ObjectiveTo further understand the clinic manifestations of childhood primary Sjogren's Syndrome(pSS) and enhance early diagnosis. MethodsFive cases of pSS from Renji Hospital, Shanghai, were reported and their clinical features were analysoed. And literatures from Medline database and Weipu database were reviewed and discussed. Results①Childhood pSS had various clinic presentations that were non-specific and sicca symptoms were absent or occur late in most cases. ② The most common presentations were recurrent parotiditis and cutaneous manifestations with various locations and forms. ③ American-European Criteria for SS were not suitable for the diagnosis of childhood pSS. ConclusionRecurrent parotiditis and cutaneous manifestations in children can be used as clues for the diagnosis of childhood pSS but needs to be further confirmed by the positive results of salivary gland biopsy and autoantibodies examination, particularly SSA/SSB.

Objective To evaluate the applicability of small molecular markers of nephrotoxicity that in prediction of drug toxicity.Method Extracts of five kinds of traditional Chinese medicines (Tripterygium wilfordii,Strychni semen,Aristolochiafangchi,Rhei Radix et Rhizoma,and Xanthium sibiricum) that had known as nephrotoxicity were ig given to rats to establish renal injury models,and the blood samples were collected after administration for 1 and 7 d.Then blood samples were analyzed by UPLC/Q-TOF-MS for five kinds of small molecule biomarkers-thymidine,lyso-phosphatidylcholine (LPC 16:1),LPC (18:4),LPC (20:5),and LPC (22:5).The support vector machine (SVM) prediction model was established to determine the toxicity.The levels of Cr and BUN in serum were determined by automatic biochemical analyzer.The rats in each group were sacrificed after blood collection,and the kidneys were taken for HE staining.Result No toxicity was observed in the control group,and the biochemical test results showed no renal injury after mentioned five kinds Chinese herbs were given for 1 d,while SVM model of nephrotoxicity had been found abnormal.After administration for 7 d,the results of SVM model show renal toxicity,which were consistent with biochemical and pathological examination.Conclusion Metabonomics combined with the earlier established SVM model enabled prediction of drug nephrotoxicity more sensitively,quickly and \ccurately,and it is of great significance for the discovery of drug toxicity as well as the prevention and treatment of drug-induced renal injuries in clinic.

Objective To prospectively compare the clinical effect of 131I therapy for Graves disease (GD) using the 131I-iodide dose determined by radioactive iodine uptake formula and by individualized experience method respectively.Methods (1) A total of 527 GD patients referred for 131I therapy were enrolled and divided into two groups using interval sampling method.Group 1 consisted of 241 patients with their 131I activity calculated by iodine uptake formula.Group 2 consisted of 286 patients with their 131I activity calculated by individualized method based on clinical experience.(2) The patients who were not cured for the first time were retreated after 3 months in the same way until remission.(3) All patients were followed for more than 1 year after GD was cured.The clinical outcome was compared between the 2 groups.x2 test and two-sample t test were used for data analysis.Results There were no significant differences in age,gender,disease course,ATD pretreatment,the time of ATD discontinuation,level of thyroid hormone and autoantibody before 131I therapy,131I uptake rate,size of thyroid and duration of follow-up between the two groups (t=0.156-1.430,x2 =0.159,all P＞0.05).Group 1 had less 131I dose than group 2 ((247.9± 107.3) MBq vs (329.3±177.6) MBq,t=6.102,P＜0.05),fewer patients whose disease was controlled at early stage (x2 =25.279,P＜0.05) and lower remission rate for the first time of treatment (x2 =13.074,P＜ 0.05),but higher repeated treatment rate (t =2.735,P＜0.05) and ratio of hypothyroidism to normalized patients at the first treatment (x2=10.190,P＜0.05).The number of patients with permanent hypothyroidism between the two groups had no statistically significant difference (x2=1.138,P＞0.05).Conclusions The first treatment dose of 131I by individualized experience method is slightly higher than that by radioactive iodine uptake formula.Individualized treatment method for GD based on experience might help to control the GD earlier and improve the one-off remission rate without increasing the rate of hypothyroidism.

Objective To explore the pathogenic genes relevant to Behcet's disease (BD) by building the differentail gene expression profiles of peripheral blood leukocytes in BD. Methods Oligonucleotide gene array from Affymetrix Company was applied to study the differed expression levels of whole genome between three age and sex matched BD patients and normal controls. Four genes, BCL6, LRAP, ICOSLG and MME, were selected to be tested for gene expression levels by real-time PCR in the groups of BD, normol controls (NC), Lupus and rheumatoid arthritis (RA) peticnts. Results ① Differential gene expression profile of BD compared to that of normal controls was built up. It contained 89 up-regulated and 57 down-regulated genes. ② Four genes mentioned above had significantly higher expression levels in active BD patients than those in NC but had lower exoression levels in stable BD patients. The expression levels of BCL6 and MME were also proved to be increased significantly in BD than in RA and SLE patients. Conclusion ① Our work shed some light on further research of the etiopathogenesis of BD. ② The expression levels of the four genes are proved to be relevant to BD the first time by us. Further analysis showes that TNF-α and IFN-γ can up-regulate the expression levels of BCL6, LRAP and ICOSLG which may be novel to BD. The MME gene is expressed on the surface of cells, which is convenient for test and may potentially be a marker for the diagnosis of BD.

