Age-related macular degeneration ( AMD ) is a kind of age-related blinding degenerative fundus lesions, totally about 30 million patients suffering from AMD all over the world, with about 500 000 people blind for it yearly. As the development of economy and the aging of the population intensified, incidence of AMD indicates a trend of rising year by year, being the third major cause of blindness in our country. At present, the pathogenesis of AMD is not fully clear, as reported it may be related to oxidative stress, inflammatory immune response, VEGF and genetic manipulation. Clinical treatments mainly include photodynamic therapy, drug therapy, radiation therapy, laser photocoagulaory operation, the pupil warm treatments, Chinese medicine and intravitreous injection VEGF antagonists such as Ranibizumab, Conbercept and so on. ln this issue, we mainly expound on the progress in the epidemiological studies of AMD, especially elaborate the progress made on genetic manipulation in recent years.

Objective To understand the effect of vanadium to liver ultramicro structure in SD rats.Methods One hundred and fifty generaMevel SD rats of 2 months were randomly divided into control groups,30 in each,the males and females were equivalent,treated with ammonium metavanadate at doses of 0,10,20,40,60 mg/L through drinking water.After 2,6 and 10 weeks of treatment,6 rats from each group were weighed and the changes of ultramicro-structure in the liver were examined with electron microscope.Results As the increasing of dose,the increase in body weight showed a downward trend,but the coefficient of liver showed a increasing trend,the consistent performance was seen in male and female rat.After two weeks of exposure,the rats acted,ate and drank less,hair roughing and no shining,often getting together were seen.After 10 weeks,the rats had not the basic activities,the hair was disheveled and loose,some died,the structure of mitochondria and endoplasmic reticulum was normal in the 10,20,40 mg/L group,the number of endoplasmic reticulum increased,in 60 mg/L group,the rough endoplasmic reticulum vanished,the liver cytoplasm had a swelling phenomenon,the mitochondria reduced.Conclusion Vanadium have little effect on the liver cells of rats in low exposure level,but high level exposure may damage the liver cells in rats.

Objective To study the preliminary effect of sartorius iliac flap in repair of acetabulum defect for developmental dysplasia of the hip (DDH) in elder children.Methods We retrospectively studied 17 elder children with acetabulum defect in DDH at the age＞6 years old who received acetabular repair with sartorius bone flap in this hospital from 2014 to 2016.All patients with acetabulum defect in DDH were treated with open reduction in general anesthesia.Three-dimensional osteotomy under the femur rotor was conducted to correct the femoral anteversion and neck-shaft angle.According to the height of femoral head dislocation lines,femur tuberosity was shorten and fixied with steel plates,joint capsule was reconstructed,The iliac sartorius musculocutaneous flap was took to the upper edge of the acetabular to repair the acetabular defect.The lower extremities were treated with plaster for 6 to 8 weeks after surgery.The hip joint function was trained and the function was evaluated with Mckay's criteria.The hip joint morphological evaluation was performed with reference to Severin's X-ray standard.Results Seventeen patients were followed up for 6 to 29 months.Postoperative reduction of the femoral head was good,the head mortar inclusive was satisfactory;there were no wound infection and femoral head necrosis.According to Mckay's criteria,2 patients got excellent,13 good,and 2 fair,with the fineness ratio at 88.2％;According to Severin's critera:11 patients excellent.Conclusion The overturned sartorius iliac flap can repair acetabulum defect,promote bone healing,and effectively improve acetabulum to cover head of femur.

Objective To study the clinicopathologic characteristics of sarcomatoid carcinoma of the lung.Methods Clinicopathologic characteristics and immunohistochemical results of 42 cases with sarcoma- toid carcinoma of the lung were analyzed.Results Forty-two cases were pathologically proved to be spindle cell carcinoma,1 cases;giant cell carcinoma,1 case;carcinosarcoma,4 cases;pleomorphic carcinoma,36 cases.The tumors were composed of both carcinomatous and sarcomatous elements.Immunohistochemistry showed that CK was positive in 24 of 24 cases,EMA was positive in 18 of 20 cases,Vim was positive in 25 of 25 cases.Eighteen cases were survival One-year survival rate was 61.2 %,with mean survival 13.3 months. Conclusion Diagnosis of sarcomatoid carcinoma of the lung depends on pathologic and immunohistochemical results.

On the basis of the Uncaria transcriptome, specific primers were designed for UrSTR. The full-length cDNA of UrSTR (GeneBank: OL310251) was 1 541 bp, encoding 345 amino acid residues, and the promoter region sequence of UrSTR (GeneBank: OL310252) was 1 179 bp. Phylogenetic tree is revealed that UrSTR had a closest relationship with STR from Ophiorrhiza pumila and Ophiorrhiza japonica. Localization of UrSTR protein is revealed located in the vacuole membrane. Plant-care analysis indicated that the promoter region sequence of UrSTR, covering multiple light, stress and hormone-response cis-regulatory elements, and verified transcriptional activity. The results of SDS-PAGE show that pET-28a-UrSTR recombinant protein was successfully expressed, and the size was anticipated. The UrSTR prokaryotic expression system needs to be optimized in the later stage. The research lays the foundation for further purification to study its structure and functional characterization of the UrSTR protein.

