Objective To analyze the clinical manifestations,immunological characteristics,pathological changes and prognosis in primary Sj(o)gren's syndrome complicated with renal involvement.Methods Three hundred and eighty-seven patients with pSS were enrolled in this retrospective study.Among these patients,198 patients were complicated with renal involvement and 189 patients without renal involvement.The data of the two groups were analyzed for clinical manifestations,blood tests,urine tests and immunological characteristics by independent sample t test,x2 test,Fisher exact probability and Logistic regression model respectively.Seventy-two patients in the group with renal involvement had kidney biopsies.Results The average of these 387 patients (female/male 345/42 cases) were (55.3±13.2) years old.The average age of 198 patients with renal involvement (female/male 168/30 cases) was (55.3±13.2) years old.The serum RF and IgG of the group with renal volvement was significantly higher than the patients without renal involvement.In the 198 patients with renal involvement;102 patients (51.5％) developed type Ⅰ renal tubular acidosis (RTA Ⅰ).Among these RTA Ⅰ patients,54 patients presented hypokalemia;12 patients developed hypokalemic plegia,30 patients with urolithiasis,6 patients with osteoporosis;69 patients presented with overt renal glomerulus impairment,36 cases had nephrotic syndrome (NS),and 33 cases had chronic glomerulonephritis;In 81 patients with renal failure,including 27 were stage 1 CKD,18 were stage 2 CKD,9 were stage 3 CKD,15 were stage 4 CKD,12 were stage 5 CKD.Among these patients with renal biopsie,light microscopy showed chronic interstitial nephritis (IN,69/72),mesangial proliferative glomerulonephritis (36/72),hyperplastic and sclerosing glomerulonephritis (9/72),partly sclerosing glomerulonephritis (9/72) and membranous nephropathy (6/72).Immunofluorescent examination revealed:IgG deposition in 21 patients (29.1％),IgA in 21(29.1％),IgM in 33(45.8％),C3 in 12(16.6％),C4 in 18(25％) and C1q in 15(20.8％).Sixty-six cases with renal biopsies were followed for (8-30)months,except the patient died of cerebral bemorrhage,all the other patients survived,and renal functions were steady or improved.Conclusion ① The pSS patients with renal involvement mainly shows interstitial nephritis.However,the occurrence of glomerulonephritis is not rare.② Rheumatoid factor (RF) or IgG is significantly associated with renal involvement in patients with pSS.③ When renal involvement is presents,renal biopsies is a necessary for patients with pSS.

Objective Carry out the reform of the online learning mode of prehospital training for refresher doctors , and evaluate the effect of reform in Peking Union Medical College Hospital .Methods An exploration was carried out on the refresher doctors prehospital training mode from traditional classroom teaching to online learning , Estab-lished the online learning system .Evaluated the effect of online learning by the way of questionnaire investigation andreexaminationamong289refresherdoctors.Results 289refresherdoctorshavecompletedthestructuredon-line courses of prehospital training , and the average pass rate of the first test was 42.2%.The questionnaire and retest was conducted , A total of 262 doctors completed the work , The recovery rate was 90.7%, and the average pass rate of retest was 74.9%, showing improvement by 32.7%.Conclusions Online learning mode is an effec-tive tool for knowledge dissemination , and has a better learning effect .

Chondrosarcomas has low morbidity in China,but we should pay more attention to the results it contributed to.Patients usually have poor prognosis because of defect of surgical resection and resistance to con-ventional chemotherapy and radiotherapy.Chemotherapy is a significant method to the chondrosarcoma treatment. If we overcome the difficulty of chemoresistance,chemotherapy will kill the sarcoma cells efficiently and inhibit the capacity of metastasis and invasion.The patients will have better prognosis.Finding the mechanisms of che-moresistance in chondrosarcomas is the key, now we review sevaral recent mechanisms of chemoresistance in chondrosarcoma.

