Retinitis pigmentosa (RP) is a common sight-threatening eye disease.RP is characterized by highly genetical and phenotypical heterogeneity.Identification of the causative genes of RP is the first step toward the understand of the molecular basis of RP and,subsequently,toward the prevention and treatment of RP.In recent years,there have been new progress in the study on RP.This review mainly focuses on recent advances in the gene-related studies of autosomal dominant RP (adRP) and autosomal recessive RP (arRP) with emphasis on the roles of common genes,as well as their possible mechanisms in RP in order to provide the essential reference for the genetics research of RP.

Objectives To study the effect of cooling blood and invigorating circulation method on prevention and treatment of pulmonary fibrosis,and explore its mechanism.Methods Male ICR mice were randomly divided into 4 groups:negative control group,pulmonary fibrosis model group,cooling blood and invigorating circulation group and prednisone positive control group.The pulmonary fibrosis model of mice were established by BLM nasal instillation.After 8 hours,the mice were given prescription of cooling blood and invigorating circulation(Xijiao Dihuang decoction) by gastric perfusion.At the 28th day after treatment,the indexes were detected.The TGF-?1 content of bronchoalveolar lavage fluid(BALF) was tested by enzyme linked immunosorbent assay,and hydroxyproline(HYP) was examined by alkaline hydrolysis.Results The degree of airsacculitis and fibrosis in model group are obviously higher than the negative control group by pathological observation.Compared with model group,HYP content was decreased obviously in herb group(P

Objective To explore the mechanism of QHR (main ingredients:Gypsum, Anemarrhema Asphodeloides Bge, Paeonia Lactiflora Pall) on secretive function of vaso-endothelial cells in rabbits with of endotoxemia. Methods Endotoxemia model was induced by colibacillary endotoxin via auricular intravenous injection in rabbits. The changes on pathomorphology and secretive function of vaso-endothelial cells in the model and QHR group were observed. Results It showed the existence of microthrombus in the lung via microscope in the model. The balance destruction of t-PA/PAI, NO/ET, TXB2/6-K-PGF1? in the model (P

AIM: To demonstrate the susceptibility of cell apoptosis varies during the progress of cell malig- nant transformation from human being in vitro. METHODS: A SV40T - transfected human bronchial epithelial im- mortalized cell line (called M) was selected in this work, which has acquired some characteristics of malignant trans- formation at the later passage. The alterations of apoptosis and bcl- 2, P53 genes between early and later passage of M cells were investigated by means of TDT labeling in situ, chromosome FISH, RNA and protein testing, etc. RE- SULTS: Incidence of apoptosis induced by cis - platin was significantly lower in later than in early passages of M. Levels of bcl - 2 mRNA and protein in later passages were higher than early passages of M, and overxpression of bcl -2 was accumulated following the development of cellular malignancy. P53 protein level was as high in early as in later passages. CONCLUSION: Overexpression of bcl - 2 decreases the cellular sensitivity to apoptotic inductors plays an important role during progress of carcinogenesis in human bronchial epithelial cancers. The inactivation of P53 protein in the SV40 - T transfected M cell line may be one of reasons of bcl - 2 overexpression, but not associated with the accumulation of bcl - 2 expressed level during cell transformation.

Sulfated polysaccharide from Porphyra haitanensis showed inhibitory effect on the lipid peroxidation in vitro. In the present study, the changes in the antioxidative enzyme activity, lipid peroxidation, and total antioxidative capacity (T-AOC) in different organs after 60 Co irradiation were detected in mice by using biochemical methods. Increased endogenous lipid peroxidation and decreased contents of T-AOC as well as decreased activities of super-oxide dismutase (SOD) and glutathione peroxidase (GSH-Px) were observed in mice after 60Co irradiation. Intraperitoneal administration of polysaccharide fraction F2 significantly decreased the lipid peroxidation. F2 treatment increased T-AOC and the activities of SOD and GSH-Px in all organs tested in 60Co irradiated mice. It is concluded that the sulfated polysaccharide fraction F2 from Porphyra haitanensis can be used in compensating the decline in antioxidative capacity arising from radiotherapy and thereby reduced the risks of lipid peroxidation.

