Objective To investigate the manifestations of cardiac involvement in the patients with mucopolysacharidosis Ⅰ (MPS Ⅰ).Methods The clinical data of 10 MPS Ⅰ patients were collected.Electrocardiography (ECG) and echocardiography (Echo) were performed in all patients and then analyzed.Results Among the ten patients,seven were men.The onset age of MPS was (0.5 ～ 8.0) years old and the age of diagnosis was (1.8 ～ 20.0) years old.Two patients had grade 2 precordial systolic murmur.ECG was abnormal in three patients with right ventricular hypertrophy in two and right axis deviation in another one.Echo showed valvular thickening and insufficiency in nine patients,enlarged left atrium and ventricle in one patient,hapulmonary hypertension and right ventricular hypertrophy in two patients and abnormal left ventricular configuration in five patients.Conclusions Cardiac involvement is common in MPS Ⅰ patients and may present as valvular thickening with regurgitation,abnormal left ventricular configuration and pulmonary hypertension.The cardiac involvement progresses with age.ECG and Echo should be done regularly during follow-up of MPS Ⅰ patients.

Objective This study aims to evaluate the impact of early surgery on long-term outcome of patients with left-sided native valve infective endocarditis (IE).Methods Clinical data were retrospectively reviewed in 239 consecutive patients with left-sided native valve IE from 2002 to 2012 in Peking Union Medical College Hospital (PUMCH).Propensity score was used to match patients in the early operation and conventional treatment groups.Results Early surgery was performed in 70 (29.3％)patients and the conventional treatment strategy was applied in 169 (70.7％) patients.The median followup period was 2 years.IE-related mortality was lower in the early operation group than in the conventional treatment group (10.0％ vs 23.1％,P =0.02).For 58 propensity score-matched pairs,the cumulative survival free from IE related death was significantly higher in the early operation group than in the conventional treatment group (P =0.027).Regression analysis of the matched cohorts revealed that early surgery was independently associated with decreased IE-related mortality (HR 0.286 ; 95％ CI 0.092-0.893 ;P =0.031).While either cardiac function with NYHA Class Ⅲ-Ⅳ (HR 4.044; 95％ CI 1.318-12.407;P =0.015) or uncontrolled infection (HR 52.064; 95％ CI 10.996-247.194; P ＜ 0.001) was associated with poor prognosis of increased mortality.Conclusions Early surgery improved long-term outcome in patients with left-sided native valve IE compared with conventional therapy.Risk factors related to increased mortality included heart failure and uncontrollable infection.

OBJECTIVE:To prepare anti-microbicide contraceptive gel and establish its quality control method. METHODS: The gel was prepared with octoxynol and policresulen as main ingredients and HPMC as matrix. The content of octoxynol in the gel was determined by HPLC,and the content of policresulen in the gel was determined by titration method. RESULTS:Prepared gel was well-proportioned and of good viscidity and its identification and test were up to the standard. The linear rang of octoxynol was 250～1 500 mg?L-1(r=0.999 6)with an average recovery of 100.84%(RSD=0.74%,n=9). The average labeled amount of policresulen was 106.64%(n=3). CONCLUSION:This preparation is feasible and stable in quality,and the quality control method is simple and accurate.

To establish a method to detect cardiac troponin I by using time-resolved fluoroimmunoassay ( TRFIA) and apply to the clinic.Methods:The assay were measured by TRIFA and double antibody sandwich method .Standard protocols were evaluated with the standard curve , the limit of detection , stability, precision and cross reaction .Healthy reference populations and clinical serum specimens were measured to established the reference interval and evaluated the perspective of the clinical application . Results:The standard curve was Y=7485 .878+1400.924 X with a correlation coefficient of 0.999.The limit of detection was 0.052 ng/ml.The intra-and inter-assay coefficients of variation ( CV) were all <10%.Reference values was <0.14 μg/L.The AUC of ROC curve was 0.971 while the sensitivity was 96.45%,the specificity was 91.43% and the accuracy was 95.69%, with 98.45% of positive predictive value and 82.05%of negative predictive value.The correlation coefficient was 0.993 between our proposed method and the commercially available CLIA kits.There was no significant difference in statistics compared with ECG , CK-MB and cTnT ( P>0.05 ).There was significant difference in statistics compared before and after treatment with AMI ( P<0.001 ) .Conclusion: The TRFIA method for detecting cTnI achieves clinical application standards and may be used for the diagnosis and serosurveillance of acute myocardial infarction patients.