Objective To explore the effects of 1α,25-dihydroxyvitamin D3 [1,25(OH)2D3] on memory CD4+T cells of focal proliferative IgA nephropathy (IgAN)patients.Methods (1) Total of twenty incipient focal proliferative IgAN patients (Lee classification:Ⅲ level) were chosen as IgAN group and 20 healthy volunteers were chosen as healthy control group.The level of serum 1,25(OH)2D3 was measured by radioimmunoassay (RIA).Peripheral blood mononuclear cells (PBMCs) were separated by the method of Ficoll density gradient centrifugation and were stimulated with anti-CD3/anti-CD28 in the absence or presence of various concentrations of 1,25(OH)2D3,Dexamethasone(DEX) and 1,25(OH)2D3and DEX combined.PBMCs were cultured for 72 hours and the levels of IFN-γ,IL-4,IL-17A,Foxp3 were measured by flow cytometry(FCM),standing for the levels of Th1,Th2,Th17,Treg.(2) IgAN group was divided into two subgroups (proteinuria ＜ 1 g/24 h subgroup,proteinuria≥ 1 g/24 h subgroup),then the serum levels of 1,25(OH)2D3,IFN-γ,IL-4,IL-17A,Foxp3 were compared.Results Compared with healthy control group,serum 1,25(OH)2D3 level of IgAN group was significantly lower (P ＜ 0.05).Serum 1,25(OH)2D3 level in proteinuria≥ 1 g/24 h subgroup was significantly lower than proteinuria ＜ 1 g/24 h subgroup and healthy control group (P ＜ 0.05).The level in proteinuria ＜ 1 g/24 h subgroup was lower than healthy control group,but the difference was not statistically significant (P ＞ 0.05).(2) The levels of IFN-γ and IL-17A and the ratios of IFN-γ/IL-4,IL-17A/Foxp3 in IgAN group increased significantly compared with healthy control group (all P ＜ 0.05),and the level of Foxp3 decreased significantly (P ＜ 0.05).The level of IL-4 also increased,but the difference was not statistically significant (P ＞ 0.05).The levels of IFN-γand IL-17A and the ratio of IL-17A/Foxp3 in proteinuria≥ 1 g/24 h subgroup increased significantly,and the level of Foxp3 decreased significantly,compared with urinary protein ＜ 1 g/24 h subgroup and healthy control group (P ＜ 0.05).The ratio of IFN-γ/IL-4 in proteinuria≥1 g/24 h subgroup and proteinuria ＜ 1 g/24 h subgroup all increased,compared with healthy control group,and the ratio in proteinuria≥ 1 g/24 h subgroup increased significantly (P ＜ 0.05).There was no significant difference in the level of IL-4 among all groups.(3) After treatment with 1,25(OH)2D3,the levels of IFN-γ and IL-17A and the ratios of IFN-γ/IL-4 and IL-17A/Foxp3 decreased significantly,and the level of Foxp3 increased significantly (P ＜ 0.05),and these effects were more obvious as the increase of the drug concentration.The level of IL-4 did not change significantly.The combination of 1,25(OH)2D3 and DEX had a synergistic inhibition on the production of IFN-γ,IL-4,IL-17A,and the ratios of IFN-γ/IL-4 and IL-17A/Foxp3,and had a synergistic promotion on the production of Foxp3.Conclusions There is a certain extent of vitamin D deficiency in focal proliferative IgAN patients,which may be associated with the severity of proteinuria.The disorder of immunomodulatory effects of memory CD4+ T cell might exist in the patients of focal proliferative IgAN.1α,25-dihydroxyvitamin D3 might have beneficial effects on the immunoregulation of memory CD4+T cells of focal proliferative IgAN patients.

OBJECTIVETo observe the effect of American Ginseng Capsule (AGC) on the liver oxidative injury and the Nrf2 protein expression in the liver tissue of rats exposed by 900 MHz cell phone electromagnetic radiation.

METHODSTotally 40 male SD rats were randomly divided into the normal control group, the model group, the Shuifei Jibin Capsule (SJC) group, and the AGC group,10 in each group. Rats in the normal control group were not irradiated. Rats in the rest three groups were exposed by imitated 900 MHz cellular phone for 4 h in 12 consecutive days. Meanwhile, rats in the SJC group and the AGC group were intragastrically administrated with suspension of SJC and AGC (1 mL/200 g body weight) respectively. Normal saline was administered to rats in the normal control group and the model group. The histolomorphological changes of the liver tissue were observed by HE staining. Contents of malonic dialdehyde (MDA), superoxide dismutase (SOD), glutathione (GSH), and glutathione peroxidase (GSH-PX)were detected by colorimetry. The Nrf2 protein expression of hepatocytes was detected by immunohistochemical assay and Western blot.

