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.

Aim To establish an in vitro insulin resistant model of HepG2 cells and 3T3-L1 adipocyte and to screen drug in vitro.Methods The insulin resistant models of HepG2 and 3T3-L1 adipocyte were induced by high concentration of insulin and by dexamethasone,respectively.The glucose consumption ofcells was detected after administration with different drugs. Results In the concentration of 10-6 mol?L-1 insulin for 36 hours,HepG2 cells were resistant to insulin and the insulin resistance was maintained for 48 hours without change of cell morphology.After 3T3-L1 adipocyte insulin resistance was induced by dexamethasone,the maximal difference of glucose consumption between the blank control and insulin resistant model group was observed at 96h after dexamethasone administration,but the insulin resistant status had only been maintained for 24 hours without dexamethasone.The glucose consumptions of insulin resistant model of HepG2 and 3T3-L1 adipocyte were promoted by some drugs such as stachyose,berberine and ginsenoside.Conclusions The insulin resistant model of HepG2 cell and 3T3-L1 adipocyte was successfully established in vitro by high concentration of insulin and by dexamethasone,respectively.It can be used to screen drugs for treatment of insulin resistance.

OBJECTIVE To study the clinical features and therapeutic efficacy of middle ear adhesion.METHODS The clinical data of 40 cases of middle ear adhesion collected during 5 years from 2002 to 2007 were studied retrospectively. RESULTS Part or all of the tympanic membrane of the patients did not move under Siegle otoscopy. The lesions of middle ear adhesion were extensive and invaded all middle ear structures including round and oval windows.The lesions were severe, especially in narrow anatomical spaces such as mesotympanum,hypotympanum and interossicular spaces.Pure tone audiometry showed that average air-bone gap (A-B gap) were (35.34?6.99) dB preoperatively.The average A-B gap were (18.47?5.76) dB 6 months after operation.Follow-up observations and postoperative CT scan showed that tympanic membranes and middle ear cavity recovered well.CONCLUSION There are various degrees of tympanic fibrous adhesion and atelectatic tympanum in middle ear adhesion,especially in mesotympanum, hypotympanum and interossicular spaces.The middle ear adhesion can be treated with operation.The key points for a successful operation include preserving normal tympanum mucosa,opening eustachian tubes, making a bigger middle ear cavity,and performing tympanoplasty with perichondrium-cartilage plates.

Objective:To observe the compatibility of Xiaoaiping injection and insulin for injection in glucose infusion. Methods:The changes in appearance, particles and pH of the mixture in 24h at ambient temperature were observed. The concentration of chloro-genic acid and insulin were determined by HPLC. Results:There were no obvious changes in appearance, pH, precipitation or turbidi-ty generation, and the contents of insulin and chlorogenic acid kept stable in 12 h. The number of insoluble particles was stable in 12 h, and then gradually increased in 24 h. Conclusion:Xiaoaiping injection and insulin in 5％ glucose infusion is stable in 12h under the testing conditions.

Objective To evaluate the value of serum uric acid(UA)levels with reference to the age,waist circumference,and body mass index(BMI)in predicting the metabolic syndrome(MS)in obese children.Methods A total of 300 obese children,including 180 boys and 120 girls,were enrolled in this study.The height,BMI,waist and hip circumference,blood pressure,serum glucose,insulin and lipid profile in all participants were measured.Oral glucose tolerance test and insulin releasing test were performed.The boys or girls were divided into 4 groups according to the 4 quantile of UA level,respectively.The clinical characteristics and correlation of UA with the clinical indexes and MS components were compared.The binary Logistic regression analysis was applied in the risk of MS and its components for the 4 groups of obese children.The area under the receiver operating characteristic curve(ROC curve)of UA level,age,waist circumference and BMI were used to predict the MS.Results UA level was increased with the increase of age,waist circumference and BMI,and the UA level was significantly correlated with triacylglycerol,postprandial 2 h glucose(2 h PG)(r=0.196,0.174 in boys;r=0.291,0.179 in girls).In boys,the adjusted odds ratio and 95％CI of the highest quartile of UA for triglyceridemia was 2.71(95％CI:0.77-9.58);which in girls,the adjusted odds ratio and 95％CI of the highest quartile of UA for hyperglycemia,hypertension were 8.45(95％CI:1.76-40.52)and 3.93(95％CI:0.66-23.33),respectively,with significant differences.In boys,the area under the ROC curve of UA level,age,waist circumference and BMI which predict the MS were 0.652 0.626,0.621,0.62,respectively,and the differences were significant(all P<0.05).Conclusions The UA level is significantly correlated with the composition of MS,UA detection combining with reference to the age,waist circumference,and BMI is helpful for the identification of high risk groups of metabolic syndrome.

Objective To investigate the effect of the asthmatic mice's contents of nuclear factorerythroid-2-related factor 2,Nrf2 and HO-1 reated with curcumin in lung tissue.Methods All 45 mice were divided into three teams randomly,the normal mice,the asthmatic mice and the curcumin mice group.We tested the expression of Nrf2 and HO-1 of three mice teams in lung by immunolfluorescence technique.We tested the protein contents of Nrf2 and HO-1 of the three teams in the lung tissue by Western blot.We tested the binding activity of Nrff2 and ARE of the three teams in lung by EMSA.Results The expression of Nrf2 and HO-1 in curcumin group was significantly higher than that in asthma group and normal control group by immunolfluorescence technique.There was no sigrnificant difference in content of cytoplasm Nrf2 protein in lung tissue between three groups by western blot(P ＞ 0.05).The content of nuclear Nrf2 and HO-1 protein in the lung tissue of the curcumin group was significantly higher than that in the other two groups,the difference is statistically significant (Nrf2,P ＜0.05;HO-1,P ＜0.01).The binding activity of Nrf2-ARE in lung tissue of curcumin group was significantly higher than that of asthma group and control group,with statistical significance(P ＜ 0.05).Conclusion Curcumin can increase the protein contents of the Nrf2 and HO-1 of mice and enhance the expression of them.

