Primary immunodeficiency disease (PID) is a heterogeneous group of congenital disorders of the immune system.The impaired immune response is caused by defect of immune active cells and moleculars,which results in increased susceptibility to pathogens.Recurrent infection is the predominant presenting complaint for patients with PID.Early diagnosis,immunoglobulin replacement therapy,antimicrobial treatment,haematopoietic stem cell transplantation and gene therapy,genetic conselling and prenatal diagnosis will help to prevent and control infections in patients with PID.In addition,we should strengthen the education of paediatricians,improve awareness about PID and collaboration across different centers to advance the diagnosis and management of PID in China and save the life of patients at the maximum limit.

The hepatic portal system is a unique circulatory system that connects two systems of capillary beds ; one in the wall of the small intestine and spleen and the second in the sinusoidal area of the liver.Therefore,alterations in vasoreactivity (vasodilation and vasoconstriction) play a critical role in the pathophysiology of portal hypertension (PHT).The RhoA/ ROCK pathway exerts an important role in the Ca2+-independent mechanism in vascular smooth muscle (VSM).This mechanism not only modulates the constriction of intrahepatic small vessels and hepatic stellate cells (HSCs) but also effects the hyperdynamic circulation due to vascular hyporesponsiveness.Understanding the detailed mechanism and role of the RhoA/ROCK signal pathway in portal hypertension could be of great utility in providing a new target for portal hypertension therapy.

Objective To investigate the effects of the 2,3,4′,5-Tetrahydroxystilbene -2-O-beta-D-glucoside on the prolif-eration of PASMCs induced by hypoxia , in orde to search new drugs for the treatment and prevention of hypoxic pulmonary vascular remod -eling.Methods 3%O2 hypoxia was used to induced the proliferation of PASMCs .After hypoxic and TSG treatment for 24h, cell growth was determined by cell counting kit -8 ( CCK-8 ) , cell cycle was analysed by flow cytometry , the mRNA expression of HIF -1αwas measured by quantitative real -time PCR, and the reactive oxygen species ( ROS) production was determined by the fluorescence micro-plate reader .Results TSG can block the proliferation of PASMCs through G 0/G1 to S phase of the cell cycle arrest without cell cytotoxic-ity.Further experiments showed that TSG blocking the proliferation of PASMCs was associated suppression the mRNA expression of HIF -1αand the production of intracellular ROS in hypoxia -stimulated-PASMCs.Conclusion TSG can inhibit the proliferation of pulmo-nary artery smooth muscle cells induced by hypoxia through suppression the mRNA expression of HIF -1αand the production of intracel-lular ROS.

Objective To establish the quality standard of Huangqi-Changyu'an capsule. Method Thin layer chromatography was used to identify psoralen, evodia mtaecarpa, and nutmeg in Huangqi-changyu'an capsule. HPLC was used to determine the content of psoralen. A Hypeisil BDS C18 column(200 mm×4.6 mm,5 μm) was used with mobile phase consisted of acetonitrile-water (30:70) at the rate of 1 ml/min. The detection wavelength was set at 245nm with the column at room temperature. Results The liner range of psoralen was 3.572~53.58 μg/ml (r=0.999 9) with average recoveries of 99.04%, and the RSD was 0.8%. The liner range of isopsoralen was 3.796~56.94 μg/ml (r=0.999 9) with average recoveries of 99.20%, and the RSD was 0.6%. Conclusion This method is sentivive, accurate and rapid for the quality control of Huangqi-Changyu'an capsule.

Primary systemic anaplastic large cell lymphoma (ALCL) is a subtype of rare and heterogeneous clinical entity, extremely rare in early stage of disease. There is no standard treatment yet. Based on retrospective studies, systematic chemotherapy followed by involved field radiation after chemotherapy is the mainstay of treatment. The role of radiotherapy in the treatment of early stage ALCL remains unclear due to lack of prospective studies. So do the optimal chemotherapy regimens and hematopoietic stem cell transplantation. The paper will review these issues.

Objective: To summarize the clinical application of reservation of partial duode- num in pancreaticoduodenectomy. Methods: 21 patients of pancreaticoduodenectomy were per- formed with partial duodenum, trans-ligment of Treits and upper-jejunum reservation from June 2000 to June 2005 in our hospital and the clinical data were retrospectively analyzed. Results: There was no death in operation; 2 cases with incision split due to infection after surgery were cured by secondary suture; the rest were discharged from hospital from hospital successfully; a mean period of in-hospital was 21 days, without bile leakage or pancreatic leakage. Follow-up period was from sixmonths to five years, and there were 18 cases with 1-year survival (85.7%), 14 cases with 2 - year survival(66.6%) and 3 cases with 3-year(14.2%). Condusion: Reservation of partial duodenum can reduce trauma caused by extensive anatomy and shorten operation time.

OBJECTIVE:To provide reference for the standardization of the instructions of felodipine.METHODS:The instructions of felodipine in China were compared with the national regulation as well as foreign instructions of felodipine.The problems of Chinese instructions of felodipine were analyzed to put forward revision measures.RESULTS & CONCLUSIONS:The problems of Chinese instructions of felodipine are items shortage or missing,lacking of warnings for drug safety.It is suggested that related administration departments and enterprises should revise and improve the instructions of felodipine.

Liver functional reserve which functioned as the major evaluation factor for the tolerance of the liver surgery or the liver trauma had already been used wildly in preoperative evaluation for liver surgery.So far there were plenty of techniques for liver functional reserve.But each technique had its own advantages and disadvantages.This article briefly discussed the most wildly used techniques for liver functional reserve in preoperative evaluation.

It is now popularized that the multi-media and network technology are applied in the field of college English teaching.This essay discusses the characteristics of multi-media network auxiliary English teaching and the cultivation of independent learning ability.

Objective To explore the value of video-assisted thoracoscopic thymectomy in the treatment of myasthenia gravis. Methods A prospective randomized controlled study,fifty-four patients who were preoperatively confirmed to be MG from 2005 to 2008 were divided into VATS group (27 cases) and conventional thoracotomy group (27 cases). Thymectomy and dissection of all fatty tissue anterior to the pericardium were performed in both the groups. The operation time,blood loss,chest drainage time,hospital stay and outcomes of the two groups were recorded and compared. Results In the VATS group,one patient was converted to open surgery because of electrocoagulation injury and brachiocephalic vein. Compared with the conventional group,the patients in the VATS group had fewer blood loss [(43.0?5.2) ml vs (117.6?17.2) ml,t=-21.196,P=0.000],shorter operation and postoperative drainage time [(89.4?15.0) min vs (98.4?12.5) min,t=-2.377,P=0.021;and (2.2?1.6) d vs (4.2?1.3) d,t=-5.003,P=0.000,respectively],and shorter postoperative hospital stay [(7.0?1.2) d vs (11.0?2.5) d,t=-7.379,P=0.000].In the conventional group,3 patients developed MG crisis and 9 had lung infection,while none of the VATS group developed the crisis,and 2 showed lung infection (P=0.236; ?2=5.295,P=0.021). The 54 patients were followed up for 6 to 24 months with a mean of 18.6 months,during the period,the MG symptoms were improved in 80.8%(21/26)of the patients in VATS group and 85.2% (23/27)in conventional group (Z=-0.126,P=0.899). Conclusions Extended thymectomy by VATS is safe and feasible with the advantage of less invasion,less surgical trauma and pain,lower rate of complication,and good curative effect.