Noonan syndrome(NS) is a congenital genetic disease characterized by distinctive facial features,short stature,chest deformity,congenital heart disease,and other comorbidities.This article reviewed the research history of NS,the clinical manifestations and their relationship with genotype,the diagnosis and treatment.

AIM: To study apoptosis and related genes expressions of cardiomyocytes in sinoaortic-denervated(SAD) rats. METHODS: SAD or sham-operation (Sham) was performed in male SD rats at the age of 10 weeks. After 16 weeks, apoptotic cells were stained in situ by terminal dexynucleotidyl-transferase mediated-dUTP nick end labeling (TUNEL). All stained results were analysised using computer image analysis techniques. Protein products and mRNA of Bcl-2, Bax, Fas and Fas-L were assessed by quantitative immunohistochemistry and RT-PCR. RESULTS: The numbers of apoptotic cardiomyocytes were significantly increased in SAD rats, the expressions of Bcl-2 were significantly decreased, whereas Bax,Fas and Fas-L were significantly increased in SAD rats. THZ]CONCLUSIONCONCLUSION: Apoptosis and dysregulation of gene expressions may be involved in the cardiomyocytes remodeling in SAD rats.

Objective To evaluate and explore the Patients with acute cerebral infarction P-selectin and C-reactive protein (CRP) clinical significance.Methods According to the degree of neurological deficit scores were divided into light group (38 cases),medium group (35 cases) and severe group (25 cases),30healthy subjects were selected as the control group,measured at different time levels of P-selectin and CRP.Results Light group,medium group,severe group admitted 24 h P-selectin levels were higher,the difference was statistically significant (P ＜ 0.05).Light group,medium group,severe group admission decreased after 3 d P-selectin levels were highest; mild group admitted 24 h,7 d P-selectin levels and medium-sized group and severe group differences were statistically significant (P ＜ 0.05).Light group,medium group,severe group admission 3 d P-selectin levels were statistically significant differences (P ＜ 0.05) among the three groups.Light group,medium group admitted 14 d P-selectin levels and heavy group differences were statistically significant (P ＜ 0.05).Light group,medium group,severe group admitted 24 h CRP levels were higher,the difference was statistically significant (P ＜ 0.05).Light group,medium group,severe group admitted 24 h,3 d,7 d,14 d CRP levels decreased,comparing the differences among the three groups were statistically significant (P ＜ 0.05) at the same time point.Linear correlation analysis showed that P-selectin hormone levels and CRP levels were positively correlated (r =0.493,P ＜ 0.05).Conclusions Serum P-selectin and C reactive protein may be involved in the pathological process of ACI.The changes of serum P-selectin and C reactive protein levels were contributed to judge severity of pathological changes,focus range and prognosis assessment.It is an important biochemical observational indicator,and it's worthy recommending in clinic.

Aim To investigate the up-regulation of miR-22 through Wnt pathway inhibits the proliferation,migration and invasion in human gastric MGC803 cells induced by diallyl disulfide(DADS).Methods The effects of proliferation,migration,and invasion of gastric cancer cells were evaluated by MTT,wound-healing and invasion assays.Online prediction software was applied to search the target gene of miR-22.Luciferase report gene assay was used to assess the target genes Wnt-1 of miR-22.The expressions of Wnt-1,β-catenin and TCF-4 were tested by qRT-PCR and Western blot,respectively.Results MTT showed that DADS and miR-22 notably decreased the proliferation compared with control group(P<0.05).Wound-healing assay showed that DADS and miR-22 could significantly inhibit the migration of MGC803 cells compared with the control group, especially in miR-22+DADS(P<0.05). Invasion assay showed that DADS and miR-22 could markedly inhibit the invasion of MGC803 cells compared with the control group, especially in miR-22+DADS(P<0.05). Online prediction software to search the target gene exhibited that Wnt-1 may be a target gene of miR-22. Luciferase report gene assay disclosed that Wnt-1 was identified as a direct target of miR-22. Qrt-PCR showed that the expression of Wnt-1 Mrna was respectively down-regulated by DADS and miR-22 compared withcontrol group, especially in miR-22+DADS(P<0.05). Western blot exhibited that DADS and miR-22 obviously suppressed the expressions of Wnt-1, β-catenin and TCF-4 proteins, especially in miR-22+DADS(P<0.05).Conclusion Up-regulation of miR-22 through Wnt pathway can remarkably suppress the proliferation, migration and invasion in MGC803 cells by DADS.

