ObjectiveTo investigate the clinical,myopathological and molecular changes in two Chinese families with oculopharyngodistal myopathy ( OPDM).MethodsWe performed muscle biopsy and histopathologic study on the probands of two families,and further examined molecular genetic testing on PABPN1 and GNE gene. Results Family 1 included 3 affected brothers in the same generation and family 2 involved 4 patients in 2 generations. Dysarthria rather than external ophthalmoplegia was the prominent oculopharyngeal symptoms for Chinese patients. No intranuclear inclusions were observed in ultrastructural examination.The number of GCG repeats in the PABPN1 gene was within normal range and no mutations were identified in the GNE gene.ConclusionsFamily 1 is the first publication on autosomal recessive OPDM in China.The age of onset of two families was comparable with Japanese patients and the pattern of muscle involvement was different. OPDM is a distinct phenotypical,histological,and genetic entity.

Objective To investigate the effect and mechanism of STAT3 gene silencing on the growth of xenografts in human pancreatic cancer SW1990 cells in nude mice.Methods The expression vector inserted with shRNA targeting at STAT3 gene was constructed and was stably transfected into SW1990 cells (SW1990-RNAi group).SW1990 cells transfected with negative control shRNA expression vector (SW1990-Con group) and parent SW1990 (SW1990 group) were used as controls.STAT3,VEGF,MMP-2 protein expressions in these groups were determined by using Western blot.The subcutaneous xenografts models were established in nude mice,and the growth of xenografts was observed,CD34 expressions were determined by immunohistochemistry and MVD was measured.Results The expression of STAT3 protein was 84.69 ± 9.31,82.00 ± 7.76,7.93 ± 1.24,repectively,in SW1990 group,SW1990-Con group,SW1990-RNAi group,and the expression of VEGF protein was 82.94 ± 8.97,80.86 ± 10.28,39.04 ± 6.23,respectively,and the expression of MMP-2 protein was 40.88 ± 5.09,38.26 ± 5.71,12.54 ± 2.15,respectively.The expression in SW1990-RNAi group was significantly lower than those in other 2 groups (P ＜ 0.05),while the expression of all three proteins between SW1990-Con group and SW1990 group was not significantly different.The weight of the xenografts in SW1990 group,SW1990-Con group,SW1990-RNAi group was (2.2 ± 0.4),(2.2 ± 0.3),(0.5 ± 0.3) g,respectively ; the MVD of the xenografts was (20.35 ± 2.41),(18.79 ± 1.94),(9.62 ± 1.06) per high power field,respectively,and the number in SW1990-RNAi group was significantly lower than those in other 2 groups (P ＜ 0.05 or P ＜ 0.01),while the difference between SW1990-Con group and SW1990 group were not significant.Conclusions Inhibition of STAT3 gene expression can significantly slow the growth of SW1990 xenografts in nude mice,and the mechanism may be related with down-regulation of VEGF and MMP-2 expression and inhibition of the angiogenesis of pancreatic cancer.

Objective To evaluate the effect of sufentanil postconditioning on myocardial ischemia-reperfusion injury in rats, and to investigate the possible mechanism. Methods Thirty male Sprague-Dawley ratswere randomly divided into 5 groups (n = 6 each): sham group (group S), I/R group (group I/R), low dose of sufentanil postconditioning group (group SL), high dose of sufentanil postconditioning group (group SH) andsufentanil postconditioning plus wortmannin group (group WM). Different drugs were injected before reperfusion: normal saline (NS) and sufentanil 1 μg/kg in group SL, NS and sufentanil 3 μg /kg in group SH, wortmannin 15 μg/kg andsufentanil 1 μg/kgin group WM, and NS in group S and I/R. At the end of reperfusion, artery blood was collected for assessment of plasma cTnI concentration; And the heart was harvested for determination of infarct size (IS) and area at risk (AAR). Results Compared with group S, cTnI concentration was increasedin the rest groups (P< 0.01); Compared with group I/R, cTnI concentration was decreased in group SL, SH and WM, while IS/AAR reduced in group SL and SH (P < 0.01); Compared with group SL, cTnI concentration and IS/AAR were decreased in group SH while increased in group WM (P <0.01). Conclusion Sufentanil postconditioning could attenuate myocardial ischemia-reperfusion injury in rats, and the mechanism involved PI3K related pathway.