Objective To detect the expression of stem-cell related factors Nanog and CD44 in spheroid body-form?ing cells of gastric cancer cell line MKN-45. Methods The gastric cancer cell line MKN-45 was used to culture spheroid bodies in non-adherent condition in a serum-free medium supplemented with epidermal growth factor (EGF) and basic fibro?blast growth factor (bFGF). Using Western blot analysis, immunofluorescence staining and quantitative real time polymerase chain reaction(qRT-PCR), the expression levels of stem cell-related genes Nanog and CD44 were studied. Results In this study, we observed that MKN45 cells formed spheroid bodies in non-adherent condition in a serum-free medium, and the levels of Nanog and CD44 mRNA expression in spheroid body-forming cells were 2.34 ± 0.22 and 1.18 ± 0.04,respectively, which were higher than those in parental cells (1.00±0.00 and 1.00±0.05). The levels of Nanog and CD44 protein expression in spheroid body-forming cells were 0.18±0.02 and 0.24±0.04, respectively, which were significantly higher than those in pa?rental cells (0.07±0.02 and 0.18±0.01, P<0.05). Nanog protein was positively stained within the perinuclear and cytoplasm of the spheroid body-forming cells, and CD44 was positively stained mainly in the membrane. Dual staining of Nanog/CD44 indicated that the embryonal protein Nanog was co-localized with CD44 in the spheroid body-forming cells. Conclusion Spheroid body-forming cells developed from human gastric cancer cell line MKN-45 in serum-free medium supplemented with EGF and bFGF show characteristics of cancer stem cell (CSC). The cells co-expressed of CD44 and Nanog maybe a phe?notype of gastric CSCs.

Objective To explore the expressions of Toll-like receptors (TLRs) 2 and 4 in mouse skin during early immune responses against Sporothrix.Methods A total of 60 BALB/c mice were randomly and equally divided into an experimental group and a control group to be intracutaneously injected with Sporothrix conidium suspensions at a concentration of 1 × 106 cfu/ml and sodium chloride physiological solution respectively.Five mice were sacrificed before the injection,and at 6,12,24,48,and 96 hours after the injection in each group,blood samples were obtained from the mice before sacrifice,and skin tissue specimens were resected from the area around the injection sites after sacrifice.Realtime fluorescence-based quantitative PCR was performed to quantify the mRNA expressions of TLR2 and TLR4,and immunohistochemical staining to observe the protein expressions of TLR2 and TLR4 in mouse skin specimens.Enzymelinked immunosorbent assay (ELISA) was conducted to determine the levels of interleukin 12 (IL-12) and tumor necrosis factor α (TNFα) in serum samples from the mice.Results After injection of Sporothrix conidium suspensions,the mRNA expression level of TLR2 gradually increased and peaked at 24 hours,which was 18.8 times that in the control group at 6 hours and 34 times at 24 hours.In addition,the mRNA expression level of TLR4 in the experiment group reached a peak,and was 56.7 times that in the control group at 6 hours after injection,then gradually decreased and reached the nadir at 96 hours.As immunohistochemical staining revealed,TLR2 and TLR4 were apparently expressed in both keratinocytes and macrophages in skin specimens from the experimental group,but not obviously in those from the control group.No significant differences were observed between the experimental group and control group in serum levels of IL-12 or TNF-α at any of the sampling time points.Conclusion TLR2 and TLR4 may play a favoring role in immunological defense by participating in the recognition of Sporothrix by keratinocytes and macrophages in mouse skin.

