AIM: To evaluate the clinical feature of 9 patients harboring mitochondrial DNA ( mtDNA ) G11778A mutation with Leber hereditary optic neuropathy ( LHON) .
●METHODS: Nine LHON patients were enrolled and followed- up between 2012 to 2015 in Shenzhen Eye Hospital, clinical data were collected and analyzed.
●RESULTS: Six cases had maternal inheritance history ( 67%) . Three were sporadic cases. The patients aged from 9 to 43 years old, with average age of (22. 00±9. 42) years. Simultaneous onset with both eyes was in 5 cases (56%). Successively onset was in other 4 cases (44%). The ratio between male and female was 2:1. In the last follow-up, the visual acuity was finger counted in 2 eyes (11%), 0. 01-0. 1 in 12 eyes (67%), 0. 12-0. 4 in 2 eyes (11%),≥0. 4 in 2 eyes (11%). All patients had pale disc and clear boundary. ln the Humphrey visual field examination, 10 eyes had typically cecocentral or paracentral scotoma, 8 eyes had diffuse visual field defect.
●CONCLUSION: ln the 9 LHON patients with mtDNA G11778A mutation, simultaneous onset cases were more than successively onset cases within 1y cases. In most cases, LHON patients kept stable visual acuity. Rare cases had a raise in visual acuity within 1y. Majority patients had typically cecocentral or paracentral visual field scotoma. In the last stage of LHON, visual field present diffused defect. The secondary affected eye was similar performed defect as the former one.

Objective To investigate the effect of hydrogen peroxide (H_2O_2) on apoptosis and calcium ion concentration of skeletal muscle satellite cells (SMSCs) in rats, and to explore the protective effect of erythropoietin (EPO).Methods The cultured SMSCs were divided into five groups: control group,H_2O_2 group, 10, 20 and 40 U/ml EPO intervention groups.Apoptosis rates and calcium ion concentration of SMSCs were analyzed by flow cytometry, and the morphology of apoptotic cells was observed by Hoechst33258 staining.Results The apoptosis rates showed significant differences (all P<0.05) among (1.93±0.57)% in control group, (22.13±1.79)% in H_2O_2 group, (16.47±2.53)%, (4.97±0.55)% and (2.93±0.47)% in 10, 20 and 40 U/ml EPO intervention groups, respectively.And calcium ion concentrations in SMSCs were 12.67 ±0.32, 27.90±0.06 and 44.53±0.93 in 10, 20 and 40 U/ml EPO intervention groups, respectively.There was significant difference in calcium ion concentration between H_2O_2 group and control group (9.70±0.09 vs.51.37± 0.64, P< 0.05).Morphology of apoptosis was observed by Hoeehst33258 dye stains in 10, 20 U/ml EPO intervention group and H_2O_2 group, while there were less apoptotic bodies in 40 U/ml EPO intervention group and control group.Conclusions EPO might have protective effects on SMSCs injured by H_2O_2 through inhibiting apoptosis and calcium ion releasing from SMSCs.

AIM: To establish a reversed-phase HPLC method of determining three kinds of berberine type alkaloids(jatrorrhizine,palmatine and berberine) in Rhizoma Coptidis and Jinqi Jiangtang Tablet(Flos Lonicerae japonicae,Rhizoma Coptidis and Radix Astragali) METHODS: RP-HPLC was used for determination.The separation was carried out on Diamonsil C_(18)column(4.6 mm?250 mm,5 ?m) with the mobile phase containing acetonitrile-0.1 mol/L potassium dihydrogen phosphate(30(∶)70) and a detective wavelength was at 345 nm.The flow rate was 1.0 mL/min. RESULTS: The average contents of jatrorrhizine,palmatine and berberine in Rhizoma Coptidis and Jinqi Jiangtang Tablet were 1.34%,1.61%,7.12% and 0.35%,0.64%,1.54% respectively. CONCLUSION: The method is simple,accurate with a good recovery and repeatability and can be used to control the quality of Rhizoma Coptidis and Jinqi Jiangtang Tablet.

Objective Base on the neighborhood activation model(NAM), to develop the Mandarin monosyllable lexical neighborhood test(M-LNT-monosyllable), which is expected to be useful for evaluating speech perception performance in children with hearing loss. Methods Test items were based on the lexical characteristics of word frequency and neighborhood density which addressed in the neighborhood activation model (NAM). M-LNT -monosyllable consisted of two parts: Lexically "easy" words with high word frequency, which were low phonemically similar to the target word and lexically "hard" words with low word frequency, which were high phonemically similar to the target word. 34 children of 3～5 year old with normal hearing were choosed as subjects to verify easy word and hard word lists. Results 1 979 words for children contained 487 easy words and 419 hard words. Three easy word lists and three hard word lists were developed to estimate the performance of word recognition among normal- hearing children. There were no differences among scores of three easy words lists(P＞0.05), and no difference among scores of three hard words lists(P＞0.05). But there were significant differences between scores of easy and hard words lists(P＜0.01). Conclusion The development of the lexicon was affected by the lexical characteristics. Normal-hearing children with some lexical techniques were affected by the lexical characteristics when they recognized the spoken words, but children with less lexical technique didn't show the same result because the recognition was processed on the phonetic level.

