Conclusions High-dose dobutamine stress echocardiography combined with two-dimensional strain imaging can increase the sensitivity of detecting myocardial ischemia and detect concealed myocardial ischemia. High-dose dobutamine stress echocardiography combined with two-dimensional strain imaging can be used in early diagnosis of coronary artery disease.

Objective To investigate the distribution of water fluoride and the status of water-improving defluoridation projects,thus to explore the condition of endemic fluorosis in Guangxi Province.Methods According to"The National Technical Scheme for Endemic Disease Contml in 2005",the fuorine content in water Was determined by F-ion selective electrode,children's dental fluorosis was checked by Dean method.and the skeletal fluorosis was checked by the standard of clinical scale of skeletal fluorosis.Results 305 water samples in 61 villages were examined,among which 71 waters were exceeded the standard,accounting for 23.28%(71/305).The projects of defluoriding drinking water were running well except one was discarded.The prevalence of dental fluorosis was 13.55%(356/2627),the prevalence of skeletal fluorosis was 4.02%(65/1615).Conclusions The situation of endemic fluorosis control is not optimistic in Guangxi,which needs fuaher prevention and controls.

Objective To construct the molecular transmission networks of HIV-1 CRF01 AE strains circulating in China and to analyze their characteristics. Methods Sequences of the pol genes of Chinese CRF01 AE strains were downloaded from Los Alamos database and the related demographic informa-tion was checked. Transmission networks were created by four steps,including phylogenetic tree construc-tion,transmission cluster extraction,minimum genetic distance identification and network visualization. Chi-square test was performed to analyze the differences in the distribution of different populations in the networks and the differences in the distribution of subjects with different degrees among different sub-populations. De-scriptive analysis was used to investigate the transmission links between sub-populations and various regions. Results The 2 419 sequences constituted 250 separate networks including 847 nodes and 610 edges. The number of subjects covered by different networks ranged from 2 to 25. Subjects with degree ≥2 represented only 26. 4%(224 / 847)of network-individuals,but were connected with 66. 5%(563 / 847)of all network-individuals. There were 37. 6%(669 / 1 781)of men who have sex with men(MSM),28. 4%(102 / 359) of heterosexual men,16. 1%(10 / 62)of intravenous drug users(IDUs)and 30. 4%(66 / 217)of hetero-sexual women involved in the networks(χ2 =23. 774,P﹤0. 001). The percentage of subjects with degree ≥2 was high in MSM(10. 8% ,193 / 1 781)compared to that in heterosexual men(5. 6% ,20 / 359)(P =0. 002)and heterosexual women(4. 6% ,10 / 217)(P=0. 004). Of the 669 MSM in the networks,95. 5%(639 / 669)linked to other MSM and only 2. 4% (16 / 669)linked to heterosexual women. However, 15. 1%(10 / 66)of the heterosexual women in the networks linked to MSM. Of the heterosexual men in the networks,35. 3%(36 / 102)linked to heterosexual women,9. 8%(10 / 102)linked to MSM. Of the sub-jects in the networks,20. 9%(177 / 847)linked to other regions' individuals. Conclusion The super-spreaders played an important role in the molecular transmission networks of HIV-1 CRF01 AE strains even though they were in a minority. The transmission of HIV-1 CRF01 AE strains between sub-populations and various regions was complicated and active.