RESULTSCompared with the normal control group, hepatocyte nucleus was atrophied or partially disappeared, the contents of liver MDA and Nrf2 protein obviously increased (P <0. 05, P <0. 01); contents of liver SOD and GSH decreased (P <0. 05) in the model group. Compared with the model group, karyopyknosis was obviously attenuated and approached to the normal level in the SJC group and the AGC group. The contents of liver MDA and Nrf2 protein expression decreased (P <0. 05), and the contents of liver SOD, GSH, and GSH-PX obviously increased (P < 0.05) in the SJC group. The contents of liver MDA and the Nrf2 protein expression decreased (P < 0.05), and contents of SOD and GSH obviously increased in the AGC group (P <0.01, P <0.05).

CONCLUSIONSThe electromagnetic radiation induced by 900 MHz cell phone could affect the expression of Nrf2 protein, induce oxidative injury, and induce abnormal morphology of liver cells. SJC and AGC could promote the morphological recovery of the liver cells. Its mechanism might be related to affecting the expression of Nrf2 protein and attenuating oxidative damage of liver cells.

Animals ; Cell Phone ; Electromagnetic Radiation ; Glutathione Peroxidase ; metabolism ; Hepatocytes ; metabolism ; Liver ; Male ; NF-E2-Related Factor 2 ; metabolism ; Oxidative Stress ; drug effects ; Panax ; Plant Extracts ; pharmacology ; Rats ; Superoxide Dismutase ; metabolism

Objective To analyze and determine the clinical, molecular pathology and genetic features of a Chinese family with dystrophinopathy. Methods Clinical data of the proband and his family members were collected. Immunohistochemistry staining was performed on muscular biopsy tissues with antimerosin, emerin and the N, C and central rod domains of dystrophin. Genomic DNA was extracted using standard procedures from the peripheral blood leukocytes. Multiplex ligation-dependent probe amplification (MLPA) was used to test Duchenne muscular dystrophy (DMD) gene to determine the ways and sites of genetic mutation, and analyze the relationships between genotype and phenotype. Results Patients from this family were clinically diagnosed as muscular dystrophy, and they presented serious manifestations although the immunohistochemistry analysis for the proband exhibited partial loss of dystrophin staining, and positive expression with merosin and emerin. Further test with MLPA detected the loss of exons 45--54 in DMD gene in the proband, while his mother had heterozygositic loss in exons 45--54. Conclusions The losses of exons 45--54 in the proband are all derived from his mother, who carries genetic mutation with normal phenotype. He has been diagnosed as dystrophinopathy. At the same time, his partial loss of dystrophin is not parallel to the out-of-frame mutation of the gene and his severe clinical manifestations. Abnormal expression of dystrophin is the pathological basis for dystrophinopathy phenotype. Its clinical outcome depends not only on the degree of the protein expression, but also on the function of the sites where the DMD gene less occurs.

Batch and continuous fermentation were adopted to investigate the effect of specific growth rate and amino acid components on RNA accumulation in Candida tropicalis ATCC 20408 in fermentation medium ( FM), yeast peptone dextrose medium (YPD), molasses fermentation medium ( MFM) and FM without corn steep liquor. The data showed that obvious differences in intracellular RNA accumulation were observed at different cell growth phases in bath fermentation prosess, and RNA level reached 11. 8% (g-RNA /g-DCW) during exponential phase, and only 6.9% during stationary phases. It was also found that intracellular RNA accumulation increased with the increase of specific growth rate in continue fermentation prosess, and the highest RNA level reached 15. 6% with the glucose conversion rate of 42. 8% at the dilution rate of 0. 5 h-1. Furthermore, the data showed that RNA lever was notably increased in batch fermentation process when amino acids or peptone was added into the fermentation medium containing no corn steep liquor. Taken together, it was reported for the first time that specific growth rate and amino acid components plays a leading role on the intracellular RNA accumulation in C. tropica lis, and specific growth rate is more important.

DNA barcoding is a technique in which species identification and discovery are performed by using short and standard fragments of DNA sequences. In this study, eleven species of Paris, including seven varieties, were sampled. Five chloroplast sequences, psbA-trnH, rpoB, rpoC1, rbcL, matK, and one nuclear marker, the second internal transcribed spacer (ITS2) of ribosomal DNA, were amplified and sequenced. The PCR amplification and sequencing efficiency, intra- and inter-specific divergence and barcoding gap were used to evaluate different loci, and the identification efficiency was assessed using BLAST1 and Nearest Distance methods. The ITS2 sequences in the studied samples of Paris were amplified and sequenced successfully using primers designed by our group, while matK showed low level in the amplification and psbA-trnH was difficult for sequencing because of over 800 bp and poly (A) structure. Analysis of the intra- and inter-specific divergence and barcoding gap showed ITS2 was superior to other loci. The ITS2 showed a much higher percentage of success (100%) in identification than other five loci, none of which indicated more than 50% except matK (52.9%). The 2-locus combination of rbcL+matK didn't improve ability of authentication. In addition, the rate of successful identification with ITS2 kept 100% when the samples were expanded to 67 samples of 29 species. In conclusion, ITS2 can be used to correctly identify medicinal plants of Paris, and it will be a potential DNA barcode for identifying medicinal plants of other taxa.