Objective To investigate the clinical outcome and prenatal diagnosis feasibility of fetal congenital cardiac diventiculum.Methods The data of 12 fetuses with congenital cardiac diventiculum were retrospectively reviewed.The prenatal and postnatal medical records,including the characteristics of diverticulum,presence of abnormalities,karyotype and the outcomes of each pregnancy were collected.Results The overall incidence of cardiac diventiculum was 0.03％ (12/40 564) and the rate of incidence between left and right ventricle was 2 ∶ 1.Mean size of diventiculum was (69.75 ± 28.73)mm2,mean diameter of the diventiculum neck was (3.58 ± 0.80) mm and mean thickness of diventiculum wall was (1.54± 0.29)mm.Seven cases (58.4％) as an isolated malformation,5 cases (41.6％) combined with cardiac defect and extracardiac abnormalities,2 cases (16.7 ％) with chromosomal abnormalities.Five cases underwent termination of pregnancy,1 case died in uterus and 6 cases were born live.The mean follow-up periods was (62.33±-36.52)month.Of the 6 follow-up cases,4 cases (66.7％) remained asymptomatic,one case underwent drug therapy because of arrhythmia and one case combined with VSD underwent operation.Conclusions Echocardiography could be an useful tool to demonstrate and monitor congenital cardiac diventiculum prenatally and postnatally.The outcome of cardiac diventiculum depends on the size,progression,and the combined abnormalities and complications.

We report here a Chinese female infant with severe hypoplasia of the cerebellum and pons, and heterozygous mutation (c.18G >T, p.E6D) in the TSEN54 gene. This mutation was not present in her parents and the 100 Chinese controls, which proved to be a de novo missense mutation. MR imaging of the patient revealed severe hypoplasia of the bilateral cerebellar hemispheres and vermis with moderate flattening of the pons. A video EEG during hospitalization demostrated abnormal background activities and generalized burst and attenuation patterns during interictal stage. The spasms and tonic spasms occurred frequently in clusters with generalized voltage attenuation.

Objective To investigate the adverse effects of taurine on rabbit corneal endothelial cells. Methods Six rabbits (12 eyes) were selected, and 6 histologic sections were prepared from each of the eyes. Rabbit corneal endothelial cells were cultured by explant culture method. Cells were innoculated on a 12-well tissue culture plate, 2%, 4%, 6%, 8% and 10% taurine solutions were added respectively (cells from the right and left eyes of the same rabbit were added the same concentration of taurine solution), and blank control was established. The growth of corneal endothelial cells was observed by inverted microscopy, and cell morphology on the 1st, 2nd, 4th, 6th and 8th day of culture was observed with Wright staining. Results Corneal endothelial cells cultured with 2%, 4% and 6% taurine solutions and those of blank control formed endothelial cell layers after culture for one week, and the cells exhibited hexagonal or round-like morphology. Corneal endothelial cells cultured with 8% taurine solution appeared to be undergrowth with small cell body on the 4th day, and cell death occurred on the 8th day. Corneal endothelial cells cultured with 10% taurine solution turned out to be undergrowth with small cell body on the 2nd day, and cell death had occurred. The same growth velocity and cell morphology were observed in the corneal endothelial cells from the right and left eyes of the same rabbit. Conclusion Taurine with concentration between 2% and 6% has no adverse effects on the growth of rabbit corneal endothelial cells.

Objective To study the effects of angiotensin converting enzyme inhibitor benazepri1 on apoptosis and the expression of Fas and FasL in the kidney of rats with adriamycin-indued nephritic glomeruosclerosis.Methods After uninephrectomy and the injection of adriamycin induced rats model with glomerulosclerosis, benazapril(6 mg/kg) was delivered daily by gavage to the rats in therapeutic groups for 12 weeks.Apoptosis was examined by means of terminal-deoxynucleotidyl trans ferase mediated d-UTP nick end label ling(TUNEL) and immunohistochemistry was utlized to detect the expression of Fas and FasL.Software of pathological analysis quantitated the level of Fas and FasL.Results Compared with those of the control group, the kidney of model group had moresevere glomerulosclerosis, much more apoptotic cells and higher level of exprssion of Fas and FasL. The degree of glomeruloscleroais, the nuxner of apoptotic cells and the level of expression of Fas and FasL were ameliofated by benazepril treatment.Conclusion Benazepril may suppress the excessive apoptosis of kidney cell by lowering the expression of the protin correlatng apoptosis Fas and FasL,so as to postpone the process of glomeruosclerosis.

Objective To study the clinical and electrophysiological features of myasthenia gravis (MG)accompanied by myogenic lesion.Methods The data of the patients who were diagnosed MG accompanied by myogenic lesion from 1998 to 2006 were collected in EMG Room, Department of Neurology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and were retrospectively analyzed.Results In this group of 53 patients, myogenic lesion was found more often in patients with early-onset MG than those with late-onset(69.81% vs 30.20%), and among the early-onset patients the frequency of female was significantly higher than male(26 vs 11,X~2=5.281, P