Objective To explore the role and mechanism of keratin 19 (KRT19) in breast cancer.Methods Quantitative real-time polymerase chain reaction (qRT-PCR) assay was used to determine KRT19 levles in 35 cases of breast cancer tissues and normal tissues.The correlation between KRT19 levels and clinical property of breast cancer was analyzed.Meanwhile,the expression levels of KRT19 in several breast cancer cells and mammary epithelial cell Michigan cancer foundation (MCF)-10A were evaluated by qRT-PCR assay.Over-expressed and knocked-down KRT19 in breast cancer ceils,3-(4,5-dimenthylthiazol-2-yl)-5-(3-carboxymethoxyphenyl)-2-(4-sulfophenyl)-2H-tetrazolium (MTS) assay was performed to detect the proliferation and chemosensitivity of these cells.The ability of forming colon of these breast cancer cells with treated KRT19 was studied via colony-forming unit assays.Western blot assay was performed to determine expression levels of ceil cycler related proteins.Results KRT19 was upregulated in breast cancer tissues comparing to normal tissues.KRT19 was positively related to tumor node metastasis (TNM) stage and distant metastasis of breast cancer.Similarly,KRT19 was highly expressed in breast cancer cells compared to mammary epithelial cell MCF-10A.The proliferation and colony-forming ability was significantly enhanced in MCF-7 cell with o,verexpressed KRT19.MTS assay showed that chemosensitivity of MCF-7 cells with overexpression of KRT19 was much more remarkably reduced than the control group.However,knocking down KRT19 in breast cancer cells MDA-MB-231 got the opposite results.Western blot assay suggested that KRT19 could obviously upregulated cyclin-dependent kinase 1 (CDK1) but not p27 expression levels.Conclusions KRT19 was upregulated in breast cancer tissues and was positively related to TNM stage and distant metastasis of breast cancer.KRT19 can significantly enhance proliferation and chemoresistance of breast cancer cells via upregulating CDK1.

Objective To investigate the impact of renal function and insulin resistance on serum total homocysteine(tHcy) levels in aged diabetic patients with nephropathy. Methods Serum tHcy, blood glucose, lipids, renal function, serum insulin were measured for 98 elderly patients with type 2 diabetes and 36 normal control subjects. Results (1) Serum tHcy levels were significantly higher in diabetic group than in control group [(17.2?7.6) ?mol/L vs (9.4?3.5)?mol/L,P

This paper introduces the working principle of the blood circle treatment system outside the body. A monitoring instrument of plasma leakage suited for the blood circle treatment system outside the body is developed based on the absorption spectrum experiments of plasma leakage. Photoelectric detection technology and virtual instrumentation are utilized in the development. A series of detecting experiments of waste solution containing plasma with continuously changing concentration show the monitoring system possesses a relatively high sensitivity. Moreover, the experiments of continual detection with plasma concentration at one certain point indicate the monitoring system has a quite good stability. The monitoring instrument is adapted to dynamically detecting the plasma leakage when the blood circle treatment system outside the body is working.

Objective To analyse the causes and clinical characteristics of re-exploration after peripartum hysterectomy due to postpartum hemorrhage. Methods Clinical data was analysed retrospectively including 88 critically ill obstetric patients who underwent peripartum hysterectomy due to postpartum hemorrhage in the Obstetric Critical Care Center of Guangzhou from January 1999 to July 2009, which were divided into re-explored group (n= 14) and non-re-explored group (n=74)depending on whether the patient underwent re-exploration after peripartum hysterectomy. The main demographic data and clinical details were compared between the two groups, including mode of delivery, indication and type of hysterectomy, interval from hysterectomy to re-exploration, surgical intervention, complications, blood loss, blood transfusion,Glasgow Coma Score(GCS), the need for mechanical ventilation, intensive care unit stay and hospital stay. Results Fourteen out of the 88 (15.91%) patients underwent re-exploration due to internal bleeding after peripartum hysterectomy.Removal of cervical stump was performed in five patients and stump hemostasis in eight cases.Significant difference was found between the re-exploration and non-re-explored group on thepercentage of patients complicated with disseminated intravascular coagulation(92.9% vs 43.2%,x2=11.598,P=0.001) and amniotic fluid embolism (28.6% vs 2.7%, x2 =8.663, P=0.003).0.000], blood transfusion [(8163.6± 3903.1 ) ml vs (2958.8± 2323.0) ml, P = 0.000], intensive care unit admission rate (100.0% vs 41.9%, x2 = 15.909, P= 0.000), the need for mechanical ventilation (100.0% vs 24.3%,P=0.000), the number of patients with GCS≤8 score (71.4% vs 25.7% ,x2 = 9.179, P = 0.002 ), the number of multiple organ dysfunction syndrome ( 71.4% vs 14.9%, x2 = 17.735, P = 0.000), intensive care unit stay [ ( 11.4 ± 10.0 ) d vs ( 1.3 ± 2.3 ) d, P =0.000] and hospital stay[(24.0±13.1) d vs (12.7±7.0) d, P=0.000]. Allof the 14 cases were clinical recovered before discharge. Conclusions The rate of re-exploration after peripartum hysterectomy is not low, and internal bleeding is the most common causes. The re-exploration after peripartum hysterectomy might be associated with coagulopathy and the mode of hysterectomy, and patients may experience more severe complications.

