Objective A retrospective study was undertaken to evaluate the outcomes of the newborns with pulmonary atresia with intact ventricular septum(PAIVS),in order to delineate strategies for the optimal management of PAIVS. Methods From July 2003 to July 2008,17 neonates with PAIVS underwent surgical treatment.The mean age at operation was(1 5.6±8.6)d and the mean weight was(3.54±0.44)kg.Initial surgical treatment included:Blalock-Taussig shunt (BT shunt)in 2 cases,closed pulmonary valvotomy and BT shunt in 1 case,right ventricular outlet tract reconstruction(RVOTR)in 2 cases,RVOTR and BT shunt in 11 cases. Results The mean duration of mechanical ventilation and intensive care unit stay were(35.5±35.1)h and(8.7±5.9)d,respectively.Two early deaths were reported inside the hospital(2/17,11.8%).Multivariable analysis demonstrated that mechanical ventilation before surgery was the risk factor for in-hospital mortality(r=1.02,P<0.01).The rest 15 cases were followed up with the average time of 19 months(2 months to 5 years)and no death was reported. Conclusions Neonates with PAIVS should be operated early.Individualized treatment strategy and regular follow-up are helpful to achieve better short-term outcome.

Objective To investigate peripheral blood T lymphocyte subsets and NK cells changes in the diffuse large B cell lymphoma (DLBCL) patients before and after chemotherapy,and analyse the relationship between the results and treatment.Methods Collect the 47 patients venous blood of the effective treatment DLBCL by pathology.T lymphocyte subsets and NK cells were determined by flow cytometric.Analyse the results statistically significant difference before treatment,the second chemotherapy cycle and the fourth chenmotherapy cycle compared with 50 healthy control persons.Results The levels of CD3+,CD4+,CD4+/CD8+ NK cells in DLBCL patients before chemotherapy [(70.04±8.87)％,(42.79±6.06)％,(1.68±0.59)％,(14.40±6.02)％]were lower than healthy controls [(63.89±6.67)％,(32.72±5.77)％,(0.85±0.25)％,(9.95±5.24)％](P ＜ 0.05),and the level of CD8+ cells is higher than the healthy controls [(27.21 ±6.54)％ vs.(39.92±7.11)％](P ＜ 0.05).The levels of CD3+,CD4+,CD4+/CD8+ cells had significant difference between the second and the fourth chemotherapy cycle in DLBCL patients (P ＜ 0.05).The levels of CD3+,CD4+,CD8+,CD4+/CD8+,NK cells had significant difference between the fourth chemotherapy cycle DLBCL patients and the DLBCL patients before chemotherapy (P ＜ 0.05).Conclusion The DLBCL patients exist immunosuppression before chemotherapy.Peripheral T lymphocyte subsets and NK cells can be used as good reflect of the cellular immune function in DLBCL patients.Clinical parameters can be used for the immune function monitoring and providing guidance for the treatment options.

Objective To observe the process of Vibrio vulnificus inducing dendritic cell strain apoptosis.Methods We established the mixed culture model of mouse dendritic cell ( DC 2.4 strain) and Vibrio vulnificus( Vv1.1758 strain ),analyzed morphological characteristics of cell apoptosis by DAPI fluorescence staining,detect DNA fragmentation level of apoptosis cells by DNA Ladder assay,analyze DC2.4 apoptosis rate by Annexin V FITC/PI staining,determine activities of caspase-3 and caspase-8 by means of spectrophotometric method and detect changes of mitochondrial transmembrane potential ( △ Ψm ) by JC-1 fluorescence labeling.Results After Vv1.1758 strain and DC2.4 cell were mixed and cultured for 4 h,DAPI fluorescence staining showed typical apoptosis characteristics-chromatin condensation and marginalization; DNA agarose gel electrophoresis showed apoptosis band; apoptosis rates at 2,4 and 6 h were respectively (37.8±9.8) ％,(54.3 ± 12.7 ) ％ and ( 68.2± 14.6 ) ％ ; Mitochondrial transmembrane potentials (△Ψm) at 1 h,2 h and 4 h reduced by 7.1％,16.1％ and 46.7％ respectively; caspase-8 activity increased at 1.5 h and reached the peak at 2 h [ (2.48±0.19) U/μg],while caspase-3 activity started to increase at 3h and reached the peak at 4 h [ ( 1.91 ±0.16) U/μg ].Conclusion Vibrio vulnificus could induce dendritic cells by two pathways: reducing mitochondrial transmembrane potential and activating caspase-8 promoter and finally activate effector caspase-3 to promote apoptosis.

