Alagille syndrome (ALGS) is the most common cause of the phenotypic characteristics of chronic cholestasis disease.It is an infrequent multisystem autosomal dominant disorder that developmental abnormalities in several organs including the liver, heart, skeleton, eye and face, kidney, vasculature and skin.Alagille syndrome is caused by mutations in both a ligand(Jagged1 (JAG1)) and receptor(NOTCH2) that activate the Notch signaling pathway.The criteria of Alagille syndrome contains the presentation of 3/5 clinical features including anomalies of the cholestasis, heart defects, spinal deformity (butterfly vertebrae), eye abnormality (posterior embryotoxon) and prominent facial features(inverted triangular face) along with bile duct paucity on liver biopsy.In recent years, with the progress of molecular diagnostic techniques and etiology research for each system phenotype, ALGS has attracted more and more attention.This article reviews the clinical manifestations,pathogenesis and diagnosis of ALGS.

Objective To investigate the early clinical characteristic of neonatal peneumonia,and its early diagnosis and therapy. Methods Clinical data were analyzed retrospectively in 191 children with nenonatal peneumonia. Results Among 191 cases,72 cases were typical(37.7%);119 cases were atypical (37.7%);35 cases showed pneumonia by sternum(18.3%);19cases with fever(9.9%);104 cases with cough(54.5%);23cases with polypnes(12.0%);15 eases were transported to higher level hospital (7.8%);no death case. Conclusion Cough is an imporeant value to diagnosis in early clinical sympthon with neonatal pneumonia,and a characteristic in neonatal pneumonia. To improve the prognsis with neonatal pneumonia is early diagnosis and therapy.

Objective To observe efficacy of Pulmicort in treatment of capillary bronchitis.Methods Children with capillary bronchitis were randomly divided into two groups,ninty-eight cases of study group were treated with Pulmicort,fifty-eight cases of control group were treated with common therapy,observe clinical symptom:cough,breathes heavily. Results Two groups efficiency were 93.8% and 72.4% respectively.The study group was significantly better than that in control group. Conclusion Pulmicort inhalation in treatment of children with capillary bronchitis are effective.

0.05). Conclusion: Bibliotherapy is helpful for mental health in the college students.

AIM:To observe the neuritogenic actions of botulinum neurotoxin serotype A heavy chain ( BoNT/A HC) on cultured Neuro-2a cells and to investigate the related signaling mechanisms for the effect of BoNT /A HC. METHODS:Neuro-2a cells were treated with different doses of BoNT/A HC (0.01, 0.1, 1 and 10 nmol/L), and then the cells were harvested at 24 h, 48 h and 72 h of BoNT/A HC exposure for detecting the neurite length and the percen-tage of the cells with neuronal processes by immunofluorescence staining .The most efficient dose of BoNT/A HC was cho-sen for exposure to Neuro-2a cells as the above.Whole cell protein was harvested at different time points for detecting the protein levels of phosphorylated ERK 1/2 ( p-ERK1/2 ) and phosphorylated Akt ( p-Akt ) by Western blot .RESULTS:Low doses of BoNT/A HC stimulated the neurite outgrowth , and increased the percentage of the cells with neurites com-pared with the negative controls (P<0.05), especially in the group with 1 nmol/L of BoNT/A HC treatment.Meanwhile, the phosphorylation of ERK 1/2 and Akt was increased after treated with BoNT/A HC.There was an increasing tendency for the phosphorylation of ERK1/2 after the exposure of the cells to BoNT/A HC.The obvious increase in p-ERK1/2 was seen from 60 min to 5 h with 1 nmol/L of BoNT/A HC treatment ( P<0.05 ) , and the increased protein level of p-Akt was mainly observed at 15 min and 60 min ( P<0.05 ) .CONCLUSION: BoNT/A HC stimulates the neuritogenesis .The neuritogenic mechanism of BoNT/A HC on Neuro-2a cells might be realized by activation of the phosphorylation of ERK 1/2 and Akt.

