Objective To investigate the clinical features of X-linked agammaglobulinemia (XLA)in children,and to improve the early diagnostic rate.Methods The medical records of 11 children with XLA between Dec 2003 and Nov 2011 were reviewed.Results The smallest age of 11 cases with XLA presenting the first symptoms was 0.4 years old and the oldest age was 4 years old with a mean of 2.4 years old.The first diagnosis time was at the age of 3.5 to 13 years old,with a mean of 7 years old.The age at first diagnosis in 63.6％ ( 7/11 ) of patients was more than 7 years old.Two patients ( 18.2％ ) had the family history of the similar disease.Two patients were died from the infection and 1 patient was missed.The other 8 patients were survived.Respiratory infections occurred in 100％ of the 11 patients.54.5％ (6/11 )of the patients were suffered with otitis media and digestive infections were seen in 36.4％ (4/11 ) of the patients.The levels of lgA,IgM and IgG in the serum of the pauents were all decreased.The levels of IgG in the serum of 9 patients were less than 2 g/L and in the serum of the other 2 patients were less than 2.4 g/L.The levels of CD19 in the serum were all less than 1％ and an inversed ratio of CD4/CD8 was observed in 9 patients.Eight patients were diagnosed as XLA by gene detection.Conclusion The age presenting first symptoms in children with XLA in this study is earlier than the reported data and the age at first diagnosis is later.Respiratory infection is the most common manifestation.High prevalence of otitis media and digestive infections are common.The patients with XLA rarely have the family history.The early diagosis and long-term treatment with the intravenous immunoglobulin may improve the prognosis of XLA.

The cost-benefit analysis in large-scale medical equipment are explored.The management of medical equipment cost adopts methods of pre-forecasting,control in the course,and post-evaluation.The utilization of this analysis method shows active guiding significance.

OBJECTIVE:To compare the contents of hesperidin in raw Fructus Aurantii Immaturus and its processed products.METHODS:HPLC was used for the quantitative analysis of raw Fructus Aurantii Immaturus and its processed products.RESULTS:The content of hesperidin in vinegar-fried Fructus Aurantii Immaturus was the highest and that in the bran-fried Fructus Aurantii Immaturus was the lowest.CONCLUSION:It is suggested that there exist significant differences in intrinsic quality among various processed products of Fructus Aurantii Immaturus.

Objective To observe the improvement of Salmeterol fluticasone propionate and Tiotropium bromide for inhalation in the treatment of chronic obstructive pulmonary disease ( COPD ) with bronchial asthma patients.Methods 89 patients were randomly divided into the observation group with 46 cases and control group with 43 cases,both groups were given conventional therapy.The control group was given Salmeterol fluticasone propionate 50μg/500μg,twice a day;the observation group was given Tiotropium bromide 18μg, twice a day, based on the therapy of control group.The two groups were treated for 14 days,and then curative effect,score of SGRQ,pulmonary function and blood gas analysis index were evaluated, and adverse reactions were recorded.Results The total effective rate of treatment group was 86.1%,higher than that of the control group(68.6%) (Z=2.68,P<0.05);Compared with the control group after treatment,functional parameters[ FEV1 ( L) ,FEV1 (%) and FEV1/FVC(%) ] of the observation group increased significantly,and the observation group were all higher than that of the control group [(2.27 ±0.45) vs (1.92 ±0.36),(64.27 ±16.11) vs (53.64 ±15.55),(54.85 ±14.81) vs (45.33 ± 13.23)],the differences were all statistically significant(t=4.20,3.16,3.19,all P<0.05);PaO2 of the two groups patients were increased and PaCO2 were decreased,scores of SGRQ were decreased obviously,and the change of the observation group was obviously[(47.61 ±3.64) vs (49.34 ±4.23),(67.33 ±5.56) vs (63.66 ±5.21), (45.34 ±3.72) vs (50.65 ±4.16)],the difference was statistically significant(t =2.13,3.32,6.54,all P<0.05) .Conclusion Salmeterol fluticasone propionate and Tiotropium bromide for inhalation can improve lung func-tional parameters and respiratory conditions of the parents with COPD and bronchial asthma obviously, reduce the score of SGRQ,curative effect is distinct,worthy of clinical popularization and application.