Objective To investigate the effect of apolipoprotein E gene knock-out(ApoE KO) and high-fat diet on morphology and the expression of mortalin in hippocampal CA_3 neurons of mice, and to explore the impact of these factors on memory and Alzheimer's disease.Methods Ten wild-type and 10 ApoE KO mice were fed with common chow as the control group and the KO group respectively while 10 ApoE KO mice were fed with high fat diet.Twelve weeks later, the weight and the lid of these mice were measured.The brain tissues were observed using HE staining, nissl staining, protargol staining,immunohistochemistry staining and image analysis by computer.Results In the ApoE KO group, weight,total cholesterol, triglyceride, low-density lipoprotein cholesterol were higher than those in the control group,and these changes were more significant in ApoE KO high-fat diet group.The nissl was higher in the ApoE KO group (0.301±0.031) and in ApoE KO high-fat diet group (0.261±0.020) than those in the control group (0.341±0.035, F=18.068, P<0.05).The mortalin in the ApoE KO group (0.322±0.060) and in ApoE KO high-fat diet group (0.391±0.041) were higher than the control group (0.256±0.061, F=15.230, P < 0.05).Conclusions ApoE KO and high-fat diet can reduce nissl, and improve the expression of mortalin.This protein may be involved in the pathogenesis of Alzheimer's disease.

Objective To explore clinical characters of type 1 diabetes mellitus with different serum potassium levels in children and adolescent.Methods One hundred and seventy-five patients with type 1 diabetes mellitus were reviewed,they were divided into 3 groups according to the serum potassium level.The patients whose serum potassium

Objective To investigate the clinical efficacy of intermittent liver-nourishing and mind-regulating acupuncture in preventive treatment of migraine. Method One hundred and twenty migraine patients were randomly allocated to treatment and control groups, 60 cases each. The treatment group received intermittent liver-nourishing and mind-regulating acupuncture and the control group took flunarizine hydrochloride capsules orally. The migraine score, the visual analogue scale (VAS) score and the headache severity rating score were recorded in the subjects before treatment and at the end of treatment and the 4-week, 8-week, 12-week, 16-week and 20-week follow-ups. The Self-Rating Anxiety Scale (SAS) score and the Self-Rating Depression Scale (SDS) were recorded before and after treatment.Result After one course of treatment, the total efficacy rate was 98.3% in the treatment group and 89.5% in the control group; there was a statistically significant difference between the two groups (P<0.05). There was a statistically significant difference in the migraine score between before treatment and at the end of treatment in the two groups (P<0.05) and between the two groups at the same follow-ups (P<0.05). There was a statistically significant difference in the VAS score between before treatment and at the end of treatment in the two groups (P<0.05) and between the two groups at the same follow-ups (P<0.05). There were statistically significant differences in the SAS and SDS scores between before treatment and at the end of treatment in the two groups (P<0.05) and between the two groups after treatment (P<0.05).Conclusion Intermittent liver-nourishing and mind-regulating acupuncture has marked efficacy in preventive treatment of migraine. It can increase the total efficacy rate and reduce the number of relapses.

