Mucopolysaccharidosis type Ⅱ is a rare single gene inherited disorder. In the earlier studies, more attention was paid to the molecular analysis of the hot spots of the mutations. With the detection of more and more novel mutations,more studies are trying to analyze the effect on splicing of different types of mutations and the genotype-phenotype correlations. This review will introduce the progress on molecular genetics, the gene mutation,analysis of the female patients and the genotype-phenotype correlations. With the study of these progress,it suggested that more attention should be paid to the possibility of gene-pseudogen recombinations, trying to study the effect on splicing from the cDNA level, which will benefit the investigation of the genotype-phenotype correlations.

AIM: To study the efficacy and safety of Ultra Q- YAG vitreolysis for vitreous floaters.
METHODS: Retrospective case series study. From September 2014 to May 2015 in Beijing Aier - Intech Eye Hospital, 263 cases (340 eyes) with vitreous floaters were involved. All patients underwent visual acuity, intraocular pressure, slit lamp, mydriatic fundus, B ultrasonic examination, and recorded the form of a vitreous opacity excluded pathological fundus lesions. All the patients were divided into two groups: Group A (<30 years old) 78 cases (82 eyes); the morphology of vitreous opacity were dot, filiform and mesh. Group B ( > 45 years old), 185 cases (258 eyes), the morphology of vitreous opacity was Weiss ring, translucent flocculent clouds or dense fibrous membrane. Patients in 30 - 45 years old were eliminated because of the untypical opacity factor. The treatment was done by the same physician. Vision changes was analyzed before and after the treatment.
RESULTS: Questionnaire survey was done. According to the scores of the questionnaires, patients were divided into 3 groups: no improvement ( 1 - 2 points), partial improvement (3-5 points), significant improvement (6-10 points ). At 1mo after treatment, Group A: no improvement in 9 eyes (11. 0%), partial improvement in 57 eyes (69. 5%) and significant improvement in 16 eyes (19. 5%); Group B: no improvement in 0 eyes, partial improvement in 23 eyes ( 8. 9%) and significant improvement in 235 eyes (91. 1%); all the patients had no complications.
CONCLUSION: The treatment with YAG vitreolysis for vitreous floaters is safe and effective, especially for the patients more than 45 years old whose vitreous floaters caused by posterior vitreous detachment.

Objective:To evaluate the efficacy and safety of dexamethasone intravitreal implant (Ozurdex) in treating the refractory macular edema caused by retinal vein occlusion (RVO).Methods:An observational case series study was conducted.Twenty-one eyes of 21 patients diagnosed as refractory macular edema secondary to RVO and treated with Ozurdex implant in Beijing Aier-Intech Eye Hospital from March 2016 to September 2019, who was with a course lasting longer than 3 months and received at least 2 times of anti-VEGF treatments, had recurrent macular edema and no visual improvement or even deteriorated, were included.Best corrected visual acuity (BCVA) was examined using standard visual chart and was converted to logarithm of the minimal angle of resolution (LogMAR) units and intraocular pressure (IOP) was examined.Optical coherence tomography (OCT) was used to measure central retinal thickness (CRT) in all eyes before and at 1, 2, 3 and 6 months after intravitreal injection of Ozurdex.The changes of BCVA, IOP and CRT before and after Ozurdex injection were observed and analyzed.During the 6-month follow-up, re-injection of Ozurdex or ranibizumab was adopted among those with macular edema recurrence or poor efficacy according to the subjects' conditions.Ocular adverse effects and potential systemic complications were observed.This study followed the Declaration of Helsinki and the study protocol was approved by an Ecthics Committee of Beijing Aier-Intech Eye Hospital (No.BJAIER2020IRB01).Results:The mean CRT at 1, 2, 3, 6 months after Ozurdex injection was (295.76±49.19), (280.33±39.44), (321.29±73.46), (300.29±75.10)μm, respectively, which were significantly decreased in comparison with (458.52±174.61)μm at baseline (all at P<0.05). There was no significant difference in mean BCVA at different time points before and after Ozurdex injection ( F=1.975, P>0.05). During the follow-up, 10 eyes had macular edema recurrence at 2 to 6 months after first Ozurdex injection, with an average of (4.1±1.5) months.Among them, 8 eyes received second Ozurdex injection, and CRT was significantly reduced and BCVA was significantly improved at 6 months after the second Ozurdex injection in comparison with those at recurrence ( t=5.254, P=0.001; t=4.277, P=0.004). The IOP was significantly elevated at 2 months after first Ozurdex injection in comparison with that at baseline ( P=0.01). Ocular hypertension (IOP≥25 mmHg) was oberserved in 3 (14.3%) eyes during the follow-up period but were well controlled after local application of eye drops.No vitreous hemorrhage, retinal detachment, endophthalmitis or other serious adverse effects or systemic complications were observed. Conclusions:One dose of intravitreal Ozurdex injection can significantly improve the structure and function of macula in refractory macular edema caused by RVO for 4 to 6 months and maintain the baseline visual acuity.Second administration of Ozurdex is still effective for recurrent RVO macular edema.Transitional IOP elevation is the main adverse event.

