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.

Thrombosis disease is a common complication of cancer. Researches show that venous thromboembelism ( VTE ) can increase the mortality rate of patients with cancer.Tissue factor (TF) and cancer procoagulant (CP) and other pathological factors are related to VTE.VTE risk factors in cancer patients can be grouped into 3 general categories:patient-related factors,cancer-related factors and treatment-related factors.Assessment of risk factors and early prevention can reduce the incidence of VTE.Immediate treatment and chronic therapy should be performed immediately after the diagnosis of VTE.

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 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.

Objective:To classify the types of radiographic feature of central salivary gland type malignant tumors of the jaws(CSMTJ) with studying the clinical data,radiographic characteristics and pathological classifications,and to help the diagnose CSMTJ correctly and effectively in future. Methods:The clinical,radiological and pathological features of 62 cases of CSMTJ were studied and summarized. Among these cases,52 cases were collected from the medical records of patients of the internal databank according to the World Health Organization (WHO) Histological Classification of Salivary Gland Tumors standard and 10 cases of CSMTJ were from Guangdong Provincial Stomatological Hospital. Results:The maxilla to mandible ratio was 1∶1.7,Among CSMTJ cases,central mucoepidermoid carcinoma of the jaws (CMCJ) was the most common pathological type and the second was central adenoid cystic carcinoma of the jaws(CACCJ). 62 cases of CSMTJ could be classified into four types:Unicystic type,Multicystic type,"Flame shape" type and Osteolytic type. Conclusion:The radiographic presentation of CSMTJ was very complicated and its classification was connected with tumor malignant degree. The classification was very helpful to determine the treatment methods and the prognosis of the CSMTJ.

OBJECTIVE To evaluate the changes in serum levels of TNF-? and IL-6 after thoracic operation,and the relationship with multiple organ dysfunction syndrome(MODS).METHODS Serum levels of TNF-? and IL-6 in 46 cases of benign disease between 2000 to 2003 were observed on the day of operation,the 3rd,7th,14th and 21th days after operation,the patients were divided into two groups according to the presence of MODS.RESULTS Cases occurred MODS in two weeks after thoracic operation,the serum levels of TNF-? and IL-6 were markedly higher in MODS than that in non-MODS,the correlation coefficients between them were r=0.9145,P

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.

Objective To search for novel antifungal structures produced by marine fungus.Methods Using the Pyricularia oryzae (P.oryzaae) bioassay method to screen marine fungi led to the isolation of marine fungus 09-1-1-1.Active metabolites were isolated from the broth of the marine fungus 09-1-1-1.Results Two compounds were extracted from the mycelium and their structures were elucidated by various spectroscopic methods as 3a,12c-dihydro-8-hydroxy-6-methoxy-7H-furo\furo\xanthen-7-one(Ⅰ,sterigmatocystin) and 1,3,6,8-tetrahydroxy-2-(1-hydroxyhexyl)anthraquinone(Ⅱ,averantin).Conclusion The P.oryzae bioassay was a cheap,quick and convenient method for the screening of bioactive substances from marine fungi.Compound Ⅱ showed potent inhibitory activity to the mycelia of P.oryzae.The MIC of compound Ⅱagainst P.oryzae was 1.6 ?g?mL~(-1).

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.