Adult ; Empyema, Pleural ; complications ; etiology ; Humans ; Male ; Neck Injuries ; complications ; Tracheoesophageal Fistula ; complications ; etiology

Objective To investigate the possibility and accuracy of predicting malignant middle cerebral artery infarction (mMCAI) with bedside electroencephalography (EEG). Methods Thirty-five patients with massive hemispheric infarction (MHI) underwent bedside EEG monitoring within 48 h of onset. The EEG indicators were interpreted blindly, and the clinical, laboratory and imaging parameters were analyzed. The patients were divided into mMCAI group and non-mMCAI group according to whether they had occurred mMCAI or not within 7 days of onset. The differences of EEG indicators, clinical, laboratory and imaging parameters between the 2 groups were compared. When the parameters of significant difference and statistical significance appeared the odds ratio (OR) of occurring mMCAI were analyzed, and their accuracy of predicting mMCAI was calculated. Results Of the 35 patients with MHI, 20 were in the mMCAI group and 15 were in the non-mMCAI group. There were significant differences in the EEG indicators (infarction on the contralateral side, including disintegration of occipital α rhythm, generalized slow-wave, dominant frequency wave low amplitude, regional attenuation without delta [RAWOD]pattern, and absence of EEG reactivity), clinical parameters (nausea accompanied with vomiting), and imaging parameters (the infracted area more than the entire MCA territory, and midline shifting 3 to 5 mm at the level of septum pellucidum) between the 2 groups (P < 0. 05). Of those, the risk of mMCAI was the highest in patients with disintegration of occipital a rhythm on the contralateral side of infarction (P = 22. 67, 95% CI 3. 89-132. 10). The sensitivity of predicting mMCAI was 85. 0%, the specificity was 80.0%, the positive predictive value was 85.0%, and the negative predictive value was 80. 0%, which were superior to other EEG indicators and clinical or imaging parameters. Conclusions Bedside EEG indicators can early predict mMCAI, moreover, the predictive accuracy is superior to the clinical and imaging parameters.

Objective To evaluate feasibility and reliability of using Simplified Acute Physiology Score Ⅱ (SAPS Ⅱ)in predicting mortality in critically ill patients with neurological diseases.Methods All 653 patients hospitalized in neurological intensive care unit (N-ICU) from Jan 2005 to Dec 2007 were retrospectively studied.SAPSⅡ scores were scaled upon admission at 24,48 and 72 h,and possibility of hospital mortality (PHM) was calculated based on SAPS Ⅱ score.Neurological diseases diagnosis made initially at time of hospitalization was classified into five categories:intracranial hemorrhage,cerebral infarction,neurologic infection,neuromuscular diseases and other neurologic diseases.At each of four time points,the SAPSⅡscores were compared between the survival group and death group,and the relationship of SAPS Ⅱ score and prognosis were analyzed.The calibration of the SAPS Ⅱ were accessed with the Hosmer-Lemeshow goodness-of-fit chi-squared statistic and the discrimination with area under the receiver operating characteristic curve (AUROC).Multivariate logistic regression was used to analyze the relationship between disease category and prognosis.Results SAPS Ⅱ scores in the death group (46.53±12.22,47.28±13.84,48.58±14.18 and 49.06±14.61)at each time point were significantly higher than those in the survival group (34.70±11.78,30.28±12.24,29.79±12.36 and 29.69±12.96;t=11.12,14.02,14.43 and 13.49 at 0,24,48 and 72 h,respectively,P＜0.01).Furthermore,univariate logistic regression analyses demonstrated that SAPS Ⅱ score was correlated with prognosis (OR=1.080,1.100,1.109,1.100 at 0,24,48 and 72 h,respectively,P＜0.01).The scores at 48 and 72 h were more accurate in predicting mortality.SAPS Ⅱ had good calibration at each time points (x2=5.305,7.557,6.369 and 8.540,P＞0.05),however,the consistency of expected mortality with observed mortality was satisfactory only at 48 and 72 h(82.6%,83.4%),so was the discrimination ( AUROC=0.825,0.847 respectively).There was no correlation between disease categories and outcome.Conclusion SAPS Ⅱ scoring,best evaluated at 48 and 72 h after hospitalization,can be used as a reliable predictor of probability of mortality in patients hospitalized in N-ICU and prediction can be applied in these patients with all different neurology diseases.