Objective To study the co-relation of radiological and histopathological features of lung cancer. Methods X-ray CT and histopathological features of 36 cases of lung cancer were analyzed retrospectively. Results High-density mass with strengthened edge was found to be paralleled with lung atery at lung gate in 6 cases. As a result, bronchial tubes became narrow with mucus in it. Squamous carcinoma tissues of 22 cases were showed growing within bronchial tubes. Local lung emphysema in early stage obstructive pneumonia and 19 cases of lung collapse in mid/late stage(86.4%) were brought out because of narrow cavity of bronchi. Megaly lymph nodes were seen by mediastina window in 12 cases. Peripheral mass with leaf-like edge were found in 8 cases,and the diameter of each was less than 2cm,while irregular deviated cavitative was seen in one case. Conlusion It is suggested that close combination of X-ray CT and histopathological can improve the accurate rate of diagnosis in lung cancer.

Objective To evaluate the effects of continuous intra-tracheal gas insufflation (TGI) during mechanical ventilation for protecting the juvenile piglets with acute lung injury (ALI) induced by endotoxin. Method Twelve healthy juvenile piglets were anesthetized and mechanically ventilated at 2 cmH2O PEEP with 10 cmH2O peak inspiration pressure. The piglets were challenged with lipopolysaccharide (LPS) and randomly (random number) assigned to two groups (n = 6 each): (1) piglets treated with mechanical ventilation alone (group MV) and (2) piglets treated with TGI by continuous airway flow of 2 L/min (group TGI). FiO2 was set at 0.4 to avoid oxygen toxicity, and the piglets were continuously monitored with an oxygen analyzer. Results Tidal volume, ventilation efficacy index and mean airway pressure were significantly improved in piglets of TGI group (P < 0.01 or P < 0.05). Four hours after ALI, pH decreased to below 7.20 in piglets of MV group, and was higher in piglets of TGI group (P < 0.01). Similarly, PaCO2 was stable and was significantly lower in piglets of TGI group than that in piglets of MV group (P < 0.01). PaO2 and PaO2/FiO2 increased in piglets of TGI group (P < 0.05). There were no significant differences in heart rate, respiraaatory rate, mean arterial pressure, central venous pressure, dynamic lung compliance and mean resistance of airway between two groups. Lung histopathological changes showed severe inflammation,and intra-alveolar hemorrhage and interstitial patchy hemorrhage were ameliorated and the lungs were more homogenously expanded in piglets of TGI group. Conclusions Continuous TGI during MV can significantly improve gas exchange and ventilation efficacy, and may provide a better treatment for acute lung injury.