Objective To investigate the clinical effect of proximal femoral nail anti rotation intramedullary nail (PFNA)in the treatment of intertrochanteric osteoporotic fractures in elderly patients.Methods 42 cases of femoral intertrochanteric fracture were treated with proximal femoral nail anti rotation intramedullary nail (PFNA)in cases (AO type,A1 type 13 cases,A2 type 24 cases,type A3 5 cases).Results The group of 42 patients after 12 -36 months of follow -up,the average follow -up time of 18 months,all the patients were healed,1 case of deep venous thrombosis,1 case of mild coxa vara.According to the Harris evaluation standard:excellent 32 cases,good in 9 cases,fair in 1 case,the excellent rate was 97.6%.Conclusion The proximal femoral nail anti rotation (PFNA) anti tensile,anti sliding and anti rotation function is good,and has the advantages of simple operation,small trauma,for osteoporotic intertrochanteric fracture patients can be applied.

Nowadays, nanotechnologies have shown wide application foreground in the biomedical field of medicine laboratory tests, drug delivery, gene therapy and bioremediation. However, in recent years, nanomaterials have been labeled poisonous, because of the disputes and misunderstandings of mainstream views on their safety. Besides, for the barriers of technical issues in preparation like: (1) low efficacy (poor PK & PD and low drug loading), (2) high cost (irreproducibility and difficulty in scale up), little of that research has been successfully translated into commercial products. Currently, along with the new theory of "physical damage is the origin of nanotoxicity", biodegradability and biocompatibility of nanomaterials are listed as the basic principle of safe application of nanomaterials. Combining scientific design based on molecular level with precision control of process engineering will provide a new strategy to overcome the core technical challenges. New turning point of translational medicine in nanotechnology may emerge.

Objective: To understand the concentrations and source of SO42-,NO3-,Cl- and NH4+ in atmospheric PM2.5 in Zhoushan,and to provide reference for controlling PM2.5 and formulating effective environmental protection measures. Methods: Monitoring sites in new districts of Zhoushan were set up to continuously collect PM2.5 from 10th to 16th of each month and under the hazy weather during 2015-2016（AQI > 200）. The mass concentration of PM2.5 was measured by weighing method,and the concentrations of SO42-,NO3-,Cl- and NH4+ in PM2.5 components was detected by ion chromatography. Results: The average daily concentration of PM2.5 in Zhoushan from 2015 to 2016 was（40.91±27.39）μg/m3. The concentration of the four water-soluble non-metal ions in PM2.5 components was 3.56-103.03 μg/m3,with an average of（23.06±20.00）μg/m3,accounting for about 56.64% of PM2.5 contents. The average monthly concentration of SO42- was the highest[（10.35±6.48）μg/m3],while the average monthly concentration of Cl- was the lowest [（0.49±0.73）μg/m3]. The concentration of the four ions was the highest in winter[（37.56±27.74）μg/m3]and the lowest in summer[（12.32±5.88）μg/m3]. The differences between different seasons was statistically significant（P<0.05）. The highest concentration of NO3- occurred in winter,which was（14.48±13.28）μg/m3. The concentration ratio of NO3- to SO42- ranged from 0 to 2.58,with an average of 0.55. There were 28 days（14.74%）with the ratio greater than one,22 days of which was in winter. Conclusion The concentration of SO42- was the highest and Cl- was the lowest in atmospheric PM2.5 in Zhoushan. The highest concentration of the four ions occurred in winter. The concentration of NO3- in winter was higher than that of SO42-,suggesting that motor vehicle exhaust might be the main source of PM2.5 in winter.

Objective Our retrospective study is aimed to analyze the correlative clinical features of be-tween expressions of miR -135b and miR-141 in colorectal cancer.Methods Clinical data and specimens of patients with colorectal carcinoma at our hospital from 2013 to 2014 were retrospectively collected .The expres-sions of miR-135 b and miR-141 between colon cancer and matched normal colonic tissues were compared .The correlation between the expressions of miR -135 b and miR-141 in colorectal carcinoma and the clinical charac-teristics were discussed .Results A total of 42 patients was retrospective analyzed .The expressions of miR -135b,miR-141 in the colon cancer tissues were obviously higher than that in the normal colonic tissues .The differences of the expression of miR -141 in the colon cancer tissues of different Dukes stages ,degree of differen-tiation,lymph node metastasis and infiltration depth were all significant .The expressions of miR -135 b and miR-141 displayed a positive linear correlation .Conclusion The expressions of miR-135b,miR-141 in colorec-tal cancer are significantly increased ,and the expressions display a remarkable correlation with the Dukes stages , degree of differentiation ,lymph node metastasis and infiltration depth of tumor .The expressions of miR-135 b and miR-141 demonstrate a positive correlation .