Objective To explore the clinical and genetic characteristics of congenital generalized lipodystrophy (CGL). Method The clinical data of one child with CGL caused by BSCL2 gene mutation were analyzed retrospectively and relative literature were reviewed. Results A 2-year-9-month old girl had clinical manifestations of a lack of subcutaneous fat, acanthosis nigricans, hepatolienomegaly and mild hypophrenia. Laboratory examinations showed hypertriglyceridemia, hyperinsulinemia and cardiomyopathy. The peripheral blood from the child and her parents were collected and 4 genes, AGPAT2, BSCL2, CAV1 and PTRF, were sequenced by Sanger. The results showed a heterozygous mutation of BSCL2 gene from maternal frameshift (c.567-568delGA, p.E189EfsX12) and paternal nonsense mutation (c.565G>T,p.E189X) respectively in the child, and both mutations were pathogenic ones. By a literature review, it is known that BSCL2 gene mutation is the most common cause of in Asian. In CGL with BSCL2 gene mutation, the commom clinical manifestations include disappearance of systemic adipose tissue, acathosis nigricans and hepatomegaly, and the incidence of myocardial infarction and mental retardation were 40% and 30% respectively. Conclusion The main clinical manifestations of CGL caused by BSCL2 gene mutation were loss of systemic adipose tissue and metabolic disorder at an early age. It was often accompanied by myocardial lesions and mental retardation. Gene diagnosis analysis should be made as earliest possible time for the children suspected of this disease.

Objective To investigate and analyze the data of continuing medical education in order to understand the real need of the learners.Methods Reviewing and making statistic analysis of the data of Renji hospital's continuing medical education courses from 2007 to 2012,including the amount,title,discipline,geographical distribution,training content and the questionnaire survey.Results It shows that the learners from coastal and developed areas have a higher demand for cuttingedge technology and the new progress in medicine(49.1％),while the learners from inland and western regions prefer to learn the basic medical theory and technology (41.9％).Senior doctors tend to study cutting-edge technology and new progress medical courses (51.7％) while the junior persons prefer the basic medical knowledge (46.6％).These mean that the needs of content of continuing medical education is associated with the regions and the level of the doctors (P＜0.05).Conclusion The conclusions prompt that medical development between different regions of China may be unbalanced.Chinese government should pay more attention in terms of allocation of health resources and personnel training.It also provides advice for future courses so that it may improve the efficiency of the medical education resources.

Objective To evaluate the method of controlled cortical impact(CCI) on long term cognitive disorder after traumatic brain injury(TBI) and to investigate the possible pathological mechanism.Methods Sixty male SD rats were randomly assigned into 3 groups:sham surgery group(n =10),control group (n =10) and CCI group(n =40).CCI application was used to make the bilateral frontal lobe controlled cortical impact model (depth:1.5 mm,velocity =3.5 m/s,dwell time =400 ms).Morris water maze test and Nissl's staining was used to assess the cognitive function and pathological changes after 8 weeks of CCI.The expressions of brain derived neurotrophic factor (BDNF) and tyrosine protein kinase B (TrkB) mRNA in frontal lobe and hippocampus tissue was detected by reverse transcription polymerase chain reaction(RT-PCR).Results The mortality in CCI group was only 12.5％.Morris water maze test results showed the escape latency in CCI group was longer than that in sham surgery and control groups(F =51.784,P ＜ 0.05).Percent of time spend in goal quarter during probe trial in CCI group was significantly less than that in sham surgery and control groups(F =13.468,P ＜ 0.05).Nissl's staining showed frontal lobe had obviously defects; Nissl's bodies of frontal cortex and CA1 region in hippocampus reduced.The expressions of BDNF and TrkB mRNA in frontal lobe and hippocampus were significantly less than those in sham surgery and control groups(P ＜ 0.05).Conclusions The CCI model can be applied for study on long term cognitive disorder after TBI with good stability and repeatability.Using the experimental parameters of CCI can damage the long term cognitive function after TBI in rats,and lead the pathology changes of brain tissue clearly.This may have some relationship with the expressions of BDNF and TrkB mRNA.

