Beh?et′s syndrome (BS) is classified among variable vessel vasculitis with unknown etiology and extensively heterogeneous clinical features. It is easily missed or misdiagnosed due to the lack of a specific laboratory diagnosis index. Based on the evidence and guidelines from China and other countries, the Chinese Rheumatology Association developed the standardization of diagnosis and treatment of BS. The purposes are: (1) To standardize the detection and interpretation of key indicators for BS; (2) To standardize rational management for BS patients with topical and systemic organ involvement to reduce complications and improve outcomes.

Objective To evaluate the influence of devascularization and shunt on liver transplantation in patients diagnosed with portal hypertension. Methods Clinical data of 182 patients diagnosed with cirrhosis,portal hypertension complicated with hemorrhages caused by esophageal and gastric varices rupture undergoing liver transplantation in the Third Affiliated Hospital of Sun Yat-sen University from January 2007 to December 201 1 were retrospectively analyzed. Nineteen patients undergoing splenectomy plus pericardial devascularization were assigned into the devascularization group,5 receiving distal spleen-renal vein shunt into the shunt group,and the remaining 158 cases with no history of devascularization or shunt into the control group. Preoperative incidence of pylethrombosis,operation time,intraoperative hemorrhage volume,the maximal blood flow velocity (Vmax )of portal vein anastomotic stoma at postoperative 1 month,postoperative incidence of pylethrombosis and 3-year survival rate were statistically compared among three groups. Results In the devascularization group,preoperative incidence of pylethrombosis was significantly higher compared with that in the control group(P<0.01).Compared with the control group,operation time of liver transplantation in the devascularization and shunt groups was significantly longer (both P<0.05 ). The incidence of pylethrombosis at postoperative 1 month was considerably enhanced in the devascularization group (P <0.05 ). The 3-year survival rates of devascularization group and shunt group were dramatically decreased compared with that of control group (both P<0.05 ). Intraoperative hemorrhage volume and Vmax of portal vein anastomotic stoma did not significantly differ among three groups (all P>0.05 ). Conclusions The medical history of devascularization or shunt will not cause severe difficulty or surgical risk to subsequent liver transplantation in patients with portal hypertension.

Objective To explore the effect of tumor necrosis factor (TNF)-α inhibitors therapy on bone mineral density (BMD) in active rheumatoid arthritis (RA) patients with low bone mass.Methods Sixtytwo active RA patients with low bone mass were treated with a standard treatment of calcium carbonate 0.5 g/d and alfacalcidol 0.25 μg/d,and were divided into two groups.Patients of the control group were treated with methotrexate 10 mg per week,while patients of the experimental group were treated with combined recombinant human type Ⅱ tumor necrosis factor receptor-antibody fusion protein 50 mg per week or adalimumab 40 mg/2 week subcutaneously for 12 months with methotrexate.BMD of lumbar spine (L2-4),femoral neck,trochanter and Ward's triangle region by dual energy X-ray absorptiometry (DEXA),as well as the bone turnover markers serum C telopeptide of type-Ⅰ collagen (CTX-Ⅰ) and serum procollagen type-Ⅰ N propeptide (PINP) were measured by enzyme-linkedimmunosorbent assay (ELISA) in both groups at the baseline,treatment for six-month and twelve-month.T test and Chi-square test was used to process the data.Results ① After 6 months of treatment,the BMD of lumbar spine,femoral neck and trochanter in the group with TNF-α inhibitors were higher than the control group [(0.68±0.08) g/cm2 vs (0.65±0.06) g/cm2,t=2.269,P=0.027; (0.63±0.08) g/cm2 vs (0.58±0.09) g/cm2,t=2.111,P=0.040; (0.61±0.10) g/cm2 vs (0.56±0.07) g/cm2,t=2.203,P=0.032; respectively].And after 12 months,the BMD of all regions were significantly higher thanthe control group [spine,(0.68±0.07) g/cm2 vs (0.62±0.08) g/cm2,t=5.115,P=0.000; femoral neck,(0.63±0.08)g/cm2 vs (0.56±0.08) g/cm2,t=3.475,P=0.001; Ward's triangle region (0.60±0.08) g/cm2 vs (0.56±0.08) g/cm2,t=2.309,P=0.025; trochanter,(0.61±0.10) g/cm2 vs (0.53±0.08) g/cm2,t=3.254,P=0.002; respectively].② Compared to the baseline,BMD of lumbar spine was significantly decreased in the control group after 12 months.While in the group of TNF-α inhibitors,BMD of lumbar spine was increased[(0.66±0.08) g/cm2 vs (0.68±0.07)g/cm2,t=3.411,P=0.001].③ Compared to the baseline,CTX-Ⅰ,a marker of bone resorption was significantly decreased at 6 months and 12 months in the group with TNF-αinhibitors [6 months,(0.33±0.2) ng/ml vs (0.46±0.22) ng/ml,t=5.548,P＜0.01; 12 months,(0.31±0.21) ng/ml vs (0.46±0.22) ng/ml,t=5.974,P＜0.01],while this decline was not found in the control group.PINP,a marker of bone formation was stable in both 2 groups during the study.Conclusion In active RA patients with low bone mass,loss of BMD in the spine and hip can be arrested by the treatmentof TNF-α inhibitors.

