ObjectiveTo observe the hereditary types and clinical characteristics of 137 patients with retinitis pigmentosa (RP) in Ningxia.MethodsOne hundred and thirty-seven patients with RP who diagnosed by the examinations of visual acuity, optometry, direct or indirect ophthalmoscope, visual field,optical coherence tomography (OCT) and electroretinogram were enrolled. The hereditary types and clinical characteristics were analyzed according to the family history and the results of ophthalmologic examinations.ResultsOne hundred and thirty-seven patients included 29 autosomal dominant RP (ADRP) patients from 8 families (7.4％), 16 autosomal recessive RP (ARRP) patients from 15 families (13. 9％), 10 X-linked RP (XLRP) from 3 families (2.8％), and 82 simplex RP (SRP) patients (75.9％).There were 15consanguineous marriage families out of 26 families with RP history (57.7 ％). The patients were classified as typical RP (102 patients, 74.5％) and atypical RP (35 patients, 25.5％). All the ADRP and XLRP patients showed typical clinical features of RP. Ten (62.5％) of ARRP patients and 53 (64.6％) of SRP patients had typical features of RP. Six (37.5％) of ARRP patients and 29 (35.4％) of SRP patients had atypical features of RP. Among atypical RP patients, 17 (48. 6％) patients were non-pigmented RP which including 3 patients were misdiagnosed as amblyopia during childhood. The logarithm of minimal angle of resolution (logMAR) best corrected visual acuity (BCVA) of ADRP patients was 1.04±0.51 at the age older than 51 years, while the BCVA of ARRP and XLRP patients were 0. 92+0. 61 and 1. 70±0. 02 respectively at 21 to 30 years of age. One hundred and twenty-three (89. 8%) patients suffered from varying degrees of myopia. OCT showed that the average thickness of macular fovea in ADRP patients was ( 185. 73 + 1. 23) μm at the age older than 51 years, while in ARRP and XLRP patients were (173. 21 ± 0. 98) and (170. 49+1. 15) μm respectively at 21 to 30 years of age. Conclusions ADRP and XLRP are typical RP. All atypical RP are ARRP and SRP. Non-pigmented RP are mainly seen in atypical RP which often misdiagnosed as amblyopia during childhood. The photoreceptors in macula are damaged in the early stage and the decline of visual acuity occurred at 21 to 30 years of age in patients with ARRP and XLRP. The ADRP patients has late slower decline of visual acuity and retain some visual acuity at the age older than 51 years.

Objective To investigate the prevalence of fatigue in AS and its relationship with other clinical and functional parameters used for the evaluation of disease activity. Methods The fatigue was assessed in 73 patients with active AS using a single-item fatigue question of BASDAI. The correlation between fatigue and the clinical parameters of disease including the Bath AS disease activity index(BASDAI), Bath AS functional index (BASFI), Bath AS metrology index(BASMI), night pain, spinal inflammation, patient's global assessment(PGA) and enthesis index(EI) were determined. Results A total of 73 patients with ankylosing spondylitis were recruited. The prevalence of fatigue was 90.4% and the fatigue score of 68.5% patients was higher than 5. After controlled for the possible confounding factors such as age, gender, age and disease duration, it was found that fatigue was highly correlated with disease activity, pain and functional disability, BASMI,physical health and mental health. Conclusion Fatigue is an important symptom in AS. Fatigue can be measured with a single-item fatigue question of BASDAI.

Objective: To summarize the recent research progress in the influence of quercetin on drug metabolizing enzymes. Methods:By referring to the relevant literatures at home and abroad in recent years, the paper summarized, analyzed and concluded the the influence of quercetin on drug metabolizing enzymes. Results: Quercetin could modulate the phase Ⅰmetabolic enzyme cyto-chrome P450 ( CYP) and the phase Ⅱ metabolic enzymes uridine diphosphate - glucuronosyltransferase enzyme ( UGTs) , sulfotrans-ferase ( SULTs) and glutathione S-transferase ( GSTs) to influence the in vitro and in vivo metabolism of a lot of drugs. Meanwhile, the modulation of quercetin on the metabolic enzymes demanded the participation of various nuclear receptors. Conclusion:Quercetin shows the inhibitory or inducing effect on a variety of drug-metabolizing enzymes, therefore, it can interact with other drugs.