Objective To investigate the relation between the expression of toll receptor-4 (TLR-4) and the increase of blood brain barrier (BBB) permeability in the rats with acute pancreatitis.Methods Male Sprague-Dawley rats were randomly divided into 8 groups:control group;acute edematous pancreatitis (AEP) 2 h,6 h groups;acute necrotizing pancreatitis (ANP) 2 h,6 h,12 h,24 h,48 h groups with 8 rats in each group.The BBB permeability,pathological score of pancreas,TLR-4 expression were determined and the relationships between them were analyzed.Results The BBB permeability in control group;acute edematous pancreatitis (AEP) 2 h,6 h groups;ANP 2 h,6 h,12 h,24 h,48 h groups were 1.55±0.29,1.64±0.17,1.69±0.24,1.89±0.12,2.66±0.32,2.91±0.29,2.89±0.69 and 1.84±0.07,respectively;the pathological scores of pancreas were 0,2.38±0.92,3.13±0.64,8.50±1.07,9.75±0.71,10.25±1.28,11.13±1.25 and 10.13±1.13,respectively;there was no significant difference between AEP groups and control group,while there was significant difference between AEP groups and ANP groups (P＜0.05 orP ＜0.01).BBB permeability was correlated with pancreatic injury ( r = 0.626,P ＜0.01).There was no TLR-4 mRNA and protein expression in the control and AEP group,while they were significantly expressed in ANP groups,and the expression were positively related with BBB permeability ( r =0.208,P = 0.027 ).Conclusions BBB permeability was present in the course of ANP.Activation of TLR-4 signal pathway may be involved in the BBB permeability increase caused by ANP.

Objective Explore reflux esophagitis age,symptoms,and the relationship between the degree of esophageal damage.Methods Summing up 198 cases of young and middle-aged patients and 104 cases of elderly pa-tients with the symptom score and Endoscopic mucosal injurs classification.Results Under the old group gastroscopy serious esophagitis more than the young group,old age group was 49.97%,25.25%for the young group,but the clini-cal symptoms of heart burn not obvious.Conclusion Reflux esophagitis damaged esophagus increased with age in-crease,but clinical heartburn symptoms do not necessarily obvious,it should attach importance to the brink throat dis-comfort,and other symptoms.

Objective To observe the sensitivity ofastrocytoma to chemotherapeutic drugs, in order to explore the drug selection using chemosensitivity testing in vitro on astrocytoma. Comparing different WHO grade astrocytoma to chemotherapeutic drugs sensitivity differences,providing the basis data for clinical treatment. Methods The astrocytoma cells were seperated and cultured in vitro from 142 tumor tissues by glioma resection, including 55 cases of low-grade astrocytoma (WHO grade Ⅰ , Ⅱ) and 87 cases of high-grade astrocytoma (WHO grade Ⅲ, Ⅳ). The proliferation inhibition and chemosensitivity of tumor cells to 11 drugs were investigated by using MTT method. Results There were 54.93% (78/142) patients recommended chemotherapeutic drug in 142 cases. The percentage was 41.82%(23/55) in the low-grade astrocytoma, and 63.22% (55/87) in the high-grade astrocytoma respectively. Teniposide,carboplatin,vincristine as the sensitive drugs were similar in different WHO grade astrocytoma. Conclusions The chemosensitivity testing could be used to filtrate sensitive drugs to clinical chemotherapy. The action is more prominent in high-grade astrocytoma than in low-grade astrocytoma. There is no difference in the choice of sensible drugs among different WHO grade astrocytoma.

BACKGROUND: The application of deacetylated chitin (chitosan, DC) prepared by traditional technique was limited because of its lower degree of deacetylation, impurity and relative lower molecular mass.OBJECTIVE: To improve the traditional technique for extraction and preparation of DC from the rustae f oratosquila oratoria.DESIGN: Contrasting observation.SETTING: Shandong Institute of Biopharmaceutics.MATERIALS: The experiment was carried out in the Shandong Key Laboratory for iopharmaceutics, Shandong Institute of Biopharmaceutics from February 2003 to January 2004. Experimental nstrument: Mettler AE240 electronic analytical balance and ZK-40AB electrothermal vacuum drying oven were rovided by Mettler-Toledo Instruments Co., Ltd. And ShangHai Shuli Instruments Co.,Ltd., respectively. Xperimental materials: oratosquila oratoria was purchased from Jinan aquatic products market.METHODS: ①C as traditionally prepared from chitin by decoloring with strong oxidant and deacetylating with concentrated alkaline liquor under high temperature. Chitin was extracted from crustae of shrimp and crab fter decalcification with diluted acid and deproteinization with diluted base. ②The improved preparation echnique: The crustae of oratosquila oratoria was decalcificated and decolorized with diluted acid, and as deproteinized and decolorized with diluted base at room temperature, and then the chitin was obtained fter the second decalcification and decolorization with diluted acid. The crude DC was obtained from hitin after deacetylation and decolorization with concentrated ase at 55-65 ℃. The crude DC was issolved n diluted acid and filtered. The filtrate was collected, precipitated with dilute base. After filtering, he sediments were retained, washed with water and dried. Then pure DC was obtained.MAIN OUTCOME MEASURES: he comparison between improved technique and traditional technique; Color, dissolvability in diluted cid, ield, degree of deacetylation and dynamic viscosity of DC prepared by different techniques.RESULTS: ①echnique comparison: Compared with traditional technique, the reaction of improved preparation technique as milder, and the deproteinization was at room temperature; it did not need an isolated decolorization;deacetylation was at low temperature, and had a refine process. ②Detection indexes of DC: he yield of DC prepared by improved technique was little lower than that by traditional technique (15%, 7% respectively). The degree of deacetylation and dynamic viscosity were both higher than those by raditional technique (＞ 95%,＜ 70%; ＞ 120mPa·s,＜ 80 mPa·s , respectively). The color was white or lmost white, which was better than that by traditional technique (yellow or gray). The DC could ompletely issolve in dilute acid, and the dissolvability was better than that by traditional technique (dissolved artly in dilute acid).CONCLUSION: Compared with the traditional technique, the improved technique was mpler and the reaction conditions were milder. And also the quality of obtained DC was obviously improved.