Objective To investigate the mRNA and protein expressions of Nav 1.1 and Nav 1.2 in hippocampus following traumatic brain injury ( TBI) in rats.Methods After the lateral fluid percussion model was established in adult male Sprague Dawley rats,the rats were sacrificed at 2,12,24 and 72 hours after percussion and collected ipsilateral hippocampus for detecting mRNA and protein expressions of Nav 1.1 and Nav 1.2 by means of fluorescent quantitation RT-PCR,Western blot and immunofluo rescence staining.Results The mRNA expressions of Nav 1.1 and Nav 1.2 were significantly down-regulated (P<0.01) in hippocampus and reached the lowest level at 2 hours following TBI.The protein expression of Nav 1.1 was significantly down-regulated (P<0.01) but recovered near to level of control group at 72 hours after TBI.While there was no statistical difference on protein expression of Nav 1.2 in hippocampus after TBI compared with control group (P>0.05).Conclusion TBI induces significant down-regulated mRNA and protein expressions of Nav 1.1 in the hippocampus,which may be one of molecular mechanisms for functional alternation of sodium channels and excitotoxic action following TBI.

Objective: To investigate the dietary nutritional status of the intellectuals in three cities in China. Methods: The intellectuals aged over 45, with senior academic title, working in universities or research institutes in Tianjin, Guangzhou and Chengdu, were selected as volunteers. Food frequency questionair was used to conduct the diretary survey. And the results were compared between the intellectuals of nutrition and non-nutrition speciality. Results: Compared with Dietary Guideline and Balance Diet Pagoda for Chinese Residents, the intakes of cereals, milk, fruits,and egg of the vulunteers were adequate, but the intakes of meat and oils were more, while those of fish and shrimp, legume,and vegetables were less than the suggested levels in Pagoda. The food pattern of the intellectuals in nutrition speciality was more advantageous than that in non-nutrition. Conclusion: The dietary pattern of the intellectuals should be improved, according to the Dietary Guideline and Balanced Diet Pagoda for Chinese Residents.

Objective To investigate the impact of paced QRS duration (pQRSd) on heart function in patients with right ventricular apical pacing. Methods Seventy-six patients with Ⅲ° atrioventricular block received pacemaker treatment were enrolled and randomized into group A (pQRSd < 190 ms, n = 52) and group B(pQRSd≥ 190 ms, n = 24). The concentration of brain natriuretic peptide (BNP),parameters of left atrial diameter (LAD), left ventricular ejection fracetion (LVEF), left ventricular end diastolic diameter (LVEDD), left ventricular end systolic dimension (LVEDD) were measured before operation, at 12 months and 24 months after implanting, respectively. The parameters of echocardiography assay, the concentration of BNP and the incidence of heart failure event after implantation were compared between two groups. Results At 12 months after implanting, LVEF of the patients in the group B decreased significantly compared with that of group A (P < 0.05). However, the echocardiography paramenters and the concentration of BNP were not significantly different between the two groups (P > 0.05). At 24 months after implanting, LAD、LVEDD、LVESD of group B increased significantly compared with those of group A [LAD,( 44.5 ± 6.2) mm vs (41.6 ± 5.1) mm, LVEDD, (52.7 ± 9.3) mm vs (48.2 ± 7.5) mm, LVESD, (37.5 ± 5.6) mm vs (33.8 ± 4.9)mm, each P < 0.05, respectively]. The concentration of BNP of group B increased significantly [(408.2 ± 102.1)ng / L vs (243.7 ± 92.8)ng / L, P < 0.001], and LVEF of the patients in group B decreased significantly compared with those of group A [(46.3 ± 6.8)% vs (51.6 ± 5.2)%, P < 0.001], respectively. No significant difference in the incidence of heart failure event (41.7% vs 26.9%, P > 0.05)between two groups during 24-month follow-up. Conclusion The prolonged paced QRS duration has a detrimental effect on long-term cardiac function during RVA pacing in patients with Ⅲ°atrioventricular block.

Objective To investigate the detection and clinical features of primary immunodeficiency disorders (PID) in children for an earlier diagnosis of disease.Methods The clinical data of the 27 children with PID diagnosed in Shengjing Hospital Affiliated to China Medical University from Dec.2003 to Nov.2011 were reviewed,including illness history,birth history,family history,clinical feature,laboratory data,diagnosis,treatment and outcome,etc.Results In 27 children with PID,antibody deficiencies were the most frequent findings (48.15％,13/27 cases),followed by combined immunodeficiency (22.22％,6/27 cases),phagocytic disorders (14.81％,4/27 cases),and immunodeficiency with other major defects accounted for 14.81％ (4/27 cases).PID was characterized by recurrent,severe and prolonged infection,but all kinds of PID had their own clinical features.Recurrent infections occurred in 24 cases.Respiratory infections and otitis media were the most common clinical manifestation.Seven patients had a family history.The fatality rate was 37.04％ (10/27 cases).Conclusions There are vast varieties of PID in our area and antibody deficiencies are the most common type.All kinds of PID have their own clinical features,which may guide us to choose appropriate lab examination.There are nearly 25％ patients with PID who have family history.The fatality rate is high.Patients who suffer from recurrent infections,especially respiratory infections or otitis media,or those with a family history should have early immunology testing so as to be detected and diagnosed of PID earlier.