Objective To evaluate the efficacy in aromatase inhibitors and tamoxifen on Luminal postmenopausal breast neoplasms.Methods PubMED,Science Direct,EBSCO Host,EMbase,The Cochrane Library,CNKI,CECDB and CQVIP were retrieved,the evaluation methodology included.The quality of the included studies and extracted data should be researched rigorously.Review Manager 5.0 software was used to evaluate the quality standards of randomized clinical trial (RCT).Results Twelve RCT were selected,included 2634 patients,1354 cases of treatment group (use aromatase inhibitors),1280cases of control group (use tamoxifen).The results of the evaluation of the system:compared to control group,treatment group significantly improved disease-free survival in 1 year (RR =1.10,95％ CI 1.01-1.19),disease-free survival in 3 years (RR =1.09,95％ CI 1.05-1.13),the overall efficiency in 1 year (RR =1.21,95％ CI 1.12-1.30),furthermore treatment group significantly reduced the incidence of cardiovascular diseases in 3 years (RR =0.47,95％ CI 0.27-0.83),but there was no significant difference in the incidence of bone and joint disease in 3 years between the 2 groups (RR =0.99,95％ CI 0.61-1.61).Conclusion Compared to tamoxifen,the efficacy of aromatase inhibitors is excellent in treatment of Luminal postmenopausal breast neoplasms,it is worth promoting.

[Summary] A single-incision video-assisted thoracoscopic sublobular limited resection was performed for a 2-cm pulmonary nodule at the right lower lobe of the lung in August 2014.The post-operative pathological diagnosis was inflammatory myofibroblastic tumor of the lung.No recurrence and metastasis was found after one year’s follow-up.

The effects of glutamate transporters on synaptic plasticity in rat models of pilocarpine-induced status epilepticus were investigated. Male Wista rats ((304.06?13.79) g) were randomly divided into 5 groups, short-term seizures (SE) and its control (SC), long-term seizures (LE) and its control(LC), normal control (Sham) groups. Epilepsy rat models were induced by injection of pilocarpine(25 mg/kg, i.d.). Glutamate transporter inhibitor, DL-threo-benzyloxyaspartate (TBOA, 7.5 nmol,1 ?l) was microinjected into right side of hippocampus after 14 days of initial status epilepticus in SE and LE groups. The same volumes of artificial cerebrospinal fluid were injected into same side of hippocampus in SC and LC groups. Electroencephalographys (EEG) were detected in SE and SC groups after 2 h of drug injection. Long term potential (LTP) at perforant pathway and dentate gyrus(PP-DG) and EEG were recorded in LE and LC groups after two weeks of drug injection. Example of Fluoro-Jade-B staining in the rat brain was made at the end of electrophysiological experiment. The results showed that there was a significant decrease in theta band power of EEG in SE group compared with that of SC group (P 0.05). The slope of excitatory postsynaptic potential (EPSP) was significantly increased in LE group compared with that of LC group (P

OBJECTIVE:To establish an effective technical system to achieve the informatization management in drug produc-tion process,and provide reference for the drug informatization management during production process in China. METHODS:Through analyzing the common problems existing in informatization management in drug production process,above problems were solved by interactive integration marketing system(IIMS)based on mobile internet. Informatization supervision was conducted for the production progress of a batch of drugs in a pharmaceutical production enterprise,and feasibility of the technical system was in-vestigated. RESULTS:There were problems commonly existed in the production process,such as supervision and data collection out of touch,no advanced means,delayed and closed informatization transmission,high cost of informatization collection,hard to develop,etc. IIMS consisted of transit label,informatization collection terminal and monitoring management platform had achieved batch production records paperless data interaction and solved informatization interaction and supervision issues in process of drug quality. In the actual study,IIMS totally supervised 15 links from raw materials purchasing to finished products,and all data were successfully collected. IIMS achieved the real-time remote control of data to make the government easy to regulate,without chang-ing the operational processes,and did not cause economic burden to the enterprises. CONCLUSIONS:The technical system is suit-able for the informatization management in drug production process in pharmaceutical production enterprise,and it can be extended to the scientific research institutes and drug supervision departments in the further.

Objective:To established a modified implanting model of VX2 liver tumor in rabbit on the base of the classic implanting method, and compared the results within the two methods. Methods:30 rabbits with the mean weight of (2.65?0.29)kg were divided randomly into two groups with 15 rabbits each. The rabbits in Group A received classic implantation for induction of the liver tumor model, and Group B were inducted by injecting a piece of tumor tissue into the left anterior lobes of liver. Implanting time of each group was recorded and compared, and spiral CT scan was performed at 8th day, 15th day, 22nd day, 29th day postoperatively. The manifestation of tumors in CT scan was observed and tumor volume was calculated simultaneously with formula V=1/2ab2 (a=the shortest diameter and b=the longest diameter).Each tumor was confirmed through pathology. Results:The implanting time of Group A and Group B were (9.47?2.85)min and (5.85?1.62)min, respectively, with significant difference between them. Besides, there was statistical difference of the achievement ratio between two groups, as it was 53.3% for Group A and 86.7% for Group B. No significant difference was found for the tumor growth between two groups. Conclusion:Modified implanting method for induction of the rabbit liver tumor model was superior to the classic implanting method.