By using the model of ischemia-reperfusion injury in the isolated working rat heart,the change of free amino acid concentrations in myocardium and the effect of taurine on the heart were investegated. It was found that concentrations of 17 free amino acids in myocardium, except Cys,decreased,and that 20mM taurine was able to ameliorate the ischemia-reperfusion injury of the heart ;e. g. , the improvement of recovery of cardiac mechanical functions, the decrease in the leakage of LDHand CK from myocardium,in the accumulation of lipid peroxides and calcium,and in the loss of free amino acids in myocardium,as well as the decrease in the occurence rate of ventricular arrhythmias during the reperfusion phase. So, it may be suggested that exogenous taurine has some protective effects against the myocardial is-chemia-reperfuaion injury.

Complexity science is a new type of scientific research method, and the combination of complexity science, life science, and medicine is still in the initial stage of exploration, and domestic medi-cal colleges do not offer correlative course currently. In order to broaden students' vision, transform their traditional thinking model, and prepare the future of multidisciplinary cooperation, medical colleges should open complexity science and medicine correlative course for senior undergraduate and graduate students. Course content should start by the famous fablebutterfly infect, followed by the introduction of chaos and fractal, self-organization and complex system theory concept, theoretical framework, and their application in the medical field The teaching material should refer to some popular science books, such as Mystery of the butterfly effect. The teaching method should draw lessons from metaphor.

Angiolymphoid Hyperplasia with Eosinophilia ; pathology ; Forehead ; pathology ; Humans ; Male ; Middle Aged ; Parotid Gland ; pathology

BACKGROUND: The brittleness and low flexural strength of ceramic material hinder, to a certain extent, its application in prosthodontic dentistry. Zirconia is enhanced in its flexural strength and toughness by the transformation toughening mechanism, which makes up for the brittleness of the traditional all-ceramic material.OBJECTIVE: To preliminarily explore a new machinable zirconia ceramic material and investigate sintering properties of dental machinable zirconia/La-monazite diphase ceramics with nano-zirconia/La-monazite diphase ceramics. DESIGN: By adjusting the composition and ratio of raw materials, and by adopting different preparation and sintering method, this study was intended to measure the related parameters and to explore the best preparation and sintering method. SETTING: Department of Prosthodontics, College of Stomatology Shanghai Ninth People's Hospital Affiliated to Shanghai Jiao Tong University; Lab of Advanced Inorganic Material Technology, School of Material Science and Engineering, Shanghai University. MATERIALS: There were 3 mol yttria-containing tetragonal zirconia polycrystals (3Y-TZP) (size≤50 nm, purity 99.99%, Yixing Xinxing Zirconia-products Co., Ltd.) and La-monazite (purity 99.99%, Baotou Rare-earth Phosphate Institution). METHODS: Experiments were performed at the Department of Prosthodontics, College of Stomatology Shanghai Ninth People's Hospital Affiliated to Shanghai Jiao Tong University from January 2004 to December 2006. The pilot experiments found that the zirconia with less than 15% of lanthanum phosphate was high in strength but poor in machinability while more than 20% of lanthanum phosphate was decreased significantly in its strength. Therefore, 15%, 18% and 20% (volume percentage) of la-monazite was added to 3Y-TZP. The green bodies of the three groups were compacted by cold isostatic pressing (200 MPa) and were sintered in air atmosphere at different temperatures: 1 560 ℃, 1 580 ℃ and 1 600 ℃ to make the ceramic samples. MAIN OUTCOME MEASURES: The volume density, porous rate (Archimedes method) and three-point bending strength (EZ-100 universal testing machine) were tested of all the ceramic samples.RESULTS: ①With the increase of sintering temperature, zirconia/La-monazite ceramics with 15%, 18% and 20% lanthanum phosphate was increased in its bulk and density. The density was the highest for 1 600 ℃ and the respective density of the three groups were 5.77 g/cm3, 5.42 g/cm3 and 5.39 g/cm3. The porous rate decreased with the increasing temperature and was the lowest at 1 600 ℃ (0.88%, 1.21%, 1.49% respectively). There was no significant difference in volume and density at different temperatures (P > 0.05). ②The flexure strength of diphase ceramic with 18% and 20% lanthanum phosphate increased with the temperature increasing to 1 580 ℃. At 1 580 ℃, the flexural strength reached the highest level, respectively (772.22±43.43) MPa, (216.03±25.20) MPa and (157.21±9.79) MPa. When the temperature reaches 1 600 ℃, the strength was decreased. CONCLUSION: Zirconia/La-monazite diphase ceramics can be prepared by adopting cold isostatic pressing (200 MPa) and sintering at 1 580 ℃.