Adult ; Chronic Disease ; Female ; Humans ; Mucus ; Pulmonary Eosinophilia ; metabolism ; pathology

Objective:To evaluate the safety and feasibility of endovascular recanalization for non-acute internal carotid artery occlusion (NA-ICAO), and to propose a new angiographic classification.Methods:From April 2015 to October 2019, 95 consecutive patients with symptomatic NA-ICAO who received endovascular recanalization were retrospectively analyzed in Beijing Tiantan Hospital, Capital Medical University. All the patients were divided into four groups according to DSA: type Ⅰ, petrous segments were distally reconstituted by collateral vessels; type Ⅱ, cavernous segments were distally reconstituted by collateral vessels; type Ⅲ, ophthalmic segments were distally reconstituted by collateral vessels; type Ⅳ, communicating segments were distally reconstituted by collateral vessels. Study data including clinical characteristics, surgical details, lesion classification, recanalization rate and perioperative complications. For the counting data, the χ 2 test was used to compare between groups. For the quantitative data, the ANOVA was used for the normal distribution data, otherwise the Kruskal-Wallis H test was used. The primary safety outcome was any stroke or death within 30 days. Results:Among the 95 patients, 67 (70.53%) had successful recanalization. The recanalization rates of type Ⅰ－Ⅳ were 92.31% (36/39), 81.82% (18/22), 47.83% (11/23) and 18.18% (2/11) respectively (χ2=29.557, P<0.001). And the complication rates of the four types were 5.13% (2/39), 13.64% (3/22), 21.74% (5/23) and 9.10% (1/11) respectively. The incidence of perioperative ischemic stroke was 2.11% (2/95). No other serious stroke and death occurred. Conclusions:Endovascular recanalization may be feasible and safe for carefully selected patients with NA-ICAO and therefore represents an alternative treatment. The patients with type Ⅰ and Ⅱ lesions had higher recanalization rates, while the patients with type Ⅳ lesions had significantly lower recalculation rate. The new angiographic classification is conducive to the selection of suitable patients and difficulty in grading.

Objective To investigate the influence of in vitro low-temperature degradation(LTD)treatment on the structural stability of 5 kinds of Y2O3 stabilized tetragonal zirconia polycrystals(Y-TZP)dental ceramics.Methods TZ-3YS powder was compacted at 200 MPa using cold isostatic pressure and pre-sintered at 1050℃for 2 h forming presintered blocks.Specimens were sectioned into 15 mm×15 mm×1.5 mm slices from blocks of TZ-3YS,Vita In-Ceram YZ,Ivoclar,Cercon Smart.and Kavo Y-TZP presintered blocks,18 slices for each brand,and then densely sintered.Specimens were divided into 6 groups and subjected to an accelerated aging test carried out in an autoclave in steam at 134℃,0.2 MPa,for 0,1,2,3,4,and 5 h.X-ray diffraction(XRD)was used to identify crystal phases and relative content of monoclinic phase was calculated.Specimens for three-point bending test were fabricated using TZ-3YS ceramics according to the ISO 6872 standard and bending strength was tested before and after aging.The polished and aging specimens of TZ-3YS and Cercon Smart zirconia ceramics were observed by atomic force microscopy(AFM)to evaluate surface microstructure. Resuits Tetragonal-to-monoclinic phase transformation was detected for specimens of TZ-3YS,Vita In-Ceram YZ,Ivoclar,and Kavo zirconia ceramics except for Cercon Smart ceramics after aging,and the relative content of monoclinic phase was increasing with the prolonged aging time.TZ-3YS was the most affected material,Kavo took the second,and Vita and Ivoclar were similar.Aging had no significant negative effects on flexural strength of TZ-3YS with average bending strength being over 1100 MPa.The nucleation and growth of monoclinic phase were detected by AFM in surface of Cercon Smart zirconia in which monoclinic phase was not detected by XRD.Conclusions The results suggest that LTD of dental Y-TZP is time dependent,but the aging test does not reduce the flexural strength of TZ-3YS.The long-term clinical serviceability of dental Y-TZP needs further observation.

OBJECTIVETo study the effect of liposomal transfection of antisense oligonucleotide (ASON) on the erythroid cell alpha-globin gene in the patients with severe beta-thalassemia, and provide a new idea for beta-thalassemia gene therapy.

METHODSA highly effective ASON targeting alpha-globin gene was transfected into severe beta-thalassemic erythroid cells cultured in vitro by liposomal at an optimal concentration. The expression level of alpha, beta, gamma-globin gene, the level of hemoglobin, and the excess alpha-globin chains precipitates in ASON group and control group were carefully analyzed by quantitative real-time PCR(Q-RT-PCR), high performance liquid chromatography (HPLC), and electron microscope, respectively.