OBJECTIVETo investigate the clinical and laboratory features of very long chain acyl-CoA dehydrogenase deficiency ( VLCADD ) and the correlations between its genotype and phenotype.

METHODEleven patients diagnosed as VLCADD of Shanghai Jiaotong University School of Medicine seen from September 2006 to May 2014 were included. There were 9 boys and 2 girls, whose age was 2 d-17 years. Analysis was performed on clinical features, routine laboratory examination, and tandem mass spectrometry (MS-MS) , gas chromatography mass spectrometry (GC-MS) and genetic analysis were conducted.

RESULTAll cases had elevated levels of blood tetradecanoylcarnitine (C14:1) recognized as the characteristic biomarker for VLCADD. The eleven patients were classified into three groups: six cases in neonatal onset group, three in infancy onset group form patients and two in late onset group. Neonatal onset patients were characterized by hypoactivity, hypoglycemia shortly after birth. Infancy onset patients presented hepatomegaly and hypoglycemia in infancy. The two adolescent patients showed initial manifestations of exercise intolerance or rhabdomyolysis. Six of the eleven patients died at the age of 2-8 months, including four neonatal onset and two infant onset patients, with one or two null mutations. The other two neonatal onset patients were diagnosed since early birth through neonatal screening and their clinical manifestation are almost normal after treatments. Among 11 patients, seventeen different mutations in the ACADVL gene were identified, with a total mutation detection rate of 95.45% (21/22 alleles), including eleven reported mutations ( p. S22X, p. G43D, p. R511Q, p. W427X, p. A213T, p. C215R, p. G222R, p. R450H, p. R456H, c. 296-297delCA, c. 1605 + 1G > T) and six novel mutations (p. S72F, p. Q100X, p. M437T, p. D466Y, c. 1315delG insAC, IVS7 + 4 A > G). The p. R450H was the most frequent mutation identified in three alleles (13.63%, 3/22 alleles), followed by p. S22X and p. D466Y mutations which were detected in two alleles (9.09%, 2/22 alleles).

CONCLUSIONThe ACADVL gene mutations were heterozygous in our patients. The mortality of neonatal onset form and infant onset form is much higher than the late onset form patients, suggesting a certain correlation between the genotype and phenotype was found. The earlier diagnosis and treatment of VLCADD are of vital importance for the improvement of the prognosis of the patients.

Acyl-CoA Dehydrogenase, Long-Chain ; deficiency ; genetics ; Adolescent ; Age of Onset ; Alleles ; Asian Continental Ancestry Group ; Child ; Child, Preschool ; China ; Female ; Genetic Testing ; Genotype ; Heterozygote ; Humans ; Infant ; Infant, Newborn ; Lipid Metabolism, Inborn Errors ; complications ; genetics ; Male ; Mitochondrial Diseases ; complications ; genetics ; Muscular Diseases ; complications ; genetics ; Mutation ; Neonatal Screening ; Phenotype ; Prognosis ; Rhabdomyolysis ; etiology ; Spectrum Analysis ; Tandem Mass Spectrometry

Objective To investigate the therapeutic effect of three-dimensional interactive reduction of femoral neck and shaft in treatment of irreducible femoral neck fracture.Methods The study enrolled 40 patients with irreducible femoral neck fractures treated by three-dimensional interactive reduction of femoral neck/shaft and internal fixation with three cannulated screws from June 2011 to July 2013 (study group).Frontal and lateral X-ray films were taken after operation.Garden index was used to evaluate the fracture reduction quality and Harris hip score was recorded.Meanwhile,a retrospective analysis was performed on 32 patients with irreducible femoral neck fractures treated by open reduction and internal fixation with cannulated screws between January 2008 and December 2010 (control group).Results Patients in study group obtained satisfactory reduction by minimally traumatic closed reduction.According to the Garden index,fracture reduction quality was level 1 in 24 patients and level 2 in 16 patients.Thirty-two patients were followed up for 12-28 months (mean,21 months),which showed fracture healing.At the final follow-up,Harris hip score was 87 points (range,61-100 points) and 4 patients had femoral head avascular necrosis.By contrast,6 patients in control group showed fracture nonunion.At the final follow-up,Harris hip score was 60 points (range,20-100 points) and 8 patients had femoral head avascular necrosis.Conclusion Three-dimensional interactive reduction of femoral neck and shaft provides good hip function recovery and decreased incidence of femoral head avascular necrosis and fracture disunion compared with the open reduction and internal fixation.