This study was aimed to investigate the expression characteristics of two transcriptional factors in Ikaros family, Ikaros and Helios isoforms and their mechanism, as well as their correlation with clinical parameters, which play important roles in transcriptional regulation of hematopoiesis. Expression of Ikaros and Helios isoforms in a total of 163 patients with leukemia and correlations between Ikaros and Helios isoforms were analyzed by PCR. The results showed that different expression patters of Ikaros and Helios isoforms existed in leukemia patients, that is, Ikaros isoform (Ik-6) was predominantly expressed in acute lymphoblastic leukemia (ALL) with BCR/ABL fusion gene, while Helios isoform (He-i) was overexpressed in T-cell ALL patients. The results of cloning and sequencing demonstrated that the isoforms of Ikaros and Helios had different genetic alterations. The statistical correlation between these two isoforms not was found in this study, although interaction between Ikaros and Helios has been reported. It is concluded that although Ikaros and Helios belong to the same family with similar structure of zinc fingers, their isoforms have different expression profile, specific genetic alterations, and different clinical relevance in patients with leukemia. The connection and interaction between Ik-6 and He-i needs further research.
Adolescent ; Adult ; Aged ; Aged, 80 and over ; Child ; Female ; Gene Expression Profiling ; Humans ; Ikaros Transcription Factor ; genetics ; metabolism ; Leukemia ; genetics ; metabolism ; Male ; Middle Aged ; Protein Isoforms ; genetics ; metabolism ; Young Adult

Objective To investigate the efficiency of European System for Cardiac Operative Risk Evaluation(EuroSCORE)in predicting in-hospital mortality for the patients after percutaneous coronary intervention(PCI). Methods Retrospective analysis was conducted on the patients who had undergone PCI in our hospital since year 2005 to 2007. We used both cumulative EuroSCORE score and logistic EuroSCORE to predict the in-hospital morality and to analyze the correlation between the predicted mortality and the actual mortality. Results According to the additive EuroSCORE, we divided the patients into three groups, the additive EuroSCORE 0-2 were divided into low-risk group,3-5 were divided into mid-risk group and ≥6 into high-risk group.The actual in-hospital mortality rates were 0%, 0.47% and 6.09% respectively. The EuroSCORE model demonstrated an overall relation between the EuroSCORE ranking and the incidence of in-hospital mortality(P<0.001). Results from the multivariable logistic regression analysis showed that the EuroSCORE was an independent in-hospital mortality predictor(P<0.01). Conclusion The EuroSCORE risk model and the in-hospital mortality were significantly correlated, indicating that the model was a promising method for predicting the in-hospital mortality of PCI patients.

OBJECTIVETo analyze the complications and treatment results of intraoperative radiotherapy (IORT) for esophageal carcinoma.

METHODSSixty patients with thoracic esophageal carcinoma underwent esophagectomy through right thoractomy, 30 patients of whom received IORT of 15 - 25 Gy.

RESULTSIn patients who underwent IORT, 2 cases of pneumonitis, 1 case of anastomotic leak and 1 case of incisional wound infection were found. In patients underwent surgery only, 1 case of thoracic empyema and 1 case of anastomotic leak were found. All the complications ultimately healed. There was no operative mortality. During the follow-up of 3 years, in patients who underwent IORT, 2 of 3 died of radiation pneumonitis 24 and 26 months after IORT with one complicated with bronchoesophageal fistula. One of 3 died of multiple lung metastases. The 3-year survival rate was 88.0% (22/25) in IORT group and 76.0% (19/25) in surgery only group.

CONCLUSIONIntraoperative radiotherapy can reduce locoregional recurrence if performed to thoracic esophageal carcinoma patients without surgical contraindication or distant metastasis. Radiation pneumonitis, a common complication difficult to manage, implies a poor prognosis and, consequently, the lung and bronchus should be protected from the radiation.

Adult ; Aged ; Combined Modality Therapy ; Esophageal Neoplasms ; mortality ; therapy ; Female ; Humans ; Male ; Middle Aged ; Neoplasm Recurrence, Local ; Radiotherapy Dosage

BACKGROUNDTo investigate the effects of DNA methylation on the expression of tumor-associated genes and the cell cycle in human gastric cancer cells.