Objective To observe the seroreversion of serological tests for syphilis in neonates born to mothers with syphilis and to evaluate the influence of antisyphilitic treatment on the time of seroreversion in uninfected neonates.Methods A total of 115 neonates born to mothers with syphilis were recruited in this study,and underwent toluidine red unheated serum test (TRUST),Treponema pallidum particle assay (TPPA)and 19S IgM-TPPA at birth.Follow up was scheduled at 1,3,6,9,12,18 and 24 months of age,and cancelled as soon as both TRUST and TPPA became negative during the 24 months.Both TRUST and TPPA were carried out at each follow-up visit.Kaplan-Meiter survival analysis and Wilcoxon rank-sum test were conducted to assess these data by using SPSS 13.0 software.Results At birth,TPPA was positive in all (100％) of the neonates,TRUST in 97 neonates (84.3％) with the titer ranging from 1 ∶ 1 to 1 ∶ 256,19S IgM-TPPA in 3 neonates who were diagnosed with congenital syphilis (CS).CS was ruled out in the other 112 neonates with negative 19S IgM-TPPA after follow up.Of the 3 neonates with CS,all became negative for TRUST within 12-18 months after birth,2 remained positive for TPPA at 24 months of age,1 remained positive for TPPA at 20 months of age,and all were still followed at the time of this writing.The cumulative seroreversion rate was 82.2％,98.9％ and 100％for TRUST at 0-3,4-6 and 7-9 months of age,respectively in 90 uninfected neonates,3.1％,30.2％,85.4％ and 100％ for TPPA at 0-3,4-6,7-9 and 10-12 months of age,respectively in 96 uninfected neonates.Of 16 neonates who missed some follow-up visits,all turned negative for TRUST and TPPA within 15 months after birth.As far as the TP-seropositive uninfected neonates were concerned,no statistical differences were observed in the seroreversion time of TRUST or TPPA between neonates receiving antisyphilitic treatment and those without antisyphilitic treatment (x2 =0.54,2.41,respectively,both P ＞ 0.05).The seroreversion of TRUST occurred earlier than that of TPPA (Z =10.45,P ＜ 0.01).Conclusions Most uninfected neonates born to syphilitic mothers turn negative for TRUST within 6 months after birth,and for TPPA within 12 months.And the seroreversion of TRUST usually occurs earlier than that of TPPA.Antisyphilitic treatment shows no significant influence on the seroreversion time of TRUST or TPPA in these seropositive uninfected infants.

Objective To investigate role of N-(30,40-dimethoxycinnamonyl) anthranilic acid (3,4-DAA) in mediating the negative immune regulation by indoleamine 2,3-dioxygenase in mice skin transplantation.Method The mice skin transplant model was established.T lymphocytes were isolated from mice splenocytes,and serum was collected.ELISA was applied to detect the IL-2 and IFN-γ concentrations in culture supernates of mice spleen lymphocytes and serum.Flow cytometry was applied to examine the phenotypes of T lymphocytes and RT-PCR to analyze the IDO mRNA transcription.Results T lymphocyte proliferation was significantly decreased by 3,4-DAA and the concentrations of IL-2 and IFN-γ were apparently decreased in 3,4-DAA group as compared with those in control group.The percentages of CD3 +,CD4 + and CD8 + T lymphocytes were lower,and the percentage of CD4 + CD25 + T lymphocytes was higher in 3,4-DAA disposal group than in control group,but there was no significant difference.IDO mRNA expression showed obvious increase in 3,4-DAA group as compared with control group.Conclusion 3,4-DAA can up-regulate the IDO expression to induce the increasing metabolism of tryptophan,subsequently inhibiting T cell proliferation and showing specific property of negative immune regulation.