Objective To investigate the potential clinical factors associated with the prognosis and relapse of adult onset Still's disease(AOSD).Methods The factors possibly influencing the prognosis and relapse of AOSD were analyzed by logistic regression and COX regression in the cohort study.Ninety-six con- secutive inpatients of AOSD diagnosed based on Yamaguchi criteria in the hospital from March 1996 to September 2004 were included in the study.Results Nine cases(9.4%)were lost during the follow-up. Eleven patients(12.6%)were diagnosed as other diseases(5 with other rheumatic diseases,4 with tumor and 2 with infections)in the 87 follow-up cases.In 76 cases,3 patients(3.9%)died and 33 patients(43.4%) got remission over one year after treatment.Splenomegaly(OR=3.14,95%CI=1.01～9.74)and treated with methotrexate(OR=0.22,95%CI=0.07～0.67)were associated with the prognosis from the logistic regression analysis of the 76 cases.The serum ferritin(RR=I.05,95%CI=1.01～1.08)and treated with methotrexate (RR=0.13,95%CI=0.02～0.76)were associated with relapse from the COX regression analysis of the 61 remis- sion cases.Conclusion We need to be very cautious in the follow-up of AOSD patients because some of them may change to other diseases.Methotrexate may be an importent therapy of AOSD not only in improve- ment the prognosis but also in reduction of relapse.

Objective To analyze gene mutations of a Niemann-Pick disease type C (NPC) proband,and carry out prenatal diagnosis for the family.Methods The coding regions of NPC1 gene in the proband (late-infantile form) and white blood cell (WBC) in peripheral blood of its parents were amplified by polymerase chain reaction and direct DNA sequencing in both directions was performed.The sequencing results were compared with human NPC1 gene sequence (NM_000271) in GenBank,and sequences of mutated exons were determined.Direct sequencing was used on 50 normal Chinese individuals' DNA samples (control) to exclude mutation's single nucleotide polymorphism (SNP).An inter-species alignment of homologous NPC1 proteins was performed using ClustalX 1.81 software.During the second pregnancy of the proband's mother,the amniotic fluid was obtained at 18 weeks of gestation and the amniocytes were cultured for gene mutation analysis.Neonate's DNA of WBC in peripheral blood was also extracted for NPC1 gene analysis.Results Mutation analysis of NPC1 gene revealed two novel heterozygous mutations (c.2284-2287 delCTCT and p.V959G) in the proband,which originated from her father and mother,respectively.These two mutations were absent in the control,suggesting that these mutations were not SNP.While comparing with the amino acid in NPC1 protein of human,mouse,rat,rabbit,cat and pig,it revealed that p.V959 belonged to a conservative amino acid region and the missense mutation of p.V959G may perturb the function of NPC protein.Neither mutation was found in DNA from amniotic fluid or from the cultivated amniocytes in the second pregnancy,suggesting a normal fetus.c.2284-2287 delCTCT and p.V959G mutation were not found in NPC1 gene analysis of WBC in peripheral blood of the neonate,which was consistent with the prenatal diagnosis.Conclusions PCR-direct sequencing could be used as genetic diagnosis for NPC proband and prenatal diagnosis for its family.The mutation p.V959G may be correlated to late infantile form of NPC.

Objective Sphingolipidoses are a group of rare genetic disorders caused by catabolism defect of sphingolipids by lysosomal hydrolases with diverse presentations,and represent an important health problem to almost all ethnic populations. To date,there is no epidemiologic study on the prevalence of sphinglipidoses,individually,or as a group,in China. We set up a series of enzymatic assays that could make definite diagnoses with the aim to collect data for an epidemiologic investigation of sphingolipidoses and also pave the way to prenatal diagnosis to decrease the rate of inborn error of metabolism. Methods Patients with suspected sphingolipidosis were recruited from pediatric endocrinology and inherited metabolism outpatient clinics of Xinhua Hospital. Leukocytes were isolated with dextran from peripheral bloods. Activities of leukocyte acid β-glucosidase,acid sphingomyelinase,arylsulphatase A,galacto-cerebrosidase,beta-galactosidase were measured using their specific artificial fluorescent substrates,while arylsulfatase A was determined by a colorimetric assay with dipotassium 2-hydroxy-5-nitrophenyl sulfate as the substrate. Results In one year,we identified 17 patients with 5 different kinds of sphingolipidoses,including 3 patients with Gaucher disease,9 patients with Niemann-Pick type A/B,2 patients with metachromatic leukodystrophy,2 patients with Krabbe disease,and 1 patient with GM1 gangliosidosis. We made brief descriptions of disease characters of each different kind disease and compared our results with findings of other ethnic groups. Conclusions Sphinglipidoses was markedly under-diagnosed in China and general pediatricians should be alerted to sphinglipidoses.