METHODSTwo gastric cancer cell lines (MKN-45 and HGC-27) were treated with DNA methyltransferase (DNMT) inhibitor, 5-aza-2'-deoxycytidine (5-aza-dC). The expressions of p16INK4A, p21WAF1, p53, p73, c-Ha-ras and c-myc genes mRNA were detected by using reverse transcription PCR (RT-PCR). DNA methylation status of p16INK4A gene promoter was assayed by bisulfite modification and sequencing. The cell cycle was analyzed by using flow cytometry (FCM).

RESULTS5-aza-dC induced the demethylation of p16INK4A gene promoter. The expression of p16INK4A mRNA was obviously up-regulated by treatment with 10 micro mol/L (MKN-45 cells) or 5 micro mol/L (HGC-27 cells) of 5-aza-dC for 24 hours. However, 5-aza-dC treatment failed to regulate the expressions of p21WAF1, p53, p73, c-Ha-ras and c-myc genes in MKN-45 and HGC-27 cells. Furthermore, 5-aza-dC induced the cell cycle arrest in G1 phase in HGC-27 cell, but not in MKN-45 cell.

CONCLUSIONSDNA methylation regulates the transcription of p16INK4A but not p21WAF1 and proto-oncogenes in human gastric cancer cell lines MKN-45 and HGC-27.

Azacitidine ; analogs & derivatives ; pharmacology ; Cell Line, Tumor ; DNA Methylation ; Enzyme Inhibitors ; pharmacology ; Flow Cytometry ; Gene Expression ; Genes, Tumor Suppressor ; physiology ; Genes, p16 ; physiology ; Humans ; Reverse Transcriptase Polymerase Chain Reaction ; Stomach Neoplasms ; genetics ; Transcription, Genetic ; Up-Regulation

Adolescent ; Adrenal Cortex Diseases ; complications ; pathology ; surgery ; Adult ; Child ; Female ; Humans ; Hydrocortisone ; blood ; urine ; Male ; Middle Aged ; Pigmentation Disorders ; complications ; pathology ; surgery

This study was purposed to investigate the mutational status of DNA methyltransferase (DNMT3a) gene and the clinical features of AML patients with DNMT3a mutations. Using PCR combined with directly sequencing, the somatic mutations of DNMT3a involving residue of amino acid 882 were detected in 77 AML patients. Furthermore, the clinical features of these patients were also studied. The results showed that the DNMT3a mutation were detected in 7 out of 59 patients with de novo AML (11.9%), which included 4 patients with DNMT3a R882C, 2 patients with DNMT3a R882H and 1 patient with DNMT3a Y874C. Morphology examination indicated that 2 patients were M(2), 1 patient was M(4) and 4 patients were M(5). Cytogenetic analysis revealed that karyotype in 5 out of 7 patients with DNMT3a mutation were normal. In total of 27 patients with normal karyotype 5 patients (22.7%) were found harboring DNMT3a mutation, while no DNMT3a mutation was found in 21 patients with abnormal karyotype. The mutation rate in patients with positive CEBPA was obviously higher than that in patients with negative CEBPA (p = 0.002). Immunophenotype analysis showed that 4 patients (4/7, 57.1%) with DNMT3a mutation expressed lymphoid antigens including CD4 or/and CD7. There were no statistical significance in age, gender, blast cells of bone marrow, white blood cell and platelet counts, hemoglobin level, ratio of CR, mutations of FLT3-ITD, NPM1 and c-kit between patients with DNMT3a mutation and patients with wild DNMT3a (p > 0.05). It is concluded that the DNMT3a mutations are more prevalent in AML patients with normal karyotype accompanying with positive NPM1 and/or CEBPA mutation, the role of DNMT3a mutation in AML prognosis needs to be further studied.
Adolescent ; Adult ; Aged ; Aged, 80 and over ; CCAAT-Enhancer-Binding Proteins ; genetics ; Child ; DNA (Cytosine-5-)-Methyltransferases ; genetics ; Female ; Humans ; Leukemia, Myeloid, Acute ; genetics ; Male ; Middle Aged ; Mutation ; Nuclear Proteins ; genetics ; Young Adult