Objective Through analysed the questionnaire about postpartum contraception knowledge-attitude-practice (KAP) among obstetricians,to understand the postpartum contraceptive knowledge,idea,attitude and related factors among obstetricians.Methods From October 2013 toDecember 2013,209 obstetricians of 10 different levels of the hospital of Tianjin area had been enrolled in this study,and KAP questionnaire was investigated.Results The knowledge of postpartum contraception among obstetricians was seriously lack,the total correct rate was 34.8％,the lowest score was 5 points,the highest was 80 points,the average score was 34.8± 13.5,more than 60 points accounted for only 2.9％ (6/209).86.6％ (181/209) obstetricians have thought it necessary to carry out postpartum contraceptive propaganda during pregnancy,and 97.6％ (204/209) obstetricians have thought it necessary to develop postpartum contraception training.92.8％ (194/209) and 94.2％ (197/209) obstetricians always informed that pay attention to contraception before discharge and postpartum visit,specific methods were not discussed.Obstetricians obtained relevant progress of contraceptive knowledge mainly through the daily medical work (92.8％,194/209),hoped that approach postpartum contraception related knowlege through continuing education courses (77.5％,162/209),special lectures in academic conference (72.2％,151/209) and special training (67.5％,141/209).Conclusions (1) The postpartum contraception knowledge among obstetricians is seriously lack.(2) The obstetricians hold positive attitude towards the propaganda for postpartum contraceptive knowledge.(3) Postpartum contraception related education only provided before discharging and postpartum visit,and the content was simply inform that pay attention to contraception,specific methods are not discussed.(4) To explore the reason why postpartum contraception situation is not ideal,that's because insufficient attention and propaganda,obstetricians don't update the contraception knowledge,and be lack of knowledge on the efficient,long-acting reversible contraception methods,exaggerated the side effects and complications.

BACKGROUND: As a convenient and useful physical activity measurement tool,pedometer is widely used in research and intervention about physical activity of population. OBJECTIVE: To assess validity and reliability of the pedometer in measuring daily physical activity. DESIGN,TIME AND SETTING: The experiment was performed at the Department of Kinesiology,Nanjing Institute of Physical Education and Sports from April to June 2007. PARTICIPANTS: Thirty college students,15 majoring in Kinesiology and 15 majoring in social and physical education,15 females and 15 males,with an average age of (20?1) years,were selected from Nanjing Institute of Physical Education and Sports. METHODS: Participants wore a Digi-Walker200 for one week to record results of pedometer measurement,and filled in Bouchard Physical Activity Diary every day to note activity in 24 hours and calculate energy consumption. All participants completed International Physical Activity Questionnaire including large and moderate intensity activity,walking and sitting in a week. MAIN OUTCOME MEASURES: Daily steps,energy expenditure,sedentary time,waking time,exercise time,and weekly physical activity condition. RESULTS: Step counts were positively correlated with daily energy expenditure,walking time,and exercise time,and negatively related with sedentary time. Step counts of different physical activity levels were significantly different: more step counts could better reflect physical activity levels. Furthermore,exercise or continuous walking (more than 15 minutes) could significantly increase step counts. Daily physical activity including 30-minute moderate intensity activity could achieve or exceed the target steps of 10 000. CONCLUSION: Pedometer provides a useful and objective indicator of daily step counts assessing daily physical activity,but it is difficult to reflect activity intensity and patterns.

Objective To evaluate the effectiveness of mindfulness-based stress reduction( MBSR) on anxiety and depression in spouses of breast cancer patients and to analyze the mediating effect of caregiver burden for the training effects. Methods Randomized control trial was used in this research.100 spouses of breast cancer patients were recruited and they were randomized into experimental group or control group. 91 of them completed the study finally (45 in experimental group and 46 in control group). Participants in con-trol group received no intervention,while participants in experimental group received MBSR once a week for 8 weeks. All participants were assessed with the Zarit Caregiver Burden Interview ( ZBI,measuring caregiver burden) ,Beck Depression Inventory( BDI,measuring depression level) and Hamilton Anxiety Scale( HAMA, measuring anxiety level) before and after intervention. Results After 8-week intervention,participants in experimental group got significantly lower total scores of ZBI,BDI and HAMA compared with pretest ((22.31 ±5.76) vs (25.98±6.00),(8.47±3.21) vs (11.87±3.51),(14.98±4.75) vs (17.93±4.65), P<0. 01), while there were no significant differences between posttest and pretest on any score in control group (P>0.05).Caregiver burden played mediating effect in MBSR for improving depression,and mediating effect was 56.6% of the total effect. Conclusion MBSR can effectively improve anxiety and depression with the spou-ses of breast cancer patients,and the caregiver burden is the intermediary variable in the effect of MBSR for improving depression.