Objective To investigate the effect of preoperative portal venous thrombosis on liver transplantation for patients with end-stage liver cirrhosis.Methods Clinical data of 182 patients with end-stage liver cirrhosis undergoing liver transplantation at the Organ Transplantation Center of the Third Affiliated Hospital,Sun Yat-sen University from January 2007 to December 2011 were retrospectively studied.Thirteen patients complicated with portal venous thrombosis (3 patients were in Yerdel gradeⅠ,6 were in grade Ⅱ,2 were in grade Ⅲ and 2 were in grade Ⅳ)were divided into the portal venous thrombosis group.Other 169 patients without portal venous thrombosis were divided into the control group. The intra-operative and postoperative conditions of patients were compared between two groups.Results Compared with the control group,there were longer operation time,more intra-operative blood loss in the portal venous thrombosis group and the patient with Yerdel grade Ⅲ-Ⅳ.There was significant difference (both in P <0.05).At one month after transplantation,one patient (8%)in the portal venous thrombosis group and three patients (2%)in the control group developed portal venous thrombosis,and there was significant difference (P <0.05).Three-year survival rate of the portal venous thrombosis group was 46% (6 /13)and that of the control group was 84%(142 /169),and there was significant difference (P <0.05).Conclusions Portal venous thrombosis of gradeⅢ and Ⅳ may significantly increase the difficulty and risks of liver transplantation.However,the good curative effect may also be obtained only when the portal venous thrombosis is strictly assessed ,and the rational portal venous reconstruction method is used during the operation.

ObjectiveTo discuss the curative effect and initial experience of liver transplantation (LT) from organ donation by citizens after death.MethodsClinical data of 63 donors and 63 recipients who underwent LT from organ donation by citizens after death in the Third Affiliated Hospital of Sun Yat-sen University from July 2012 to April 2014 were analyzed retrospectively. The informed consents of donor family members and recipients were obstained and the ethical committee approval had been received. Among the donors, 50 were males and 13 were females with the age ranging from 3 to 57 years old and the median of 29 years old. According to the category for China organ donation after cardiac death, 27 donors were donation after brain death (DBD, C-Ⅰ), 32 were donation after cardiac death (DCD, C-Ⅱ), and 4 were donation after brain and cardiac death (DBCD, C-Ⅲ). Among the recipients, 53 were males and 10 were females with the age ranging

Objective To investigate the risk factors, prophylaxis and treatment of de novo malignancies following liver transplantation (LT). Methods Clinical data of 9 patients with de novo malignancies out of 416 patients who underwent LT and received complete follow-up in Liver Transplantation Center, the Third Affiliated Hospital of Sun Yat-sen University from October 2003 to December 2008 were analyzed retrospectively. Seven patients were male and 2 were female. The age ranged from 30 to 60 years old with a median of 57 years old. Four cases had smoking history before operation, 3 cases had long-term passive smoke exposure, and 1 case had family history of hepatocellular carcinoma. The informed consents of all patients were obtained and the ethical committee approval was received. Allogeneic modified piggyback LT was performed in all the patients. The immunosuppressive regimen was methylprednisolone + tacrolimus (FK506) or cyclosporine A (CsA). The patients were followed up after operation and the incidence, treatments and prognosis of malignancies were observed. Results The incidence of malignancies following LT was 2.2% (9/416) including digestive system malignancies (n=3), respiratory system malignancies (n=3), hematological malignancies (n=2) and soft tissue sarcoma (n=1). The elapsed time from LT to diagnosis was 10 to 73 months (median:49 months). The patients received surgery, radiotherapy, chemotherapy accordingly. After the operation, the immunosuppressant of 5 patients changed to sirolimus and 4 patients continued to take FK506 half the dose before tumor diagnosis. Six cases died of tumor progress and multiple organ failure during the follow-up. The interval time from diagnosis of tumor to death ranged from 2 to 25 months (median: 9 months). Conclusions Smoking and use of immunosuppressant may be high risk factors of malignancy following liver transplantation. Normative follow-up and prophylaxis and treatment at the earlier stage are the keys to improve the therapeutic effect.

Objective To analyze the clinical features,diagnosis and treatment of fibromyalgiav syndrome (FMS).Methods Retrospectively analyzed 52 cases of FMS treated in our hospital between July,2008 to July,2011,and its clinical features,laboratory examination results,treatment and prognosis were analyzed.Results The ratio of female and male patients was 9∶1 in 52 FMS patients.The diagnosis was delayed for four years in average.Pain of FMS was diffuse and tenderness was symmetrical.Fatigue and sleepdisorder were the most common symptoms.There were no specific laboratory tests for this condition.Symptoms of 21 patients (57％) were alleviated among 37 patients who received drug treatment only,and comparatively,the symptoms of 12 patents (80％) were alleviated among 15 patients who received combined drugs and non-drug treatment.Conclusion The incidence of FMS is high and its clinical features vary.Clinician should pay much attention to the disease.Because the study sample size is small,so further study is needed to demonstrate the superiority of the combined therapy.

