Objective To understand the clinical characteristics of primary small vessel vasculitis with involvement of lungs in children and analyze the causes of misdiagnosis.Methods Clinical data from 16 patients with primary small vessel vasculitis were collected and their pathological,antineutrophil cytoplasmic antibody(ANCA),pulmonary lesion manifestations,image and misdiagnosis were further analyzed.Results Among 16 cases,9 cases were microscopic polyarateritis(MPA) and 4 cases were Wegeners granulomatosis(WG) and 3 cases were Churg-Strause syndrome(CSS).The ages of onset were from 3 to 14 years old with average age of 7.5 years old.Seventy-five percent(12/16) were ANCA positive.The patients with lung involvement had cough,chest pain,dyspnea,hemoptasis and asthma.Findings on lung X-ray films were variable.Some showed multiple patch shadows,others showed single or multiple masses or nodular shadows with or without cavity formation.After the treatments with corticosteroid and cyclophosphamide,10 cases turned better;including 8 cases with normal on lung X-ray films.Three cases given up;3 cases died.Conclusion It is very difficult to make diagnosis of primary small vessel vasculitis with involvement of lungs and should be paid more attentions to this issue.

Objective To investigate the effects of silymarin on expressions of nuclear factor kappa B(NF-?B) and intercellular adhesion molecule-1(ICAM-1) in the kidneys of rats with unilateral ureteral obstruction(UUO)-induced renal fibrosis.Methods Seventy-two male Sprague-Dawley rats were randomly divided into 3 groups: sham-operated group(n=24),operated group(n=24) and silymarin treated group(n=24).Renal tubulointerstitial fibrosis model was established via UUO.Silymarin was given by gavage with 30 mg/(kg?d) in silymarin treated group,and the same volume normal saline was given by gavage in operated group and sham-operated group.In each group,8 rats were killed at 7,14 and 21 d after operation.Histological changes were observed in tubulointerstitial injury under microscope.The expressions of NF-?B and ICAM-1 in renal tissue were determined with immunohistochemical method.Results Tubuloin-terstitial injury scores in operated group at 7,14 and 21 d were 1.168?0.108,1.776?0.064 and 2.301?0.157,respectively,and Tubulointerstitial injury scores in the treated group at 7,14 and 21 d were 1.043?0.114,1.677?0.083 and 2.084?0.201,respectively.Tubulointerstitial injury scores in silymarin treated group were significantly lower than those in operated group(Pa

Objective:To investigate the gross motor function, manual ability and language communication of children with cerebral palsy (CP) and their correlation.Methods:A total of 318 children with CP (132 with spastic diplegia, 27 with spastic quadriplegia, 32 with spastic hemiplegia, 54 with dyskinesia, 41 with ataxia and 32 children with multiple difficulties) aged 4 to 12 years were classified according to the Gross Motor Function Classification System (GMFCS), the Manual Ability Classification System (MACS), and the Communication Function Classification System (CFCS). Spearman correlation coefficients were used to analyze the correlation among the three ratings.Results:Only 125 of the 318 children (39%) had the same classification level according to all three scales, showing moderate correlation and different levels for patients with different types of disability. The GMFCS and MACS levels of the subjects with spastic quadriplegia and those with dyskinesia were highly correlated. The GMFCS and CFCS levels of the hemiplegic children and those with spastic quadriplegia were also highly correlated. The MACS and CFCS levels were strongly correlated for children with spastic quadriplegia and multiple disabilities.Conclusions:The functioning of children with CP differs with their CP subtype. Correlations among the three functional assessments also differ for children with different subtypes. Combining the three classification systems provides a more comprehensive picture of the children′s ability to function in daily life.