Objective To investigate the effect of the revision of Chinese Versions of Reading the Mind in the Eyes Test(RME) on the diagnosis of mild cognitive impairment (MCI) and mild Alzheimer's dementia (AD).Methods We compared performance on the cognitive test battery between 96 patients with MCI and 55 patients with AD and 95 control individuals.Neuropsychological tests, encompassing RME-T, Mini Mental State Examination (MMSE) and Auditory Verbal Learning Test (AVLT), were conducted on 96 patients with MCI, 55 patients with AD and 95 healthy control individuals.Completion rate of RME-T in the normal group and the total scores and each item score of RME-T were analyzed, and the feature between RME-T and RME-S (RME-short) was compared.Results The total score of RME-T was (23.7±4.7) in normal group, (19.9 ± 4.9) in MCI group,and (19.9±7.5) in AD group.There were significant differences in the total score of RME-T among the three groups (t=13.9, P＜0.01).The differences in the total score of RME-T between normal group and AD group, between normal group and MCI group were also significant (both P＜0.01).RME-S was composed of 20 items after rejecting 16 items of RME T whose project completion rate was lower than 50％ in the normal group and in which there was no significant difference among normal, MCI and AD groups.The correlations between the total score of RME-T and 3 item scores of RME-T were not significant (P＞0.05).However, the total score of RME-S was highly correlated with each item score of RME-S (P＜0.01).The total score of RME-T and RME S were highly correlated with each total score of the cognitive test battery (P＜0.01).With the normal and MCI groups, normal and AD groups as the subjects, the receiver operating characteristic (ROC) curve analysis found that AUC(RME-S) was＞ AUC(RME-T) in normal and AD groups (P＜0.01), whereas, AUC(RME-S) was＜AUC(RME-T) in normal and MCI groups (P＞0.05).Conclusions RME has good reliability and validity, and RME-S is better than RME-T in internal consistency, which is more suitable for identifying mild cognitive impairment and Alzheimer's disease in Chinese population, and it is worthy of further popularization and application.

Objective To study the effects of ultramicro propolis powder on reducing blood lipid and regulating lipid metabolism of mice.Method Hyperlipemia model was induced in mice to observe the effect of ultramicro propolis powder on serum and liver lipid levels,their antioxidant function and liver index.Results Ultramicro propolis powder could significantly reduce serum total cholesterol,triglyceride and arteriosclerotic index,increase high-density lipoprotein-choleslerol concentration,reduce liver index and malonaldehyde content and improve hepatic superoxide dismutase activity.Conclusion Ultramicro propolis powder can regulate lipid metabolism and enhance the antioxidant function in hyperlipemic mice.

Objective:To develop a new preparation process of PLGA microparticles for protein drugs by SPG membrane emulsifi-cation combined with W/O/W double emulsion-solvent technique. Methods:Lysozyme was used as the model drug to prepare the mi-croparticles. The influence of formula factors on the properties of the microparticles was studied, and the physicochemical properties, in vivo compatibility and degradation of the microparticles were investigated as well. Results:The drug loading of lysozyme-loaded mi-croparticles was 35%, the entrapment efficiency was 72. 43% and the average size was 63. 89 μm with PDI of 0. 675. DSC and FTIR showed that lysozyme was entrapped in the microparticles. The microspheres had promising biocompatibility and sustained degradation in vivo. Conclusion:The paper describes a new satisfactory preparation process of PLGA microparticles for protein drugs with good in vitro and in vivo properties.

Neurogenesis is a process in which the neuronal stem cells differentiate into functional neurons including the cell proliferation,differentiation and migration.Previously,it was believed that neurogenesis is a prenatal process and the adult ependymal cells are incapable of regeneration.Now it is clear that mammalian brain retains the ability to generate new ceils in specific regions.One of the regions is subventricular zone of the lateral ventricles,new generated neurons and glial cells later migrate to olfactory and repair dysosmia through the RMS road.Here we will review the advances in adult neurogenesis in mammal subventficular zone.