Objective To investigate the comparison of the clinical treatment efficacy of propafenone and amiodarone in patients with paroxysmal supraventricular tachycardia ( PSVT ) .Methods Retrospective a total of 89 patients with PSVT from June 2013 to June 2015 in our hospital were selected.According to the different administration methods, the patients were divided into the propafenone group of 46 cases and the amiodarone group of 43 cases.The changes of left ventricular end-systolic volume, left ventricular end-diastolic volume, ejection fraction, cardioversion success rate, mean cardioversion time, heart rate and side effects were compared.Results After treatment, the left ventricular end-systolic volume (52.31 ±8.34) mL/m2 , (54.28 ±7.23) mL/m2, left ventricular end-diastolic volume (47.31 ±8.56) mL/m2, (45.28 ±7.16) mL/m2 and ejection fraction (61.57 ± 0.76)%, (61.39 ±0.69)% were without significant differences.After treatment, the heart rates of the two groups were significantly decreased, the propafenone group ( 85.31 ±12.84 ) times/min, amiodarone group ( 87.26 ±11.95 ) times/min, and there were not significant differences.The recovery time of propafenone group was ( 19.34 ±4.76 ) , higher than the amiodarone group ( 25.69 ±6.09 ) ( P <0.05 ).But the success rate of cardioversion (52.17%) was lower than the amiodarone group (88.37%) (P<0.05).The side effect rates in the propafenone group (21.74%) was significantly higher than that in the amiodarone group ( 4.65%) ( P <0.05 ) .Conclusion Propafenone and amiodarone cardioversion PSVT have achieved good results, but propafenone rapid onset, the average cardioversion time is short, which is only suitable for acute tachycardia and without serious organic heart disease patients.Amiodarone onset is slower,but the cardioversion success rate is higher.

Objective In this study, the best domestic digestive departments' websites are evaluated, adopting the meth-od of cross-sectional descriptive research, to evaluate the quality of such sites, and describe their characteristics. Methods Using a pre-defined evaluation standard, two well-trained evaluators independently explore and analyze 10 websites of domestic best departments of digestive diseases. Selection of departments is based on a domestic au-thoritative released rank of the best departments which has high credibility. Results These sites perform well in the content, perform in function and design, but perform not well in management & maintenance. Conclusion As the public use the websites of professional websites more and more to obtain information and services, domestic best di-gestive departments should better their sites' quality to meet the growing demands of consumers.

Objective To assess the usefulness of contrast echocardiography in differential diagnosing space-occupying lesions of the heart.Methods Contrast echocardiography was assessed for 38 patients of space-occupying lesion of the heart (SOLH).Contrast enhancement of SOLH was assessed visually,comparing with that in the adjacent myocardium:significant contrast enhancement,intensity higher than or equal to the adjacent myocardium; mild or partial contrast enhancement,intensity lower than the adjacent myocardium,or no contrast enhancement.Respectively,malignant tumors,benign tumors,and thrombus were diagnosed.Twenty-eight patients among the 38 cases had gold standard diagnosis.The consistency between visual assessment diagnosis and gold standards were compared with Kappa statistic.Results Visual semi-quantitative diagnosis of contrast echocardiography:thrombus,benign tumors and malignant tumors were diagnosed for 8,8 and 12 cases,separately.Except two highly vascular benign tumors (1rhabdomyoma and 1 paraganglioma) were misdiagnosed as malignant tumors,the rest 26 cases matched gold standard diagnosis.Quantitative analysis of contrast echocardiography,16 cases were suitable for quantitative an alysis,10 cases were diagnosed as benign tumors or thrombus (quantitative analysis could not differentiate the two),the rest 6 cases were diagnosed as malignant tumors.Quantitative analysis got the same 2 misdiagnosis as visual semi-quantitative diagnosis.Kappa value on visual semi-quantitative diagnosis of contrast echocardiography comparing with gold standard diagnosis was 0.892.Conclusions The characteristics of vascularity of SOLH can be accurately determined by the contrast enhancement level in diseased region through visual semi-quantitative analysis with contrast echocardiography.Contrast echocardiography can be mainly used in SOLH except thrombus that has been clearly diagnosed,especially for patients with on going or fore-past malignancy in other organs.

