Objective Wegener's granulomatosis is a rare multisystem vasculitis of unknown aetiology. The clinical features of the disease,the treatment and long-term follow-up is observed in this study.Methods Eleven WG patients were from Rheumatology Department of Guangdong Province People's Hospital between 1999—2005.Data were obtained retrospectively.The patients' clinical manifestation and laboratory results were studied.Results All patients had upper and lower respiratory tracts involvement.The upper respiratory tract(nose,sinus,throat,trachea,and middle ear)-(E)lung(L),and kidneys(K)were the most commonly in- volved organs.The majority of patient received i.v.puls methylprednisolone 0.5 g/d?3 d,followed by oral pred- nisone 1 mg.kg~(-1).d~(-1)and i.v.pulses of cyclophosphamide 750 mg/m~2.The long-term outcome was good. Conclusion Wegner's granulomatosis is a systemic vasculitis occurring in patients with histopathologic mani- festation of necrotizing vasculitis with granuloma formation.Corticosteroid and immunosuppressive treatment may achieve good outcome.

Pig copper zinc superoxide dismutase (CuZnSOD) is an important antioxidant enzyme. Some studies focused on the function of CuZnSOD gene, but the transcriptional regulation of the CuZnSOD gene is not yet fully elucidated. Therefore, the aims of the study were to determine the core promoter region and to explore its mechanism of transcriptional regulation. The 853 bp DNA sequence of 5'-flanking promoter was amplified by performing PCR. A series of CuZnSOD promoter fragments with gradually truncated 5'-end were produced by nested PCR and inserted into pGL3-Basic vector. The activities of the promoters were measured by the dual-luciferase assay system after transient transfection into the NIH/3T3 cells. The results demonstrated that there were 2 potential transcription start sites in the regions from initiation codon to -87 bp and -266 bp, respectively. The region from -383 bp to +67 bp in CuZnSOD gene promoter showed higher activity than other regions, and further deletion analysis demonstrated that the region from -75 bp to -32 bp contained an essential promoter sequence for pig CuZnSOD gene transcription. In addition, several potential transcription factor binding sites were predicted with bioinformatics method. These results suggest that these transcription factor binding sites may be involved in the transcriptional regulation of CuZnSOD gene.
Animals ; Cloning, Molecular ; Gene Expression Regulation ; Genetic Vectors ; Mice ; NIH 3T3 Cells ; Promoter Regions, Genetic ; Superoxide Dismutase ; genetics ; Swine ; Transfection

Colorectal Neoplasms ; pathology ; surgery ; Humans ; Liver Neoplasms ; secondary ; surgery

Objective The aim of this study was to investigate the relationship between phosphotyrosine interac-tion domain containing 1 ( PID1 ) gene and variation in intramuscular fat ( IMF ) content and the possibility to generate transgenic animals by testicular injection .Methods Expression vector pIRES2-acGFP-PID1 carrying pig PID1 gene was incubated with transfection reagents and injected into the testes of male New Zealand rabbits .We examined the F1 genera-tion by fluorescence detection , PCR, Western blotting and measuring the IMF content .The F1 generation gave birth to the F2 generation.Then we examined the F2 generation through detecting the positive rate and the IMF content .Results The exogenous PID1 gene and fluorescent protein gene were expressed at different levels both in the F 1 generation and the F2 generation, and the positive rates were 35.88%and 34.33%, respectively.The IMF content was significantly elevated (P<0.05) in the transgenic positive individuals compared with the negative ones and the control group , and the PID1 protein expression was similarly higher .Conclusions The results of this study demonstrate that PID 1 gene affects intramuscular fat content significantly .Moreover, the results of our analysis provide further evidence that transgenic animals can be gener -ated by testicular injection , and the exogenous gene can be inherited steadily .

