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.

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.

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 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 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.

Objective To evaluate the imaging performance and characteristics of peripheral primitive neuroectodermal tumors (pPNET), in order to raise awareness of the disease and the diagnostic accuracy. Methods Thirteen patients with pPNET who confirmed by puncture or pathology were enrolled in this study. The CT and MRI features were retrospectively analyzed. Four patients received simple routine CT examination, and 9 patients had MRI examination based on X-ray, CT examination. Results The part of body involved was more widely.Two patients occurred in the sinuses, 1 patient in the chest wall, 1 patient in the lung, and 3 patients in the abdominal and pelvic(1 patient involved the pelvic floor through the pelvic wall lesions to basins), 1 patient in the spine, and 5 patients located in the extremities. The characteristics of pPNET by CT and MRI in soft tissue of 8 patients showed single large soft tissue mass, and the biggest was 11.2 cm × 10.6 cm. In bonds and joints of 5 patients, CT and MRI showed wide range of soft tissue mass, mixed internal signal/density with obviously uneven enhancement, significant bone destruction and dissolvable osseous changes, and no obvious tumor bone and calcification. Conclusions Each site has its relative imaging characteristics of pPNET. CT and MRI can show lesions involving the scope, internal structure, with or without adjacent tissue invasion and metastasis, and provide guidance for clinical treatment and selecting treatment regimen. But in the end, it depends on the diagnosis of pathological and immunohistochemical examination.

To detect the effects of pioglitazone on adipose triglyceride lipase (ATGL) in adipose,liver,and muscle of high-fat diet-induced obese rats.Insulin resistance aud decreased expression of adipose triglyceride lipase were found in the adipose,liver,and muscle tissues.Pioglitazone may improve insulin sensitivity and ameliorate ATGL expression in tissues of high-fat diet-induced obese rats.

Adult ; Aged ; Breast Neoplasms ; diagnostic imaging ; drug therapy ; prevention & control ; Combined Modality Therapy ; Drugs, Chinese Herbal ; therapeutic use ; Female ; Humans ; Middle Aged ; Phytotherapy ; Radiography

Objective To investigate the effect of the asthmatic mice's contents of NF-κB、IκB、p-IκB treated with curcumin.Methods Thirty Balb/c mice were divided into three groups randomly,the normal mice,the asthmatic mice and the curcumin mice.The bronchial hyperresponsiveness of the three groups were exanined by lung function.The NF-κB 、IκB 、p-IκB content of three teams were tested.Results The content of cytoplasm NF-κB in asthmatic mice was lower than the control (P ＜ 0.01).However,the content of cytoplasm NF-κB in curcumin team was more than the asthmatic mice (P ＜ 0.05).In the other hand,The content of nuclear NF-κB in asthmatic mice was more than the control(P ＜0.01).However,the content of nuclear NF-κB in curcumin mice was lower than the asthmatic mice (P ＜ 0.05).The content of cytoplasm p-hκB in asthmatic mice was more than the control and the content of IκB was lower than the control(P ＜ 0.01).Howerer,the content of IκB in curcumin team have much more than the asthmatic mice and the content of p-IκB have much lower than the control(P ＜ 0.01).Condusion Curcumin alleviates the airway hyperresponsiveness of the asthmatic mice through the suppression of NF-κB transcribe to the nuclear via alleviating the phosphorylation of I-κB.