Mitochondrial diseases is one of the common genetic metabolic diseases in children.The inheritance patterns are complex,both mendelian inheritance and mitochondrial maternal inheritance.Although the clinical feature is various and the diagnosis is difficult,but with the development of molecular biology,some clinical and genetic features in mitochondrial syndrome were gradually clear.This paper introduced the clinical feature and genetic characteristics in several common mitochondrial syndrome of children.

After the difficult position of hospital library and the experiences of Shanghai Public Health Clinical Center Library in meeting clinical and medical needs were described, such as relying on the advantages of Fudan library col-league, improving professional literacy, providing subject service, and expanding service contents, the author pointed out that providing excellent service through multiple channels and ways is the key for hospital library to improve its value and position and its sustainable development .

Hospital values in hospital management plays a leading position,the hospital values is the essence of hospital management and the core is embodied the spirit of the decisive factors in the hospital.Only thorough analysis of the reasons for the distortion of values and values distorted the adverse effects can be full demonstration Hospital values is the soul of hospital management.

Autistic Disorder ; immunology ; physiopathology ; Humans ; Immune System ; physiopathology

Sleep disorders are main clinical symptoms in perimenopausal women. The symptoms are difficulty in initiating or maintaining sleep, early morning awakening and so on. These sleep problems had made serious im-pacts on the quality of women's life during menopausal periods. Menopausal sleep disorders can be caused by changes of serum estrogen levels, vasomotor symptoms, mood disorders and patient's state of disease. This paper presented an overview of inducing factors and current situation of menopausal disorder treatment.

Using brartemicin as the leading compound, fifteen novel trehalose derivatives were designed and synthesized, and the structures were determined by 11H NMR, MS and element analysis. Inhibitory effects of the target compounds on the proliferation of A549, HepG2 and HUVEC cells were detectec by MTT assay. The abilities of adhesion, invasion and migration of A549 and HepG2 cells inhibited by the synthesized compounds were evaluated through Matrigel experiment and Transwell assay. The results showed that, the target compounds had no significant cytotoxicity (compared with the control, P>0.05) to A549, HepG2 and HUVEC cells at the dose range of 1-32 µmol.L-1. At the above dose range, the inhibitory effects of A549 cells adhesion, invasion and migration and HepG2 cells adhesion and invasion by compounds 79 and 82 are better than brartemicin.
Antineoplastic Agents ; chemical synthesis ; pharmacology ; Cell Adhesion ; Cell Line, Tumor ; drug effects ; Drug Design ; Hep G2 Cells ; drug effects ; Human Umbilical Vein Endothelial Cells ; drug effects ; Humans ; Trehalose ; analogs & derivatives ; chemistry

Objective To investigate the clinical significance of serum interferon-λ1(IFN-λ1) levels in patients with systemic lupus erythematosus(SLE).Methods ELBA was used to evaluate the serum level of interferon-λ1 in 60 patients with SLE and 20 healthy individuals as normal control.SPSS10.0 statistic software was employed to investigate the relation between the serum IFN-λ1 level,the laboratory index and SLE disease activity index(SLEDAI).Results The serum IFN-λ1 level in SLE patients(74.2±16.1 pg/ml) was higher than that of the normal controls(70.4±12.6 pg/ml,P=0.339).The serum IFN-λ1 level in SLE patients with leucopenia(80.1±18.1 pg/ml) was higher than those of the normal control(P=0.052) and the SLE patients without leucopenia(70.6±13.7 pg/ml,P=0.024).The serum IFN-λ1 level was not correlated with SLEDAI,complements C3,C4,24-hour-proteinuria.Conclusion IFN-λ1 may be involved in the pathogenesis of leukopenia in SLE and it has not been proved that its level was correlated with the disease activity.

Objective To study the effect of intravenous injection of esmolol,labetalol and their combination in the treatment of ischemic heart failure (ICD).Methods 80 patients with ICD were randomly divided into treat-ment group 1 (20 cases),treatment group 2(20 cases),treatment group 3 (20 cases)and control group(20 cases). The treatment group 1 was intravenously given esmolol on the basis of routine treatment.the treatment group 2 was intravenously given labetalol on the basis of the conventional treatment.the treatment group 3 was intravenously given esmolol and labetalol on the basis of routine treatment.The control group only received routine treatment.The thera-peutic effects of the four groups were observed and compared,and the changes of blood pressure,heart rate,walking distance and ultrasonic electrocardiogram before and after treatment were recorded.Results In the treatment group 1,3 cases with marked effect,effective in 14 cases,the total effective rate was 85%.In the treatment group 2,2 cases with marked effect,effective in 14 cases,the total effective rate was 80% cases.In the treatment group 3,4 cases with marked effect,effective in 15 cases,the total effective rate was 95%.In the control group,1 case with marked effect, effective in 10 cases,the total effective rate was 55%.The treatment effect of the treatment group 1,2,3 was better than the control group (P <0.05).The treatment effect of the treatment group 1,2 was significantly lower than the treatment group 3 (P <0.05).The treatment effect between the treatment group 1 and group 2 had no significant difference (P >0.05 ).After treatment,6 min walking distance and LVEF,FS,SV and other indicators of cardiac function were significantly improved in the four groups (P <0.05 ).The four groups had no significant adverse reactions such as nausea,vomiting,liver and renal function after treatment.Conclusion Intravenous injection of esmolol,labetalol or both drugs for treatment of ICD can improve cardiac function,with no adverse reaction,and it is safe and reliable,worthy of clinical application.

Using brartemicin as the leading compound, fifteen novel trehalose derivatives were designed and synthesized, and the structures were determined by 11H NMR, MS and element analysis. Inhibitory effects of the target compounds on the proliferation of A549, HepG2 and HUVEC cells were detectec by MTT assay. The abilities of adhesion, invasion and migration of A549 and HepG2 cells inhibited by the synthesized compounds were evaluated through Matrigel experiment and Transwell assay. The results showed that, the target compounds had no significant cytotoxicity (compared with the control, P>0.05) to A549, HepG2 and HUVEC cells at the dose range of 1-32 µmol.L-1. At the above dose range, the inhibitory effects of A549 cells adhesion, invasion and migration and HepG2 cells adhesion and invasion by compounds 79 and 82 are better than brartemicin.

Peroxisome proliferator-activated receptors(PPAR) are ligand-activated nuclear transcription factors which regulate the expression of many genes.Numbers of researchs indicate PPAR exert anti-inflammatory activities by inhibiting the expression of inflammatory singal transcription passage such as NF-?B,AP-1,STAT,meanwhile preserve from acute lung injury.This review will focus on structure of PPAR and their ligand and activity regulation and antiinflammation mechanism of action and protection to acute lung injury.