BACKGROUND:Inflamed dental pulp stem cells are a new kind of dental pulp stem cells, and there is no systematic review on the cells by now. OBJECTIVE:To systematical y review the research progress in inflamed dental pulp stem cells. METHODS:A computer-based online search in PubMed, Web of Science, CNKI, WanFang and VIP databases was performed for related articles published from the establishment of the databases to February 2014. The keywords were“(pulptis or inflam*dental pulp*or human dental pulp with irreversible pulpitis) and stem cel*”in English and Chinese, respectively. Hand searching was also done to obtain further information or papers about the studies. The results were qualitatively analyzed to comprehensively summarize the progress in the research of inflamed dental pulp stem cells. RESULTS AND CONCLUSION:Total y 11 papers were involved in result analysis that comprehensively review the research progress in inflamed dental pulp stem cells at the fol owing aspects:the research of history, material origin, cellculture, cel-surface markers, proliferation ability, multi-directional differentiation potential, animal models and clinical use. Researches of inflamed dental pulp stem cells are stil in the initial stage, and cultivating conditions and the establishment of animal models are stil in the exploratory phase. Controversies stil exist in the capacity of proliferation and multi-directional differentiation of the inflamed dental pulp stem cells. And fewer studies have been done in the characteristics of immunity, subpopulation and clinical use of the inflamed dental pulp stem cells.

Objective To investigate the value of FIESTA sequence for the abdominal scanning of the patient undergoing uneffective respiratory triggering. Methods 40 cases undergoing uneffective respiratory triggering were adopted as the subjects. GE SIGNA 1.5T and fat-suppressed technique got involved in. Two chief physicians and two associate chief technologists compared the image quality and the lesion detective rate. Results FIESTA sequence was better than FSE T2WI in the aspect of image quality, but worse in lesion detective rate. Conclusion FIESTA sequence is clinically valuable for the abdominal scanning of patient undergoing unffective respiratory triggering.

Objective To evaluate advantages and disadvantages of different pacing modes of cardiac resynchronization therapy (CRT).Methods Twelve dogs with heart failure were performed in every dog at random,and the pacing modes employed in the test included right atrium-different sites of ventricle,and ventricular sites included right ventricular bifocal (RV-Bi),biventricular (Bi-V),left ventricular (LV).The pacing frequency was 180 times per minute,and the results were measured before pacing and after 15 minutes when the pacing became stable in Color Doppler echocardiography,including left ventricular enddiastolic diameter (LVEDd),left ventricular ejection fraction (LVEF),interventricular mechanical delay (IVMD),interventricular septum and left ventricular posterior wall motion delay (SPWMD),left ventricular 12-segment peak time standard deviation (Ts-SD).Results (1)Compared with before pacing,at the RV-Bi,Bi-V,and LV pacing modes,LVEDd,IVMD,SPWMD,and Ts-SD decreased,LVEF increased,the difference was statistically significant [(42.42 ± 3.94) mm vs (34.00 ± 4.07) mm,(34.17 ± 3.95)mm,(33.75 ±4.18)mm; (28.08 ±4.01)mm vs (13.00 ±3.64) mm,(11.95 ±2.54)mm,(12.08 ±3.51) mm; (75.00 ± 10.22)mm vs (51.75 ±9.84) mm,(20.66 ±7.41) mm,(20.75 ±7.56) mm; (25.08±4.16)mm vs (14.91 ± 3.31)mm,(7.50 ±4.24) mm,(7.41 ±3.39)mm;(32.91 ±4.46)mm vs (41.50 ±4.16)mm,(42.00 ±4.63) mm,(42.41 ±4.99)mm,P ＜0.05].(2)Compared with RV-Bi pacing mode,at the Bi-V,LV pacing modes,SPWMD and Ts-SD decreased,the difference was statistically significant(P ＜ 0.05); there was no significant difference among LVEDd,IVMD,and LVEF (P ＞0.05).(3)There was no significant difference in LVEDd,IVMD,SPWMD,Ts-SD and LVEF between LV and Bi-V pacing (P ＞ 0.05).Conclusions The hemodynamic effects of RV-Bi and LV pacing modes were similar to that of Bi-V pacing,and they can be used as CRT biventricular pacing alternative modes; however,the mechanisms of improving ventricular synchronization are not identical in above pacing modes.