Autoinflammatory diseases (AIDs) are characterized by abnormal activation of the innate immune system. Schnitzler syndrome, SAPHO (synovitis, acne, pustulosis, hyperostosis, and osteitis) syndrome, Yao syndrome, and PFAPA (periodic fever, aphthous stomatitis, pharyngitis, and adenitis) syndrome are rare polygenic AIDs. Although these diseases differ in etiology and pathogenesis, they all present with complex nonspecific clinical symptoms, such as periodic fever, inflammatory lesions of bones or skin, which makes diagnosis challenging and often leads to delayed treatment or even misdiagnosis. In recent years, rapid advances in molecular biology and genomics have gradually elucidated the pathogenesis of these rare diseases, offering new insights for optimizing diagnosis and treatment. This review summarizes the progress in the pathogenesis, clinical manifestations, diagnosis, and treatment of these rare polygenic AIDs, aiming to provide references for further refining management strategies for such diseases.

Objective:To establish the basic procedures of the application of optical surface monitoring system (OSMS) in the deep inspiration breath hold (DIBH) radiotherapy for patients with left sided breast cancer and compare the performance of OSMS and cone-beam CT (CBCT) in the determination of the set-up errors of DIBH radiotherapy for patients with left sided breast cancer.Methods:Twenty patients with left sided breast cancer received DIBH radiotherapy. Through the registration of CBCT images with the planning CT images, and the registration of OSMS radiography images with the outer contour of the body surface, translational set-up errors and rotational errors were determined along the lateral-medial ( Rx), superior-inferior ( Ry) and anterior-posterior ( Rz) directions. Pearson correlation analysis was performed to evaluate the correlation of the set-up errors determined by two methods, and Bland- Altman plot analysis was used to assess the coincidence of these two methods. Results:Two methods were positively correlated. The Rz volume was 0.84, 0.74 and 0.84 in the x, y and z directions, and 0.65, 0.41 and 0.54 in the Rx, Ry and Rz directions, respectively (all P<0.01). The 95% CI of agreement were within preset 5 mm tolerance (-0.37-0.42cm, -0.39-0.41cm, -0.29-0.49cm ) in x, y and z directions for two methods. The 95% CI of agreement were within preset 3 ° tolerance -2.9°-1.4°, -2.6°-1.4°, -2.4°-2.5°in Rx, Ry and Rz directions for two methods. The system errors of 20 patients with left sided breast cancer receiving DIBH radiotherapy were <0.18cm and the random errors were <0.24cm. Conclusions:OSMS is equivalent to CBCT in the determination and stimulation of set-up errors for patients with left sided breast cancer receiving DIBH radiotherapy. The combination of CBCT and OSMS is a safe and reliable method.

Objective To evaluate the necessity of arc by arc setup verification in patients with brain metastases receiving stereotactic radiotherapy (SRT) by analyzing the inter-and intra-fraction setup errors and residual errors collected from the ExacTrac X-ray portal image.Methods Clinical data of brain metastases patients treated with SRT in the previous two years were retrospectively analyzed.The ExacTrac X-ray setup images were collected after the normal setup procedure.Setup errors were calculated by registering the cranial bony structures of the ExacTrac X-ray setup images to that of the digitally reconstructed setup images.The inter-and intra-fraction setup errors and residual errors were statistically analyzed.Results Seventy-five patients from 116 lesions received 337 cycles of SRT of the head.The inter-and intra-fraction translational setup errors in the x,y and z directions were (0.93±0.86) mm and (0.15±0.59) mm;(1.83± 1.27) mm and (0.25±0.73) mm;(0.96±0.80) mm and (0.14±0.56) mm,respectively.The inter-and intra-fraction rotational setup errors in the x,y,z directions were (0.65°± 0.62°) and (0.19°± 0.40°);(0.97°±0.94°) and (0.13°± 0.25°);(0.92°± 0.71°) and (0.10°± 0.29°),respectively.The residual translational setup errors in the x,y,z directions were (0.06±0.23) mm,(0.08±0.24) mm and (0.08±0.22)mm,and (0.12°± 0.27°),(0.09°± 0.18°) and (0.06°± 0.19°) for the residual rotational setup errors,respectively.For a reference setup error threshold of 0.7 mm/0.7°,99.1％ of the SRT exceeded the threshold and required setup correction.For 1 006 non-coplanar arcs,rotating the treatment couch from 0° to the treatment angle made 66.4％ of arcs exceed the threshold and require at least once setup correction.Conclusions During SRT for brain metastasis,the inter-and intra-fraction setup errors should be emphasized.It is necessary to perform arc by arc setup error verification.