Objective To compare the efficacy of percutaneous and endoscopic treatment for the biliary stricture(BS) after liver transplantation (LT).Methods The result of percutaneous transhepatic cholangiography (PTC) and drainage ( PTC group) and endoscopic retrograde cholangiopancreatography (ERCP group) for the BS in 132 post-LT patients were analyzed retrospectively.Ninety-nine patients received PTC treatment,and 59 patients received ERCP treatment,26 patients converted to PTC treatment because of the poor efficacy or failure of the ERCP treatment.The operation success rate,complication rate,cure rate and remission rate of the two groups were compared with X2 test.Results The BS types of PTC and ERCP group were different significantly( P ＜ 0.01 ),with more non-anostomotic stricture in PTC group and more anostomotic stricture in ERCP group.The operation success rate of PTC group was higher than of ERCP group( 100％ vs 97％ ) (P ＜0.01 ),and the complication rate of PTC group was lower than of ERCP group.The overall cure and remission rate of PTC and ERCP group were not different significantly(32.3％ vs 45.8％,94.9％ vs 88.1％ ) (P ＞0.05).The cure and remission rate of PTC and ERCP treatment for each subtype of BS were not different significantly ( P ＞ 0.05 ).Conclusions The efficacy of PTC treatment for the post-LT BS is equivalent to that of ERCP treatment.PTC can be considered the first-line option for the post-LT BS.

Objective To investigate the occurrence and prevention measures of long term complications in long term survival recipients after liver transplantation.Methods In the recipients undergoing liver transplantation from Sept. 2003 to Dec. 2004,by Nov. 30,2011,there were 62 cases with the survival time more than seven years.The clinical data and follow up examination results of these 62 cases were retrospectively,including weight,blood pressure,blood sugar,blood lipids,and liver and kidney functions. The incidence of long-term complications was statistically tested.Results Postoperative metabolic complications including overweight or obese occurred in 21 cases (33.9％), new onset diabetes in18 patients (29％), hyperlipidemia in 17 cases (27.4％),hypertension in 9 cases ( 14.5 ％ ),and kidney dysfunction in 12 patients ( 19.4％ ).The incidence of diabetes and hyperlipidemia in the patients with overweight and diabetes (respectively 52.4％ and 42.9％) was significantly higher than in the normal weight group (respectively 17.1 ％ and 19.5 ％)(P＜0.05).In 58 recipients with primary diseases of hepatitis B-related liver diseases,one case had hepatitis B virus reinfection. In 17 recipients with primary disease of primary liver cancer,tumor recurrence occurred in 2 cases.During the follow up period,4 patients received liver re-transplantation due to hepatic artery stenosis (1 case) or biliary complications-induced loss of the transplanted liver function (3 cases).Conclusion The major complications of the long term survival recipients after liver transplantation are metabolic complications and primary disease relapse. Postoperative long-term follow up and monitoring of recipients is recommended to prevent and treat a variety of long-term complications.

Objective To study the related factors associated with the reversal of posttransplant diabetes mellitus (PTDM) following liver transplantation. Methods The clinical data of 62patients with PTDM in 232 patients receiving liver transplantation (26. 7 %) were retrospectively analyzed and the patients were divided into two groups: patients with transient PTDM (34 cases) and those with persistent PTDM (28 cases). Pre-operative and post-operative variables, including sex,age, body mass index, family history of diabetes, hepatitis B virus infection, pretransplantation fasting plasma glucose, the immunosuppressant regime, FK506 concentration and duration of steroid usage, were analyzed retrospectively. Results The variables, including sex, age, body mass index,family history of diabetes, hepatitis B virus infection, pretransplantation fasting plasma glucose,FK506 concentration at month 1, 3 and 6 after operation, rate of cyclosporine usage and duration of steroid usage had no significant difference between the two groups (P＞0. 05). Compared with the persistent PTDM patients, the transient PTDM patients were characterized by younger age at the time of transplantation (54 ± 8 vs. 42 ± 6 years, P＜0. 05), longer time before the development of PTDM (18 ± 23 vs. 35 ± 42 days, P＜0. 05), and higher rate of mycophenolate mofetil or sirolimus usage (0vs. 8. 9 %, P＜0. 05). Based on a multivariate analysis, age at the time of transplantation was determined as the single independent predictive factor associated with reversal of PTDM following liver transplantation (odds ratio: 1. 312, 95 % confidence interval: 1. 005 - 1. 743). Conclusion Age at the time of transplantation, duration before the development of PTDM and rate of mycophenolate mofetil or sirolimus usage are associated with reversal of PTDM following liver transplantation. Among these factors, age at the time of transplantation is only the single independent predictive factor.