Objective To analyze the pathogenesy and mutation of X-linked juvenile retinoschisis (XLRS) 1 gene in XLRS families, and to provide the theory basis in directing gene diagnosis. Methods The mutation of XLRS1 gene code in two XLRS families were detected and screened by polymerase chain reaction (PCR) and DNA direct sequence determination. Results Pro193Ser mutation was detected in family 1. Conclusion Pro193Ser mutation could be found in XLRS families, which can be used for genetic consultation and prenatal gene diagnosis.

This paper deals with the regional anatomy of the neck and some common acupoints. Acupoint Renying (ST 9) is taken as an example to elaborate the needling techniques and needling precautions of neck acupoints.

Objective To investigate the changes and the significances of pregnant women with hypothyroidism companied with gestational diabetes mellitus.Methods 76 cases of hypothyroidism and subclinical hypothyroidism companied with gestational diabetes mellitus pregnant women admitted to our department from Sept 2014 to Mar 2016 were selected as the observation group.According to the levels of FT3,FT4 and TSH,the observation group were divided into two group(A group and B group).Cobas e601 was used to examine TSH and PT,APTT,TT,FIB and ATⅢ were determined with ACL-TOP.PLT,MPV and PDW were determined by Sysmex2000i.Results FIB (3.98±1.74 g/L),MPV (10.18±1.53 fl)and PDW (15.03％±1.54％)in A group were significant higher than those in B group (3.91±1.62 g/L,9.37±1.48 fl,14.41％± 1.35％％) and the control group (3.47±1.43 g/L,8.96±1.42 fl,13.67％±1.26％).FIB,MPV and PDW in B group was higher than those in the control group.PT (12.26±1.41 s,12.21±1.39 s) and APTT (31.80±8.72 s,30.43±8.54 s) in A group and Bgroup were higher than those in the control group (12.13 ± 1.32 s,29.24± 8.37 s).Otherwise,AT Ⅲ (78.47％± 10.36％,79.58％ ± 10.22％) in A group and B group was significant lower than that in the control group (86.56％±8.86％).There were distinct difference (t=3.072～6.153,P＜0.05).Conclusion The disorder of blood coagulation existed in pregnant women with hypothyroidism companied with gestational diabetes mellitus.Early diagnosis and intervention can reduce the occurrence of thrombotic disease and cardiovascular disease.

Objective To analyze the risk factors of aortic valve calcification,and discuss its correlation with coronary artery disease.Methods A total of 188 patients who underwent coronary angiography (CAG) and transthoracic echocardiography (TTE) were divided into two groups:101cases with aortic valve calcification (AVC) and 87 cases without AVC (NAVC).General data such as sex,age,height,weight and hypertension history,results of blood test such as glucose,lipid and homocysteine(HCY)level were recorded.Results In AVC versus NAVC group,age were (67.0±9.0) years vs.(59.4 ± 6.9) years (t =6.74,P =0.000),men were 36 cases (35.6％) vs.44 cases (50.6％) (t=4.26,P=0.039),hypertension patients were 72 cases (71.3％) vs.50 cases (57.5％)(x2=3.92,P=0.048),total cholesterol were (5.4 ± 1.0) mmol/L vs.(4.5 ± 1.0)mmol/L (t =5.70,P=0.000),triglyceride were (2.2 ± 1.1 ) mmol/L vs.( 1.6 ± 0.8) mmol/L (t =4.04,P =0.000),HCY were (17.6±8.8) μmol/L vs.(14.9±6.6) μmol/L (t=2.86,P=0.028),respectively.One-way analysis showed that age,sex,hypertension,total cholesterol,triglyceride had relationship with aortic valve calcification.When we divided the cases into two groups (with and without coronary disease),there is no significant difference in HCY(t=0.88,P=0.382) between the two groups.Logistic regression indicated that age,total cholesterol,triglycerides,HCY were independent risk factors of aortic valve calcification,the incidence of aortic valve calcification was related with the severity of coronary artery lesion (x2 =9.48,P =0.024 ).Conclusions The independent risk factors of aortic valve calcification are age,cholesterol,triglyceride,HCY.Higher incidence of aortic valve calcification may result in greater severity coronary artery lesion.

Objective To explore the protective effect and mechanism of gardenoside on the damage of dopaminergic neurons induced by lipopolysaccharide (LPS). Methods Both neuron-enriched cultures and neuron-astrocyte cultures were pretreated with vehicle or gardenesides ( 10, 20 and 40 mg/L) for 30 min at 37℃. The culture media were subsequently renewed in order to remove gardenesides. LPS was then added into all culture media at a final concentration of 10 mg/L Twenty-four hours later, the culture media was collected to measure TNF-α, NO, IL-6, GDNF and MMP-9; the cells were collected to count the number of cells labeled with an antibody against tyrosine hydroxylase (TH) and to assess the expression of TH mRNA using reverse transcription-polymerase chain reaction. Results Gardeneside didn't promote the survival of dopaminergic neurons in neuron-enriched culture, but significantly increased the survival of dopaminergic neurons in neuron-astrocyte culture, compared with the vehicle group, the survival of dopaminergic neurons increased from 203.0%±17.4% to 256.7%±15.2% ( F = 17.22, P = 0.001 ) in 40 mg/L gardenaside group. The amount of TNF-α, NO and GDNF released from the neuron-astrocyte cultures after 24 h of addition of LPS was not changed significantly, while the expression of IL-6 and MMP-9 was increased significantly. In this study, the gardenoside concentration-dependently attenuated the LPSinduced increase of the expression of IL-6 and MMP-9, compared with the vehicle group, the expression of IL-6 and MMP-9 decreased to 67.2%±6.4% (F= 12.89,P =0.001 ), 77.3%±9.8% (F =8.27,P = 0.001 ) respectively in 40 mg/L gardenoside group. Conclusions Astrocytes play a neuroprotective role on dopaminergic neurons, which is decreased by LPS via inducing the secretion of pro-inflammatory factors. Gardeneside may protect dopaminergic neurons from LPS-induced injury in an astrocyte-dependent manner and it inhibits the production of proinflammatory factors instead of promoting the secretion of GDNF. From the point of view that a very low toxicity of gardenesides has been well documented, this report may reveal a new way of developing therapeutic interventions for inflammation-related diseases such as Parkinson's disease.