Objective To assess the aorta elastic properties in the procession of atherosclerosis by ultra‐high frequency ultrasound ,and to detect the relationship between the aorta elastic properties and the atherosclerotic plaque burden .Methods Mice deficient for the apolipoprotein E (ApoE‐/‐) with high‐cholestrol diet were studied as an age‐dependent model of atherosclerosis .At 8 ,16 ,24 and 32 weeks of age , the blood pressure in the ascending aorta was measured by catheter ,and the aorta mechanical properties were assessed by measuring aortic elastic modulus of the ascending aorta with ultra‐high frequency ultrasound .The plaque burden was assessed by high‐frequency ultrasound and Masson′s trichrome stain , separately .Results Vessel thickness at the lesion‐prone sites of the lesser curvature of the aorta and the proximal brachiocephalic artery increased with age ,consistent with the Masson′s trichrome staining which showed age‐dependent worsening of atherosclerosis in the mice model .Elastic modulus of the aorta significantly increased from 8 to 32 weeks of age in E‐/‐mice .There was a statistically difference between any two groups .Conclusions With the progression of atherosclerosis and the increased plaque burden ,aorta mechanical properties deteriorated in Apo E‐/‐mice .Ultra‐high frequency ultrasound was a potential tool for assessment of plaque burden and aorta mechanical properties in mouse model .

Objective To evaluate effect of Qi-ming granule on vision function of patients with mild, moderate non-proliferative diabetic retinopathy ( NPDR ) .Methods Using the randomized double blind and placebo-controlled clinical trial method.From Oct.2012 to Jun.2014, 36 patients with 68 eyes who included in the standard were randomly divided into treatment group,control group,given Qi-ming gran-ule and placebo respectively for 6 months, exam with mfERG before treatment and after treatment, evaluate the curative effect.Results After 6 months treatment of Qi-ming granule, the implicit time of the 4th ring of the N1 wave about the mfERG was shorter than that of preoperative( P <0.05), the amplitude density was more than both that of preoperative and control group( P <0.01), the amplitude density of the 5th ring increased compared to that of control group;The peak latency of 3 rd ring of P1 wave was shorter than that of control group( P <0.01), the amplitude density of 1st,3rd,5th ring restored compared with that of preopera-tive and control group( P <0.01), the amplitude density of 4th ring improve than that of control group( P<0.01).Conclusions Qi-ming granule can improve the multifocal ERG of patients with mild, moderate non-proliferative diabetic retinopathy, restore the vision function.

Bone Marrow Neoplasms ; diagnosis ; Humans

Objective To evaluate the microcirculation abnormalities in the patients with hypertrophic cardiomyopathy(HCM). Methods Twenty patients with HCM and 20 healthy volunteers were included in the study. Two-dimensional images on the apical four-chamber, two-chamber and long-axis views, including 5 cardiac cycles before 'flash' and 15 cardiac cycles after 'flash', were reeorded and stored for off-line analysis in EchoPAC workstation. Then,contrast time-intensity curves were obtained for each available left ventrieular segment. Results There were significant differences in the A,k and the product A×k between the hypertrophic wall segments (P = 0.01, P<0.001, P = 0.036,respectively) and nonhypertrophic wall segments (P<0.001,respectively) and healthy subjects. A,k and the product A×k in hypertrophic wall segments were significantly different from those in nonhypertrophic wall segments in patients with HCM (P = 0.021, P = 0.016, P = 0. 001,respectively). Accordingly,the normalized value of A and the product A × k were lower in hypertrophied wall segments (P = 0.031, P<0.001, respectively) and nonhypertrophied wall segments(P = 0.002, P<0.001,respectively) in patients with HCM than those in normal segments of healthy controls. The normalized value of A and the product A × k were lower in hypertrophic wall segments than nonhypertrophic wall segments in patients with HCM(P = 0.045, P = 0.021,respectively). Conclusions Myocardial contrast echocardiography is a useful method to investigate myocardial perfusion abnormalities in patients with HCM.