Purpose To explore the molecular features of diffuse large B-cell lymphoma(DLBCL)with high expression of MYC.Methods The clinical data of 45 cases of DLBCL were collected.Immunohistochemical EnVision method was used to classify the patients into the group with high expression of MYC and the group with low expression of MYC.All samples were subjected to DNA targeted sequencing and molecular typing was performed using the LymphGen online tool.Cellular origin was determined by using the Lymph2Cx method.The correlation be-tween MYC overexpression and clinicopathological parameters was analyzed by the x2 test and Fisher precise test.Survival curves were drawn and survival-related factors were analyzed u-sing Cox univariate and multivariate regression.ResultsCases were classified into DLBCL with high expression of MYC(n=17)and DLBCL with low expression of MYC(n=28).Com-pared to the group with low expression of MYC,the group with high expression of MYC had more PIM1,MYD88,CD79B,CD58 and PRDM1 mutations(76.5％vs 28.6％,70.6％vs 32.1％,58.8％vs28.6％,29.4％vs3.6％,29.4％vs 3.6％,P＜0.05),MCD were more frequently found(58.8％vs 10.7％,P=0.001),GCB were rarely found(17.6％vs 50.0％,P=0.030).Overall survival was significantly shorter in DLBCL with high expression of MYC(P＜0.05).Cox multi-factorial analysis showed that age was an independent prognostic factor for DLBCL(P＜0.05).Conclusion Patients with high expression of MYC were frequently characterized as MCD and ABC,and PIM1,MYD88,CD79B,CD58 and PRDM1 muta-tions were common.Patients with high expression of MYC had a poorer prognosis.

Objective:To investigate the efficacy and feasibility of urethroplasty using inner prepuce graft combined with Orandi flap for the treatment of the obliterated penile urethral atresic stricture.Methods:From January 2016 to September 2019, the clinical data of 18 obliterated penile urethral stricture cases were analyzed retrospectively. All the patients were treated using inner prepuce flap combined with Orandi Flap. The average age of the patients was 62.1 years old (range 20-81 years old). Ten cases had suprapubic cystostomy before operation. The maximum flow rate was 1.6-6.2 ml/s, with an average of 4.2ml/s. The intubation general anesthesia and lithotomy position was used. The foreskin of penis was incised longitudinally. The urethra was exposed and the segment of stricture was opened longitudinally. The range of stricture length was measured with soft ruler, ranging from 2.0 to 7.5 cm, with an average of 5.0cm. After the fibrotic tissue was completely removed, the dorsal inner prepuce was incised with needed length and width for harvesting a free skin flap in order to transplant and reconstruct the dorsal urethral. The Orandi flap was used to cover the ventral urethra, and a F14-16 silicone catheter was retained. The length range of the inner prepuce graft was 2.0-7.5 cm, with an average of 5.1cm; the width was 1.2-1.8 cm, with an average of 1.4cm. The length of Orandi flap was 2.2-7.7 cm, with an average of 6.0cm; the width was 1.0-1.5 cm, with an average of 1.3 cm. The catheter was removed 3-4 weeks after operation. The patients were then followed up after 3, 6 and 12 months postoperatively and then with annual assessments for the symptoms, urinary flow rate, and urethrography or soft urethroscopy when necessary.Results:All of the 18 operations were completed successfully. The patients were followed up for 6-36 months, with an average of 22 months. There were 5 patients with terminal dripping, 4 patients with recurrent urinary tract infection within half a year after operation, and antibiotic treatment being effective. The quality of life scores at 3 months and 6 months after operation were 0.8 (0-2) and 0.6 (0-1), respectively, which were statistically significant compared with before operation ( P<0.001). Conclusions:The use of free inner prepuce flap combined with Orandi flap is an effective treatment for the obliterated penile urethral atresic stricture, especially for the patients who are unwilling or unsuitable to harvest the oral mucosa. It has the advantages of convenient harvesting and less complications.

The latest epidemiological data suggests that the situation of adult diabetes in China is severe, and metabolic diseases have become significant chronic illnesses that have a serious impact on public health and social development. After more than six years of practice, the National Metabolic Management Center(MMC) has developed distinctive approaches to manage metabolic patients and has achieved a series of positive outcomes, continuously advancing the standardized diagnosis and treatment model. In order to further improve the efficiency, based on the first edition, the second edition guideline was composed by incorporating experience of the past six years in conjunction with the latest international and domestic guidelines.