Objective To investigate the expression level of fatty acid synthase (FASN) in different grades ofgliomas and its relationship with glioma malignancy.Methods Glioma U87 and U373 cell lines were routinely cultured in vitro;immunofluorescence assay was performed to detect the cellular localization of FASN in glioma U87 and U373 cell lines.Sixty-seven human glioma samples,collected in our hospital from March 2012 to March 2013,were used in our study;grade Ⅰ was noted in three samples by WHO grading,grade Ⅱ in 29,graded Ⅲ in 22,and grade Ⅳ in 13;immunohistochemistry was performed to detect the expression levels of FASN,CD34 and Ki-67 and microvascular density (MVD) in these human glioma samples.Spearman's correlation was used to analyze the relationship between FASN expression and both Ki-67 expression and MVD in human gliomas.Results (1) FASN could express in the cytoplasm of glioma U87 and U373 cell lines.(2) immumohistochemical staining indicated that FASN mainly located in the cytoplasm,Ki-67 in the cell nucleus,and CD34 in the cytomembrane.FASN expression in samples of grade Ⅰ (3.3683±0.6549) was significantly lower than that in samples of grade Ⅱ (4.0512±0.4859,P＜0.05),and FASN expression in samples of grade Ⅲ (4.1881±0.5755) was significantly lower than that in samples of grade Ⅳ (4.6996±0.5164,P＜0.05);MVD in samples of grade Ⅱ was significantly smaller than that in samples of grade Ⅲ(P＜0.05),and MVD in samples of grade Ⅲ was significantly lower than that in samples of grade Ⅳ(P＜0.05);Ki-67 expression in samples of grade Ⅱ was significantly lower than that in samples of grade Ⅲ (P＜0.05).(3) There was a positive and significant correlation between FASN expression and MVD (r=0.606,P=0.030);a positive and significant correlation between FASN expression and Ki-67 was also noted (r=0.636,P=0.014).Conclusion High expression level of FASN indicates high proliferation capability and high blood supply in haman gliomas.

Objective To investigate the expressions of Transforming Growth Factorβ2 (TGF-β2) and Smad3 in human gliomas associated with pathologic grading. Methods The expressions of TGFβ2 and Smad3 protein were detected with SP immunhistochemistry in 80 human glioma specimens. The Kapan-Meier survival curves of progression-free survival time and overall survival time in different expression levels was compared with log-rank. Results The expression of TGFβ2 and Smad3 correlated with the pathological grading (r=0.545, r=0.570, P<0.01). Both progression-free survival time and overall survival time were significantly different between low expression group and high expression group (P<0.05). Conclusion Both TGF-β2 and Smad3 correlate well with the occurrence and differentiation of human gliomas,which help for the diagnosis, treatment and prognosis judgment．

OBJECTIVETo develop an HPLC method for the quantification of six active components in three species (Swertia davidi, S. nervosa and S. mussotii) .

METHODThe determination was performed on a Hypersil BDS colunm (4. 6 mm x 200 mm, 5 microm). Acetonitrile and 0.5% phosphoric acid solution were used as the mobile phases with a gradient elution. The flow rate was 1.0 mL x min(-1). The UV detection wavelength was at 240, 274, 325 and 334 nm. The column oven temperature was at 25 degrees C.

RESULTSix components were separated commendably in 60 minutes. The calibration curves of swertiamarin, gentiopicroside, norswertianolin, swertianolin, demethylbellidifolin and bellidifolin were in good linearity over the range of 0.520-20.8, 0.202-8.06, 0.107-4.28, 0.097-3.86, 0.094-3.77, 0.101-4.02 microg, respectively (r = 0.999 9). The average recoveries were 98.7%, 98.1%, 98.3%, 98.8%, 98.1% and 98.6%, respectively, and the RSD were less than 3.0% (n = 6).

CONCLUSIONThe method is accurate,simple and reproducible, and can be used to control the quality of Swertia.

Chromatography, High Pressure Liquid ; instrumentation ; methods ; Drugs, Chinese Herbal ; analysis ; Glucosides ; analysis ; Iridoid Glucosides ; Iridoids ; analysis ; Pyrones ; analysis ; Swertia ; chemistry ; Xanthones ; analysis

[Objective]To probe the standard of early diagnosis of femoro-acetabular impingement(FAI),and the imageological appearance of 16 row spiral CT noncontrast enhanced scan and three-dimensional reconstruction,and MRI of progression of FAI patients.And to approach the significance of using the imageology result to identity doubtful FAI patients with other patients.[Methods]Six FAI patients were enrolled.CT and MRI noncontrast enhanced scan and CT three-dimensional reconstruction were performed to assess the hip joint.The CT scan was performed on the GE Lightspeed 16 row spiral at 1.25 mm slice reconstruction.The MRI scan was performed on the Siemens Avanto 1.5T supraconduction magnetic resonance meter.The CT and MRI scan included the acetabulum to the lesser trochanters.[Results]The results of DR of pelvis orthophoria discovered subtotal congenital anatomic abnormality of hip.The result of 16 row spiral CT noncontrast enhanced scan and three-dimensional reconstruction discovered total congenital anatomic abnormality of hip.The result of MRI noncontrast enhanced scan discovered phlegmasia of hip joint of midanaphase patients of FAI.[Conclusion]Non-buninoid femoral head,hyper-deep of hip,acetabular anteversion and hypsokinesis,and typical sings and symptoms lead to early and exactitude diagnosis.The results of 16 row spiral CT three-dimensional reconstruction and MRI can provid straight reference operation schedule;The result of MRI noncontrast enhanced scan can detect FAI with early avascular necrosis of the femoral head.

0.05).Conclusion Semiconductor laser can induce apoptosis of MG-63 cells by mitochondrion pathway,and the process may be associated with the increasing of ROS.

Objective To estimate the quality of HIV 1/2 antibody labeled ELISA Kit made in China by indirect method and dual antigen sandwich method. Methods Eight kits were divided into 4 groups according to the manufacturers (indirect method and dual antigen sandwich method for the same manufacturer in one group) and were used for the determination of 100 serum samples. The positive and suspected positive results were affirmed by Western blot analysis. Results The sensibility, efficacy ratio and NPV range of indirect method were 97.97%～100%, 93.49%～98.04% and 97.05%～100%, respectively, but 2 kits failed to detect the HIV positive sample. The sensibility, efficacy ratio and NPV range of dual antigen sandwich method were 100%, 96.15%～100% and 100% without mis diagnosis. Conclusion Dual antigen sandwich method is better than indirect method in some indexes of quality.