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.

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.

The causes of iatrogenic injury in choledo-cho-pancreatico-duodenai junction include iatrogenic factors, anatomic factors and pathological factors. T-tube, methylthionine chloride and fiber choledochoscopy are useful methods for early diagnosis. Accurate exploration of the injury site and reasonable choice of management were significant in dealing with the iatrogenie injury and can lead to a satisfactory result. Choledo-chojejunostomy and Oddi sphincteroplasty are not recommended unless the patients had distal bile duet stricture or the stones can not be removed. Accurate detection of the injury site, evaluation of the severity, and proper choice of the surgical method are important for the prognosis of the patients.

Objective To explore the diagnosis and re-operation of the recurrent retroperitoneal soft tissue sarcoma(RPS).Methods Clinical data of 25 patients with recurrent RPS were retrospectively analyzed.Results All of 25 cases of recurrent RPS were diagnosed by operation and postoperative pathologic examination.They underwent a total of 42 time operations,with mean time of re-operations was 1.68.Of the 42 time operations,26 time were complete resection,11 time partial resection and 5 time only biopsy.A total of 19 cases underwent removal of contiguous intra-abdominal organs and 5 underwent resection of major invaded blood vessel.The 1-,3-and 5-year survival rate of recurrent RPS was 52.0%,40.0% and 28.0% respectively.The rate of complete resection in recurrent RPS with major blood vessel invasion was 35.7%.In contrast,the complete resection rate in recurrent RPS without major blood vessel invasion was 75%(P=0.013).On the other hand,there was no significant difference in complete resection rate between the recurrent RPS with adjacent organs invasion and the recurrent RPS with no adjacent organs invasion(P=0.462).Conclusions Preoperative imaging results are crucial factor for assessment of operative resectability.Unless there are obvious comtraindications,recurrent RPS should undergo aggressive surgical excision.

0.05).Histological type,gross type,tumor site,lymph node metastasis and depth infiltration were related to the prognosis of gastric cancer(P

Objective To evaluate the urethra lengthening technique in the management of stricture of the external orifice and navicular fossa of urethra. Methods From June 1995 to April 1999,15 cases of severe stricture of the external orifice and navicular fossa of urethra were treated by the urethra lengthening technique and the therapeutic effect was evaluated. Results The patients have been followed up for 3～36 months with a mean of 21 months.Satisfactory results with normal voiding and good apearence of the penis have been observed in all but 2.Slight stricture of the external orifice has been noted in 2 patients and has been relieved after 3～6 times of urethral sounding. Conclusions Urethra lengthening technique yields a high success rate with consistant normal voiding and is compliant with the normal anatomy and physiology of the urethra.

Objective To study the histopathological changes of skin after phenol chemical peeling.Methods Immunohistochemical staining (ABC method) and transmission electron microscopy were used to investigate the skin of changes 15 cases of freckle.Results After phenol chemical peeling,the melansomes in basal layer cells decreased dramatically, the elastic and collagenous fibers in dermis enlarged ,and the skin thickened .Conclusions After phenol chemical peeling,the melansomes in cuticular layer cells decrease remarkably and the freckle disappears.The proliferative activitities of fibroblasts in dermis have been improved,resulting in thickness of elastic and collagenous fibers in dermis. It may be the reason why the skin delays to be aged.

In recent years, along with the in-depth research of Y-STRs, a lot of new loci are found and applied in forensic medicines. To solve some questions, the International Society of Forensic Genetics have published two guidance and recommendations successively concerning the Y-STRs in 2001 and 2005, which clarify contents regarding the term, the nomenclature principle of loci and alleles and the population genetics et al of Y-STRs. This report mainly describes the nomenclature principle of loci and alleles of Y-STRs.

Objective To investigate the effect of the prescription of Yiqiyangyin Huoxuehuayu on the mice model with viral dilated cardiomyopathy by examining the levels of serum ?-IFN.Methods Replicated the animal models of early viral dilated cardiomyopathy by injecting the CVB3m virus to the peritoneal cavity of the Balb/c mice.Two hundred and ten Balb/c mice were divided into 4 groups at random:blank group,model group,high and low dose traditional Chinese medicine group.The blank group has 30 mice.The other three group has 60 mice each.The mice of high dose traditional Chinese medicine group and low dose traditional Chinese medicine group were treated with the prescription of Yiqiyangyin Huoxuehuayu.The change of pathomorphology in the cardiac muscle tissue,the rate of death,the weight change and the cardiac weight index were observed after 4 weeks of the treatment.At the same time,ELISA method was used to exam the levels of serum ?-IFN.Results The prescription of Yiqiyangyin Huoxuehuayu can induce the expression of serum ?-IFN in the mice with viral dilated cardiomyopathy.The mean level of serum ?-IFN of the high dose traditional Chinese medicine group and low dose traditional Chinese medicine group were significantly higher than that of the model group.Conclusions The prescription of Yiqiyangyin Huoxuehuayu can inhibit the viral replication and regulate the immunologic function.

Objective To investigate the typing, staging and therapeutic outcome of intrahepatic cholan-giocarcinoma (ICC). Methods The clinical data of 60 ICC patients who were admitted to the Renji Hospital of Shanghai Jiaotong University from January 2000 to December 2008 were retrospectively analyzed. ICC was classi-fied according to the type and TNM staging proposed by the Liver Cancer Study Group of Japan (LCSGJ). The relationships between typing, staging, surgical procedures and therapeutic outcome were investigated, as well as the relationship between the LCSGJ staging system and UICC staging system. Results The 1-, 3-, and 5-year survivals of the 60 ICC patients were 44% (23/52), 19% (10/52) and 10% (5/52), respectively. The median survival time for patients with radical resection (n =30) , liver transplantation (n = 10), palliative surgery (n = 11) and exploratory laparotomy (n=9) were 13, 3, 3 and 1 months, respectively. The radical resection rates for mass-forming type, periductal-infiltrating type, intraductal growth type and mixed type were 23/31, 9/15, 5/6 and 3/8, respectively. There were significant relationships between tumor type, degree of differentiation and prog-nosis (χ2 = 8. 308, 10. 009, P < 0. 05), and between tumor type and lymph node metastasis (χ2 = 13. 261, 5.702, P <0.05). There was no significant difference in survival time between patients with different pathological types, but the prognosis of patients with mucinous adenocarcinoma was better than that in the other types, with a median survival time of 20months. The median survival time of patients in LCSGJ stage Ⅳ was 3. 0 months, which was significantly longer than that in UICC stage Ⅳ (χ2 =3. 877, P <0. 05). Tumor staging was intimately related to the macroscopic type χ2 =8.288, P <0. 05). Conclusions The prognosis of ICC is poor. The typing and staging of LCSGJ is concise and practical, which is helpful in guiding treatment and evaluating progno-sis. Surgical treatment should be applied to mucinous ICC, and liver transplantation is not recommended for the treatment of ICC.