BACKGROUND:Traditionally, forced expiratory volume in one second (FEV1) was used to evaluate exercise response of patients with asthma; however, patients obviously had panting after exercise, so FEV1 was affected commonly. Impulse oscillometry (IOS) is a new technique for measuring respiratory impedance that do not require maximal inspiration and forced expiration.OBJECTIVE: To study airway resistance with IOS before and after exercise in healthy and asthmatic patients and investigate the significance of exercise excitation and IOS assessment.DESIGN: Synchronically non-randomized case contrast study.SETTING: Department of Respiratory Medicine, East Hospital Affiliated to Tongji University.PARTICIPANTS: A total of 14 male patients with bronchial asthma who were regarded as the asthmatic group were selected from Department of Respiratory Medicine of Shanghai East Hospital from January to October 2006. They were in a clinical stationary phase. Another 14 male healthy subjects were selected as the control group and ages of all subjects ranged from 29 to 50 years. All subjects provided the confirmed consent.METHODS: IOS was used to measure basic value of respiratory resistance, and then subjects underwent exercise challenge. Nose of subjects was clipped breathing through mouth. Within 3-4 minutes, heart rate was increased to 90% and maintained for 6 minutes during challenge. Respiratory resistance was repeatedly measured at 1, 5, 10, 15 and 20 minutes after exercise, including airway hyperresponse (AHR), total respiratory resistance, central resistance, peripheral resistance and resonance frequency at 5, 20 and 35 Hz of pulse frequency, elasrtic resistance and inertia resistance (X5 and X35) at 5 and 10 Hz of pulse frequency. In addition, difference of AHR at 5 and 35 Hz was calculated, and change ratios of both Rcentral and Rperipheral were calculated as (highest value after exercise-baseline value)/baseline value × 100%.MAIN OUTCOME MEASURES: Basic value of respiratory resistance by using IOS and exercise challenge test.RESULTS: All 14 patients with bronchial asthma and 14 healthy subjects were involved in the final analysis. Peripheral resistance (Rperiphera) was significantly higher than central resistance (Rcentral) in asthmatic patients (P ＜ 0.01). The maximal increase of respiratory impedance occurred from 5 minutes to 10 minutes after exercise in asthmatics. Resonance frequency (Fres) of asthmatics before and after exercise was significantly increased than that of controls (P ＜ 0.01).Change ratios of Fres from asthmatics were higher than that from control group (P ＜ 0.01). After challenge, R5, R5-R20,Zrespir and X5 from asthmatics changed significantly than that from controls (P ＜ 0.01). The increment change value of After exercise Zrespir increased significantly, because obstruction of small bronchi during expiration and impedance increased abruptly. Air trapping was expressed in VT-Zrespir graph in 57.1% patients. There was no difference in the VT-Zrespir graph of controls before and after exercise.CONCLUSION: The main site of airflow obstruction was in small airways in asthmatics after exercise challenge. The general acceptance of IOS method was good among the asthmatic patients. The airway response of exercise challenge may be assessed more accurately with IOS that do not require a maximal inspiration and forced expiration.

Objective To discuss the application effect of zero OT-U type bionics urine bag in neurology critically ill patients to reduce the indwelling catheterization complications. Methods A total of 50 cases of critically ill patients with indwelling catheter were chosen from July 2014 to May 2015. They were randomly divided into the experimental group and the control group with 25 cases in each group according to the time of admission. The control group used common urine bags and the experimental group adopted bionics urine bag. The excessive urine, urinary tract infection, incontinence associated dermatitis, the time with indwelling urinary catheter,replacing the urine tube to urinate in two groups were compared. Results The rate of excessive urine, urinary tract infection, incontinence associated dermatitis and the time with indwelling urinary catheter in the experimental group were 12%(3/25), 4%(1/25), 32%(8/25) and (10.120 ±3.295) days, while the results were 84%(21/25), 48%(12/25), 80%(20/25) and (28.040±9.480) days in the control group, the differences were different (χ2=27.931,7.741, 7.018, t=8.927, P<0.01). The cases of voluntary micturition, induced micturition and secondary catheterization were 17, 5 and 3, and the results in the control group were 3, 12 and 10, the differences were were different ( χ2=16.452, P < 0.01). Conclusions The zero OT-U type bionics urine bag has a bigger superiority in reducing the related complications in neurology critically ill patients with indwelling catheter. At the same time , the zero OT-U type bionics urine bag reduced the patient's pain, improved the family satisfaction and reduced the pressure on care and treatment.

Objective To investigate the family burden of patients with severe mental illness. Methods 148 patients with schizophrenia and bipolar disorder were assessed with Family Burden Scale of disease (FBS). Results Family burden broadly exists among those patients with schizophrenia and bipolar disorder, and the burden tends to involve several dimensions, including family relations (51%), home entertainment (50%), daily routine (45%), economic burden (43%), mental health of family members (36%) and physical health of family members (32%). Conclusion Integrated psychological and social intervention should be made to reduce the family burden of patients with schizophrenia and bipolar disorder.