Objective To explore efficiently treatment of advanced stage superior segment esophageal cancer.Methods The 30 cases with advanced stage superior segment esophageal cancer were randomly separated two groups:Treatment group 14 cases were local chemotherapy treated by gastroendoscopy,comminute with radication;Control group 16 cases were treated by surgical operative treatment,combinate with vadication.Results The treatment group and conerol group remission rates were 85.71% and 68.75%,1 year's survival rates were 71.42% and 50%.Conclusion Superior segment advanced stage esophageal cancer is local chemotherapy treated by gastroendoscopy,cuvative efficavy is distimction.

Objective To analyze the endoscopic features of patients with the lower esophageal sphincter (LES) and crural diaphragm (CD) separation and explore its significance in hiatus hernia (HH) diagnosis.Methods From January 2011 to June 2012,the data of patients underwent esophagus high resolution manometry (HRM) examination were retrospectively analyzed and of which the patients with LES-CD separation were selected.Patients who received digestive tracts operation or lack of complete endoscopic date were excluded.A total of 93 cases were enrolled for analysis.According to Chicago Criteria,the cases were divided into three groups.There were 21 cases in type Ⅰ group (LES-CD separation＜1 cm),37 cases in type Ⅱ group (LES-CD separation≥l cm and ＜2 cm),35 cases in type Ⅲ group (LES-CD separation≥2 cm).The differences among groups in endoscopic features were analyzed.HRM were taken as golden standard,the sensitivity and specificity of endoscopy in HH diagnosis were analyzed.Measurement data were expressed as x ± s,one-way ANOVA was performed for multi-groups comparisons,LSD method or Dunnett T3 test for pairwise comparisons and chi-square test or Fisher's exact test for two groups' comparisons.Results There were no significant differences in the distance between esophagus gastric junction (EGJ) and foreteeth of the three groups (P＞0.05).Distances between EGJ and diaphragmatic HH pressure impression in type Ⅲ group,type Ⅱ group and type Ⅰ group were (3.57±0.78) cm,(1.89±0.81) cm and (1.14±0.67) cm,respectively and the differences were significant (LSDt=9.26,11.44,3.57; all P＜0.05).The percentage of continuously-open cardia and showing pressure impression of the gastric pouch of type Ⅲ group was higher than that of type Ⅱ group and the percentage of continuously-open cardia and showing pressure impression of gastric poach of type Ⅱ group was higher than that of type Ⅰ group (80.0％,40.5％ and 4.8％,x2 =11.64,29.76 and 8.59; 91.4％,27.0％ and 4.8％,x2 =30.69,40.73 and 4.32' all P＜0.05).The occurrence rates of the His angle blunting and sac of type Ⅲ group were higher than those of typeⅡ group and type Ⅰ group (74.3％ and 77.1％,24.3％ and 24.3％,4.8％ and 4.8％,x2 =17.97 and 25.41,both P＜0.05),there was no significant difference between type Ⅱ group and type Ⅰ group (P＞0.05).The rates of esophagitis of type Ⅲ group and type Ⅱ group were higher than that of type Ⅰ group (71.4％,59.5％ and 14.3％,x2 =17.14 and 11.15,both P＜0.05),there was no significant difference between type Ⅲ group and type Ⅱ group (P＞0.05).There was one case of Barrett's esophagus in each group.The sensitivity and specificity of endoscopy in HH diagnosis were 91.4％ and 81.0％,respectively.Conclusions The distance between EGJ and hiatal pressure impression,continuously-open cardia,the His angle blunting,hiatal pressure impression of the gastric pouch and hernia sac were important indicators of HH endoscopic features.The sensitivity of endosocopy is higher than the spcificity of endosocopy in HH diagnosis.