There are many subjects related to anesthesiology and the band between anesthesiology and clinical practicevery is close. So students can not be proficient in anesthesiology by traditional teaching methods. The use of three-dimensional case teaching method in anaesthesiology teaching is a good attemptment. three-dimensional case teaching method integrate case method, multimedia technology and clinical case. It can help medical students analyze and solve clinical problems, improvetheir academic performance and enhance their clinical basic skills.

Objective To investigate the accuracy of serum neuron-specific enolase (NSE)predicting malignant middle cerebral artery infarction(mMCAI).Methods A total of 40 patients with acute massive cerebral infarction within 24 hours after symptom onset were recruited.Blood samples were collected at 24,36 and 48 hours after symptom onset.Serum NSE concentration was determined by automatic electrochemiluminescence analyzer.mMCAI was defined as hernia signs in clinical practice,and CT/ MRI showed mass effect.The receiver operating characteristic curve was used to analyze the accuracy of serum NSE concentration in predicting mMCAI at 3 time points.Results Sixteen patients(40%)developed mMCAI.The serum NSE concentration for predicting the accuracy of mMCAI was poor at 24 hours after symptom onset;the serum NSE concentration for predicting the specificity of mMCAI was high (96%)at 36 hours after symptom onset,but the sensitivity was lower(69%);the serum NSE concentration for predicting the specificity(92%)and sensitivity(88%)of mMCAI were high at 48 hours.Conclusions The serum NSE conoentration and its dynamic changes may predict the occurrence of mMCAI,and the predicting time points are appropriate from 36-48 hours after symptom onset.

AIM:To investigate whether the cigarette smoke extract (CSE) causes senescence of C2C12 myo-blasts and the relationship between senescence and histone deacetylase 2(HDAC2).METHODS: Murine C2C12 cells were induced to differentiate into myoblasts.The HDAC2 activator and inhibitor were used to investigate the effects of CSE in the myoblasts on cell senescence and the expression of HDAC2.The expression of HDAC2 at mRNA and protein levels was determined by real-time PCR and Western blot, respectively, and the positive cell rate of β-galactosidase staining for cell senescence was also detected.RESULTS:The optimal concentration of CSE was 60 mL/L and the intervention time was 24 h.After the intervention of CSE, the positive cell rate ofβ-galactosidase staining was increased, accompanied with the reduction of HDAC2 expression at mRNA and protein levels.The expression of HDAC2 at mRNA and protein levels was increased by 4, 5, 6, 7-tetrabromobenzotriazole (TBB), accompanied with the reduction of positive cell rate ofβ-galacto-sidase staining.Furthermore, when HDAC2 expression at mRNA and protein levels was reduced by HDAC2 inhibitor valp-roic acid, the positive cell rate of β-galactosidase staining was increased.CONCLUSION: CSE promotes the senescence by reducing the expression of HDAC2 in C2C12 myoblasts.