OBJECTIVE:To establish the method for simultaneous determination of related substances in Amlodipine hydrochlo-rothiazide and valsartan tablets. METHODS:HPLC method was used. The determination was performed on Symmetry C18 column with mobile phase consisted of water-acetonitrile-trifluoroacetic acid(90:10:0.1,V/V/V)and water-acetonitrile-trifluoroace-tic(10:90:0.1,V/V/V)(gradient elution)at the flow rate of 0.6 mL/min. The detection wavelength was set at 237 nm(impurity A) and 225 nm(impurity B,C,D). The column temperature was 30 ℃. RESULTS:The linear range of impurity A,B,C,D were 93.43-987.34 ng/mL(r=0.9994),12.27-255.92 ng/mL(r=0.9996),78.96-657.17 ng/mL(r=0.9999),28.39-218.16 ng/mL(r=0.9997),respectively. The limits of quantitation were 91.27,11.35,78.31,26.56 ng;the limits of stability and detection were 22.98,3.13,19.17,8.16 ng. RSDs of precision tests were lower than 2.0%. Only impurity A was found in repeatability test,RSD=0.79%. Recoveries were 99.4%-100.6%(RSD=0.41%,n=9),98.9%-102.0%(RSD=1.04%,n=9),99.4%-100.9%(RSD=0.56%,n=9),98.6%-101.2%(RSD=0.92%,n=9),respectively. CONCLUSIONS:The method is simple,rapid,sensitive and repeatable,and can be used for the determination of related substance in Amlodipine hydrochlorothiazide and valsartan tablets.

OBJECTIVE:To establish the quality standards for Fufang ningshen granule. METHODS:TLC was used for the qualitative identification of Radix angelicae and White paenoy;HPLC was adopted for the contents determination of spinosin and verbascoside:the column was Kromasil C18 with the mobile phase of acetonitrile-0.1% acetic acid(16∶84,V/V)at a flow rate of 1.0 ml/min,detection wavelength was 330 nm,column temerpature was 30 ℃. RESULTS:The TLC spots of R. angelicae and W. pae-noy were clear with good separation,negative control without interference. The linear range was 0.055 44-0.277 2μg for spinosin(r=0.999 4)and 0.055 98-0.279 9 μg for verbascoside(r=0.999 5);RSDs of precision,stability and reproducibility tests were lower than 3.0%;recoveries were 95.62%-100.53%(RSD=1.77%,n=9) and 95.63%-102.57%(RSD=2.74%,n=9). CONCLU-SIONS:The standard can be used for the quality control of Fufang ningshen granule.

Humans ; Pneumoconiosis ; diagnosis

Command of the JCI standard is conducive to promoting the "Patient-centered" quality management concept awareness and realizing the patient safety objective. To this end, a campaign to stage the hospital quality management system and bottom-up quality activities has been launched. These efforts may help improve the hospital quality management system, covering such aspects as standardized document management, human resource management, normalized diagnosis and therapy management centering on clinical path, clinical nursing service quality, safety management, equipment and facility management, as well as data analysis and quality improvement system. These have been subject to and certified by JCI with a gold medal.

BACKGROUND:SIRT6/NF-κB is the important signal axis to cellsenescence, but the effect of SIRT6/NF-κB signal axis to hematopoietic stem and progenitor cell(HSC/HPC) senescence is unclear. OBJECTIVE:To induce (t-BHP) in vitro and to investigate the role of SIRT6/NF-κB signal axis in Sca-1+HSC/HPC senescence induced by tert-butylhydroperoxide in vitro. METHODS:Sca-1+HSC/HPC was isolated and purified by magnetic activated cellsorting. Sca-1+HSC/HPC senescence was induced by 100μmol/L tert-butylhydroperoxide in vitro. The senescence-associatedβ-Galactosidase staining, cellcycle analysis and culture of mixed hematopoietic progenitor cellwere used to investigate the biological effects of tert-butylhydroperoxide on Sca-1+HSC/HPC senescence. The expression of senescence associated SIRT6, NF-κB mRNA and protein was examined by real-time fluorescence quantitative PCR and western blot assay. RESULTS AND CONCLUSION:Compared with control group, the percentage of positive cells expressing SA-β-Gal and cells in G0/G1 phase increased and the number of forming colony of mixed hematopoietic progenitor decreased in the aging group. It showed lower expression of SIRT6 and higher expression of NF-κB in the aging group. The SIRT6/NF-κB signal axis may play a key role in the Sca-1+ HSC /HPC senescence inducted by tert-butylhydroperoxide.