RESULTSThe mRNA expression of alpha-globin gene was significantly lower in ASON group (9.04 +/- 0.29) than in control group (24.23 +/- 0.29) (P<0.01). Simultaneously, the disequilibrium between alpha- and beta-, gamma-globin gene expression was partly modified by ASON, the ratios of ASON group and control group being 0.79 +/- 0.02 and 2.26 +/- 0.06 respectively (P<0.01). HPLC demonstrated that the levels of HbA2 and HbF increased with downregulation of alpha-globin gene in beta-thalassemic erythroid cells, particularly HbF. The precipitates of alpha-globin chains in ASON group were lessened under electron microscope, particularly in early erythroblast while no change in the control group.

CONCLUSIONThe high effective ASON contributes to inhibit the alpha-globin gene expression of severe beta-thalassemic erythroid cells, partly modify the disequilibrium between alpha-, beta- and gamma-globin gene expression and obviously reduce the precipitates of alpha-globin chains in erythroid cells. It might provide a new idea for gene therapy of beta-thalassemia.

Cells, Cultured ; Child ; Genetic Therapy ; Humans ; Liposomes ; Oligonucleotides, Antisense ; genetics ; Transfection ; alpha-Globins ; genetics ; metabolism ; beta-Globins ; metabolism ; beta-Thalassemia ; genetics ; metabolism ; therapy ; gamma-Globins ; metabolism

OBJECTIVETo obtain and investigate the chromaticity properties of colored dental 3Y-TZP ceramics with different colorant combinations.

METHODSColorant combinations were mixed with TZ-3Y-S powder, then the mixtures were compacted at 200 MPa using cold isostatic pressure, densely sintered at 1500 degrees C for 2 h forming 5 graded colored dental zirconia ceramics. Specimen were sectioned into 10 mm x 10 mm x 0.5 mm and 10 mm x l0 mmx 1 mm slices from the sintered blocks. Color measurement was performed under black background using spectrophotometer. The chromaticity properties were compared with that of the VITA In-Ceram YZ shade guide.

RESULTSFive colored dental zirconia ceramics were obtained. The lightness decreased gradually, and the chroma value increased gradually. The color differences of the two thickness specimens were little. The parameters of the color space were L*: 67.76-77.78; a*: -2.19-3.80; b*: 12.13-25.01, which was similar with that of the VITA In-Ceram YZ shade guide, while the lowest value of lightness was relatively higher than that of the VITA In-Ceram YZ shade guide.

CONCLUSIONColored 3Y-TZP dental ceramics are suitable for clinical use. There is a need to develop more darkness shaded zirconia dental ceramics.

Aluminum Oxide ; Ceramics ; Color ; Dental Porcelain ; Humans ; Zirconium

Objective To examine the distribution and trends of hospitalization rates for coronary heart disease (CHD) from 2007 to 2009 in Beijing.Methods We calculated hospitalization rates for CHD using data from Beijing Hospital Discharge Information System.Information of census registered population in Beijing was obtained from Beijing Municipal Bureau of Statistics.CHD includes acute myocardial infarction,unstable angina and other forms of CHD. Age-standardized hospitalization rates for CHD per 100 000 population aged 25 years or more were calculated.Results During 2007 - 2009,a total of 248 049 patients aged 25 years or more hospitalized in Beijing with the primary discharge diagnosis of CHD were enrolled,of whom 73.7％ were permanent registered Beijing citizens.The average hospitalization rate for CHD in 2007 -2009 was 651.2/100 000 for the permanent residences in Beijing (741.2/100 000 in men,560.9/100 000 in women).The highest average hospitalization rote (671.9/100 000) was seen in exurban area compared to other areas in Beijing.The average hospitalization rate for acute myocardial infarction,unstable angina,and other CHD was 126.4/100 000,226.4/100 000 and 298.4/100 000,respectively.The hospitalization rate for CHD increased 18.1％ from 2007 to 2009 (from 598.1/100 000 to 706.5/100 000 ).The same trend was seen in women ( 20.2％ ) and men ( 16.6％ ).The hospitalization rates of CHD in the urban,suburban,and exurban areas of Beijing all increased in the three years,and the greatest increase (36.6％ ) was found in exurban area. Hospitalization rates of acute myocardial infarction and unstable angina increased 24.5％ and 55.3％,respectively,in the three years,while hospitalization rates of other CHD decreased 5.7％.Conclusions The hospitalization rate of CHD is higher in men than in women in Beijing.The hospitalization rates for CHD increased from the observation period,especially in those living in exurban area.Awareness of the magnitudes and trends of CHD hospitalization rates is of great importance in evaluating the burden of cardiovascular disease,allocating and utilizing health care resources,and estimating the health insurance for Beijing.