Objective:To investigate the prenatal genetic testing for suspected Beckwith-Wiedemann syndrome (BWS) to improve its prenatal diagnosis rate.Methods:This study reported a pregnant woman, who had a pregnant history of termination due to the same reason at 18 weeks, with fetal acromphalus and unusually thickened placenta indicated by ultrasound examination at 13 weeks of gestation. After chorionic villus sampling, single nucleotide polymorphism (SNP) array was used to analyze copy number variations in the whole genome, and methylation-specific multiplex ligation-dependent probe amplification (MS-MLPA) was also performed to detect the methylation and copy number variations in H19 and KCNQ1 genes on chromosome 11p15. Peripheral blood samples were collected from the couple for chromosome G-banding karyotype analysis and SNP array. Results:The SNP array indicated a 176 kb heterozygous deletion in the 11p15.5 region. MS-MLPA revealed a loss of methylation at imprinting control region 2 and a 50% reduction of copy numbers of KCNQ1 (L02903) gene. No abnormality was found in the parents in the SNP array and G-banding karyotype analysis. The fetus was prenatally diagnosed with BWS. Conclusions:When intrauterine abnormalities, such as acromphalus and abnormal thickening of the placenta, are found by ultrasound during early pregnancy, prenatal genetic tests related to BWS, including MS-MLPA and SNP array, are suggested to avoid a missed diagnosis of BWS.

Abstract?AlM: To evaluate the visual impairment in cerebral palsy children with series objective indicators, and conclude their clinical features of visual function.? METHODS: Objective tests including following pursuing test, optokinetic nystagmus(OKN) drum test, refractive error examination, fundus examination, ocular deviation examination, pattern visual evoked potential ( P-VEP ) tests and brain magnetic resonance imaging ( MRl) were carried out in 43 cerebral palsy children ( 86 eyes ) with ocular visual dysfunction; The visual impairment data of the cerebral palsy children were collected, and the clinical features and possible mechanism were analyzed.?RESULTS: 1. Of the 43 cerebral palsy children ( 86 eyes) with the visual impairment presented diversified, 25 ( 50 eyes, 58. 1%) of refractive error, 24 ( 48 eyes, 55. 8%) of strabismus, 12 ( 24 eyes, 27. 9%) with nystagmus, 19 ( 38 eyes, 44. 2 %) of optical nerve atrophy or hyperplasia, 35 ( 70 eyes, 81. 4%) of VEP abnormality. Among children with spastic cerebral palsy, the incidence of visual impairment was statistically significant difference compared with other groups (P<0. 01). 2. There were 16 cases (32 eyes,37. 2%) with esotropia, 6 cases ( 12 eyes, 14. 0%) with exotropia and 2 cases ( 4 eyes, 4. 7%) with vertical deviation. Strabismus was most common in spastic cerebral palsy children, totally 13 (26 eyes, 30. 2%) with esotropia, and exotropia was common in hypotonia and other types cerebral palsy children; 3. 23 ( 46 eyes, 53. 5%) with hyperopia, 8 ( 16 eyes, 18. 6%) with myopia, 16 ( 32 eyes, 37. 2%) with astigmutism and 14 cases (28 eyes, 32. 6%) with anisometropia;4. Cerebral palsy children were usually with decreased VEP amplitude and prolong latency, and poor wave formation, mostly in spastic cerebral palsy children; 5. Visual abnormality was most common in occipital cortex damage and periventricular leukomalacia ( PVL ) . The incidence in PVL and occipital cortex had no statistically significant difference ( P > 0. 05 ), no nystagmus in patients with severe occipital cortex damage.?CONCLUSlON: Cerebral palsy children were usually with visual impairment, and presented with special clinical features; Comprehensive objective visual tests are accurate and reliable for evaluation of the visual function in cerebral palsy children.