ObjectiveTo investigate the effects of grape seed proanthocyanidin extracts (GSPE) on advanced glycation end product receptor (RAGE),NF-Κb and connective tissue growth factor (CTGF) in the myoeardium of diabetic rats.MethodsTotal 30 streptozotocin (STZ) induced diabetic rats were randomly divided into 2 groups:diabetic group (DM1,n-15) and GSPE (250 mg/ kg,i.g) treated diabetic group (DM2,n= 15).Another two control groups:normal rats(C1,n= 10)and normal rats treated with GSPE (250 mg/kg,i.g) (C2 group,n= 10) were also observed.After 24 weeks,blood was collected to measure fasting plasma glucose (FBG) and RAGE.The protein expression of NF-Κb was determined in myocardial tissue by immunohistochemical staining and Western blot.The protein expressions of RAGE and CTGF were measured by Western blot.Results The levels of FBG and RAGE were significantly higher in diabetic rats than in control rats (P＜0.05).After GSPE treatment,RAGE level significantly reduced (P＜0.05),but FBG had no change in diabetic rats.The protein expressions of RAGE,NF-Κb and CTGF in the myocardial tissue of diabetic rats had marked increase compared with control rats (P＜ 0.05),however,their levels significantly reduced after GSPE treatment (P＜0.05).ConclusionsGSPE may protect diabetic rats against cardiomyopathy,possibly by decreasing the protein expressions of RAGE,NF-Κb and CTGF.

Arrhythmogenic Right Ventricular Dysplasia ; diagnosis ; Electrocardiography ; Humans ; Male ; Middle Aged

Acute Disease ; Child ; Child, Preschool ; Female ; Humans ; Infant ; Male ; Myocarditis ; diagnosis ; diagnostic imaging ; Ultrasonography ; methods ; Virus Diseases ; diagnosis ; diagnostic imaging

Objective To investigate the expressions of apoptosis associated protein 3 (APR3) and nuclear factor 3 of activated T-cell (NFAT3) in the tissue of epithelial ovarian tumors and its correlation with the clinicopathological features.Methods 92 patients with epithelial ovarian tumor were collected,23 cases with malignant tumor,24 cases with borderline tumor,45 cases with benign tumor.The expressions of APR3 and NFAT3 were detected by immunohistochemical methods,and the differences of different types of epithelial ovarian tumor were compared.The correlation of the expressions of APR3 and NFAT3 with the clinicopathological features of epithelial ovarian tumor was analyzed.The correlation of the expressions of APR3 with the expressions of NFAT3 in epithelial ovarian tumor was analyzed.Results The positive expression rate of APR3 in patients with malignant epithelial ovarian tumors (78.26％) was significantly higher than borderline tumors (41.67 ％) and benign tumors (22.22 ％),the differences were statistically significant (x2 =5.864,7.632,all P ＜ 0.05).The expression of APR3 in patients with malignant epithelial ovarian tumors was significantly correlated with differentiation,clinical stage,lymph node and abdominal organs metastasis and ascites (x2 =7.425,7.262,8.421,5.031,all P ＜ 0.05).The positive expression rate of NFAT3 in patients with malignant epithelial ovarian tumors (56.52％) was significantly higher than borderline tumors (29.17％) and benign tumors(17.78％),the differences were statistically significant (x2 =6.829,7.547,all P ＜0.05).The expression of NFAT3 in patients with malignant epithelial ovarian tumors was significantly correlated with differentiation,clinical stage,lymph node and abdominal organs metastasis (x2 =5.253,6.367,8.021,all P ＜ 0.05).The expressions of APR3 and NFAT3 in patients with malignant epithelial ovarian tumors were positively correlated (r =0.032,P ＜ 0.05).Conclusion The expressions of APR3 and NFAT3 in the tissue of malignant epithelial ovarian tumor obviously increase,are significantly correlated with differentiation,clinical stage,lymph node and abdominal organs metastasis and are positively correlated,and it may be correlated with the development and progression of malignant epithelial ovarian tumor.