To study the effect of primary cilia-mediated sonic hedgehog (Shh) signaling on differentiation of rat bone marrow stromal cells (MSCs) into neuron-like cells. Methods Rat MSCs were isolated and cultured. The study was divided into normal resveratrol-cultured group, resveratrol-induced group, SAG (Smoothened [Smo] agonist) group, and cyclopamine (Smo inhibitor) group. Cell morphology was observed under inverted microscope; the expressions of Ac-Tu, Ptc, Smo, and Glil were examined by immunofluorescence method. Western blotting analysis was used to detect the protein expressions of Smo and Gli, and RT-PCR was applied to detect mRNA expressions of Smo and Glil. Results No expression of primary cilia was found in the normally cultured MSCs. After pre-induction or 24 h starvation, MSCs expressed primary cilia, Ptc, Smo and Glil proteins, with Ptc protein in the primary cilia and Smo, Glil in the cytoplasm. In normal cultivation, resveratrol did not promote the translocation of Smo or induce differentiation of MSCs into neuronal-like cells. When MSCs expressed primary cilia, resveratrol and SAG led to translocation of Smo from the cytoplasm into primary cilia, accompanied by MSCs differentiation into neuronal-like cells and significantly increased expression of Smo and Glil mRNA and protein (P< 0. 05). When cyclopamine was added, Smo remained expressed in the cytoplasm, the expression of Smo and Glil mRNA and protein was significantly decreased (P < 0. 05), and MSCs differentiation into neuronal-like cells was inhibited. Conclusion MSCs express primary cilia and have Shh signaling. Primary cilia-mediated Shh signaling participates in MSCs differentiation into neuronal-like cells.

Adolescent ; Child ; Child, Preschool ; Diabetes Mellitus, Type 1 ; diagnosis ; Diagnostic Errors ; Female ; Humans ; Infant ; Male

Arthrogryposis, renal dysfunction, and cholestasis (ARC) syndrome is an autosomal recessive disorder caused by mutations in the VPS33B or VIPAS39 gene. The aim of this study was to investigate the clinical features and VPS33B gene mutations of an infant with ARC syndrome. A 47-day-old female infant was referred to the hospital with the complaint of jaundiced skin and sclera for 45 days and abnormal liver function for 39 days. The patient had been managed in different hospitals, but the therapeutic effects were unsatisfactory due to undetermined diagnosis. Physical examination showed jaundice of the skin and sclera. Systemic skin was dry with desquamation in the limbs and trunk. There were no positive signs on cardiopulmonary examination. The liver was palpable 2.0 cm under the right subcostal margin. The hips and knees were flexed, and the extension was limited, with low muscular tone in the four limbs. Biochemical analysis demonstrated raised serum total bile acids, bilirubin (predominantly conjugated bilirubin) and transaminases, but the γ-glutamyl transpeptidase level was normal. Routine urine test revealed increased glucose as well as red and white blood cells. On genetic analysis, the infant was proved to be homologous for a VPS33B mutation c.1594C>T(p.R532X). She was definitely diagnosed to have ARC syndrome. Symptomatic and supportive therapy was given, but no improvement was observed, and the infant finally died at 3 months and 29 days of life.
Arthrogryposis ; blood ; genetics ; Bile Acids and Salts ; blood ; Bilirubin ; blood ; Cholestasis ; blood ; genetics ; Humans ; Mutation ; Renal Insufficiency ; blood ; genetics ; Vesicular Transport Proteins ; genetics

Objective: To investigate the effects of pravastatin o n atherosclerotic plaque and cardiovascular events. Methods: Fifty- seven patients with coronary artery disease (44 male and 13 female, 58.4±11.3 y ears) were randommized into pravastatin and control groups. The patients in prav astatin group were administered 10 mg of pravastatin from the night of coronary angiography day. After 7.3 months (mean) of follow-up, plasma lipid parameters and coronary angiograph were repeated. Results: (1) A favorable effect on plasma lipid parameters was found. After administration, total choles terol(TC), low density lipoprotein cholesterol (LDL-C) and triglyceride(TG) red uced by 15.0% (P<0.01), 18.0% (P<0.01) and 6.0%, respectively. High den s ity lipoprotein cholesterol(HDL-C) increased by 10.6%. However, in control grou p, TC and LDL-C showed a tendency to reduce, but no significant difference was found between those of pre- and post-administration. (2)There was no significa nt difference in luminal diameter between pre- and post-administration in both groups. (3) Cardiovascular events in pravastatin group was significantly lower than those in control (P<0.05). (4) Pravastatin had no significant effect on HR, BP and left ventricular ejection fraction in both groups. Conclusio n: Pravastatin can stabilize coronary atherosclerostic plaque and reduce the incidence of cardiovascular events by improving plasma lipid parameters.