Objective To investigate the expression pattern and significance of two importantoocyte-secreted factors:growth differentiation factor 9(GDF9)and bone morphogenetic protein 15(BMP15)during oocyte maturation in women with polycystic ovary syndrome(PCOS)and infertile women due to husband factors.Methods Total of 25 oocytes[9 at germinal vesicle GV stage,9 at M Ⅰ stage and 7 at M Ⅱ stage]were obtained from 12 patients with PCOS and 82 oocytes(29 at GV stage,26 at M Ⅰ stage and 27 at M Ⅱ stage)were from 56 controls.The nested quantitative real time(RT)PCR was uscd to detect the abundance of GDF9 and BMP15 mRNA in each oocyte.Results(1)The expression level of GDF9 mRNA at the GV stage,M Ⅰ stage and M Ⅱ stage in PCOS group were 44.8(4.2-529.0),27.6(9.8-172.7)and 49.0(0.2-65.9)respectively,the expression in were 149.9(55.4-387.9),29.9(2.5-205.8)and 657.8(149.4-1376.2)in control group,respectively.The expression of GDF9 mRNA at M Ⅱ stage was significantly lower in PCOS group than in controls(P ＜ 0.01),however,the differences didn't reach statistical significant at GV or M Ⅰ stage between the two groups(P ＞ 0.05).The expression of GDF9 mRNA displayed some changes at different maturation stage in controls(P ＜ 0.05,P ＜ 0.01),however,the expression didn't demonstrate any dynamic changes in PCOS group(P ＞ 0.05).(2)The expression level BMP15 mRNA at the GV stage,M Ⅰ stage and M Ⅱ stage in PCOS group were 0.1(0.1-22.0),3.2(0.6-55.0)and 6.4(3.2-8.5),respectively,the expression were 41.6(6.5-96.1),4.0(2.0-10.4)and 49.7(2.3-139.5)in control group,respectively.The expression of BMP15 mRNA at GV stage was significantly lower in PCOS group than in controls(P ＜ 0.01),however,the differences were not significant at M Ⅰ or M Ⅱ stage between the two groups(P ＞0.05).The expression of BMP15 mRNA also displayed some changes at different maturation stage in controls(P ＜ 0.05),however,the level didn't demonstrate any dynamic changes in PCOS group(P ＞ 0.05).Conclusion It was suggested that the low expression of oocyte secreted factors in mature oocytes from PCOS patients might be associated with impaired oocyte quality and developmental competence in PCOS.

Objective To compare the safety and clinical efficacy between paclitaxel liposomes and paclitaxel in patients with advanced esophageal cancer .Methods A total of 90 patients with advanced esophageal cancer were enrolled into this study and were randomly divided into paclitaxel liposomes treatment group(treatment group) and paclitaxel treatment group(control group) . The patients of each group were treated with paclitaxel liposomes or paclitaxel 80 mg/m2 intravenously on day 1 and 8 ,and nedapla‐tin 75 mg/m2 intravenously on day 1 ,respectively .After two cycles of chemotherapy ,three weeks per period ,we evaluated the short term efficacy and adverse reactions according to the WHO standard .Results The short term efficacy between control group and treatment group showed no significance statistically (P>0 .05) .However ,the incidence of allergic reactions in the treatment group was significantly lower than control group (P<0 .05) .Conclusion Paclitaxel liposomal or paclitaxel combine with nedaplatin was effective equivalently in the treatment of patients with advanced esophageal cancer .But the allergic reactions of paclitaxel liposomal were lower than paclitaxel .It is worthy of clinical promotion .

Objective To survey on the prevalence of schistosomiasis among floating population in Zhejiang province. Methods A survey on prevalence of schistosomiasis among floating population was conducted from September to November 2008, and the stratified cluster sampling method was adopted in the survey. Totally 129 villages of 19 counties or districts were selected as survey sites, and 100 samples of migrants aged 6 to 65 from schistosomiasis-endemic areas were taken in each selected village. All selected individuals were surveyed by questionnaire and underwent serum indirect hemagglutination (IHA) test. For individuals with positive serum IHA testing the fecal examination was carried out to detect the eggs by nylon sedimentation method. SPSS 13.0 software was used for data processing. Results The number of migrants in survey sites was 3 357 420, among whom 303 219 were from schistosomiasis-endemic areas (9.03%).The positive rate in serum IHA test was 2.06% (286/13 898), 276 IHA-positives individuals received fecal examination, and 7 cases were positive (2.52%). Based on above data it was estimated that there would be potentially about 33 500 serum IHA-positive cases and 845 egg-positive cases among floating population in Zhejiang province. Conclusion The risk of schistosomiasis transmission still exists in Zhejiang province due to the infected migrants from endemic areas, and a surveillance system and quick response are required for prevention of re-emergence of the disease.

Objective To test the RET-proto-oncogene in a multiple endocrine neoplasia type 2( MEN2) family for confirming the diagnosis and classification , guiding treatment and prevention , and improving the prog-nosis.Methods There were 2 patients of MEN2 with clinical diagnosis and 1 asymptomatic first-degree relative in the pedigree .PCR and direct gene sequencing of PCR produces were used to scan the entire 21 exons of RET-proto-oncogene in the 3 members of the pedigree and 3 normal controls .Results The 2 patients and 1 asympto-matic first-degree relative in the pedigree all had a mutation of the codon 634 in exon 11.It was a heterozygous missense mutation C634R(TGC → CGC).The 3 normal controls showed no abnormalities .Conclusion The gene test of RET proto-oncogene helps to confirm the diagnosis of the pedigree as MEN 2, which can guide the treatment and help identify one asymptomatic mutation carrier .