AIM: To determine the optimal operation method and reduce operative complications by retrospective analysis of outcomes of different operative manipulation for patients with congenital ectopia lentis ( CEL) .
●METHODS: A retrospective study ranged from Jan. 1, 2010 to Jan. 1, 2015 was conducted and 31 CEL patients (57 eyes) treated with different operations were taken into this study. The postoperative outcome, operation related complications was analyzed in terms of different operative manipulations.
● RESULTS: The most common types of CEL are idiopathic and Marfan - related CEL. All eyes ( 98%) benefited from operation except 1 Marchesani syndrome eye concurrent with optic atrophy induced by glaucoma. A total of 3 eyes ( 5%) were treated by phacoemulsification combined with intraocular lens implantation; 8 eyes ( 14%) were treated by phacoemulsification, capsular tension ring implantation combined with intraocular lens implantation, among these 8 eyes, 1 eye was treated with capsular tension ring implantation combined with suture fixation; 39 eyes ( 68%) were treated by lens excision and anterior vitrectomy through corneal incision combined with intraocular lens implantation with suture fixation, among these 39 eyes, 2 eyes were treated with trabeculectomy , but not combined with intraocular lens implantation; 5 eyes ( 9%) were treated by intracapsular cataract extraction and anterior vitrectomy combined with intraocular lens implantation with suture fixation; 2 eyes ( 4%) were treated by lens excision and vitrectomy through pars plana combined with silicon oil injection.
● CONCLUSION: Almost all patients can obtain satisfactory outcomes through various operative manipulations. Phacoemulsification combined with intraocular lens implantation was the first choice for patients with lens dislocation range less than 90°. Phacoemulsification, capsular tension ring implantation combined with intraocular lens implantation was recommended for 90° - 180°, and lens excision and anterior vitrectomy through corneal incision combined with intraocular lens implantation with suture fixation was more suitable for lens dislocation range more than 180°. The appropriate operative methods for different types of CEL patients is the key to success.

Background Refractive lens exchange is one of corrective surgeries for high myopic eyes and is concerned in clinic recently. Its clinical value is worthy of consideration. Objective This study was to investigate the efficacy and safety of refractive lens exchange for high myopic eyes. Methods Phacoemulsification and intraocular lens implantation was performed on 124 eyes of 65 patients with high myopia. The mean age of these patients was 51. 4±8. 57 years old,and the preoperative corrected visual acuity was 4. 11±0. 51. The mean spherical equivalent was ( -20. 17±5. 34) D. The mean axial length was (31. 33±2. 08) mm and intraocular lens power 2. 88 D. The follow-up time was 31 months. The uncorrective visual acuity, best corrective visual acuity, the spherical equivalent lens and complications were observed after operation. Written informed consent was obtained prior to the surgery. Results The uncorrective visual acuity improved after the operation in all the eyes. The uncorrective visual acuity was ≥0.5 in 15 eyes(12% ). The best corrected visual acuity improved in 114 eyes (92% ) following the surgery and that of 64 eyes (51. 6% ) was 2s 0. 5. The mean postoperative spherical equivalent was ( -2. 57 ± 1. 76 ) D in the entire follow-up duration. Posterior capsular opacification was found in 58 eyes (46. 7% ) and received laser capsulotomy. Retinal detachment occurred in 4 eyes throughout the follow-up period. Conclusion Refractive lens exchange is an effective and safe method for high myopic eyes. But preoperative fundus examination and long-term postoperative follow-up should be carried out to prevent the complications.

Objective To investigate the expression of B-cell activating factor ( BAFF ) and its specific receptor BAFF-R in patients with B-cell non-Hodgkin′s lymphoma ( B-NHL) and to analyze the cor-relations between BAFF and the development of B-NHL.Methods RTQ-PCR and Western blot assay were used to measure the expression of BAFF and its specific receptor BAFF-R in patients with B-NHL.Fluores-cence immunocytochemical staining was used to determine the localization of BAFF and BAFF-R in Raji cells, a B-NHL cell line.The expression of BAFF in tumor tissues from patients with B-NHL of different his-tologic subtypes was measured by immunohistochemistry.WST proliferation and TUNEL assays were used to evaluate the effects of BAFF and BAFF-R on the proliferation, survival rate and apoptosis of Raji cells.Lin-ear correlations between the concentrations of lactate dehydrogenase ( LDH) and the expression of BAFF and BAFF at mRNA and protein levels in patients with B-NHL were analyzed.Results BAFF and its specific receptor BAFF-R were expressed in Raji cells and played an important role in the survival and proliferation of B-NHL cell line.The expression of BAFF in tumor cells from patients with B-NHL varied with the different histologic subtypes of B-NHL.Patients with small B-cell malignant lymphoma, large B-cell lymphoma ( LBCL) , mucosa-associated lymphoid tissue lymphoma ( MALT lymphoma) and follicular lymphoma showed higher levels of BAFF, while those with mantle cell lymphoma showed lower levels of BAFF.Compared with the healthy subjects, patients with B-NHL showed significantly increased expression of BAFF at mRNA and protein levels.The levels of LDH were closely related to the expression of BAFF at mRNA and protein lev-els.Conclusion BAFF and its specific receptor BAFF-R might play an important role in the growth and survival of malignant B cells.