AIM:To discuss the application value of retro-mode imaging by F-10 confocal scanning laser ophthalmoscope (cSLO) for detecting drusen in patients with age-related macular degeneration (AMD).METHODS:This was a retrospective case study.During the period of October 2015 to December 2016, 67 patients with unilateral AMD (67 affected eyes and 67 fellow eyes) were included in this study.All patients underwent color fundus photography, optical coherence tomography (OCT) and retro-mode imaging by F-10 cSLO.The features of drusen by color fundus photography, OCT and retro-mode imaging were comparatively observed in the affected eyes of patients with unilateral AMD.Positive numbers of drusen in the fellow eyes of patients with unilateral AMD detected by color fundus photography, OCT and retro-mode imaging were calculated and compared.RESULTS:Retro-mode imaging by F-10 cSLO gave easier to identify images of drusen than color fundus photography and OCT in the affected eyes of patients with unilateral AMD.In the fellow eyes of 67 patients with unilateral AMD, retro-mode imaging showed drusen in 56 cases(84%), color funds photography showed drusen in 36 cases(54%), OCT showed drusen in 48 cases(72%), the difference was statistically significant(χ2=14.31, P<0.05).The positive numbers of drusen detected by retro-mode imaging were significantly higher than color fundus photography, the difference was statistically significant(χ2=13.87, P′<0.0125).There was no statistically significant difference in the positive numbers of drusen detected by retro-mode imaging and OCT(χ2=2.75, P′>0.0125).CONCLUSION:Retro-mode imaging by F-10 cSLO provides a non-invasive technique and should be useful for detecting and monitoring drusen in AMD.

OBJECTIVETo compare the immunophenotypic and clinical characteristics between NPM1 mutated acute myeloid leukemia (AML) (NPM1m(+)AML) and unmutated AML(NPM1m(-)AML) not otherwise characterized (NOS) under similar FAB subtypes constituent ratio.

METHODSImmunophenotyping and NPM1 gene mutation type-A, B and D and other leukemic related fusion genes were detected by multiparameter flow cytometry and real time RT-PCR or PCR, respectively. 104 AML patients with NPM1m(+)AML and performed immunophenotyping assay were included, 97 with NPM1m(-)AML.

RESULTSThere were significant difference between the two groups at presentation in terms of sex, white blood count(WBC), platelet counts (PLT), blast ratio, normal karyotype ratio, WT1 expression level, FLT3-ITD mutation positive rate and remission rate of first course of induction therapy (P < 0.05). On the immunophenotype, the expression of early differentiation antigens (CD34, HLA-DR, CD117, CD38), lymphocytic antigens (CD7, CD4, CD19, CD2), myeloid and monocytic differentiation-associated antigens (CD13, CD14, CD15) were lower, and that of CD33 as well as CD123 were higher in NPM1m(+)AML patients. Among them, only CD34, HLA-DR, CD7, and CD4 positive cases were significantly lower in NPM1m(+)AML group than in NPM1m(-)AML group (P < 0.05), the rest of them had significant difference in the number of positive cells (P < 0.05). Above features were further analyzed between the M1/M2 and M4/M5 subgroups. M1/M2 cases retained the women prominent and had a higher WT1 expression level (P < 0.05). The expression of monocytic differentiation-associated antigens including HLA-DR and lymphocytic antigens were higher and that of CD117 were lower in M4/M5 subtype (P < 0.05). Among them, the positive rates of HLA-DR, CD64, CD11b, CD10, CD15, and CD4 were significantly higher in M4/M5 than in M1/M2 in NPM1m(+)AML group (P < 0.05).

CONCLUSIONThe most clinical characteristics in NPM1m(+)AML patients are consistent with reports, but some immunophenotype are different to the previous reports under similar FAB subtypes constituent ratio. The major immunophenotypic features of NPM1m(+)AML patients are lower expression of progenitor, myeloid and lymphoid lineage antigens. Monocytic differentiation-associated antigens are only higher expression in M4/M5 cases when comparison with M1/M2 cases within NPM1m(+)AML group.

Adolescent ; Adult ; Aged ; Antigens, CD ; metabolism ; Child ; Child, Preschool ; Female ; HLA-DR Antigens ; immunology ; Humans ; Immunophenotyping ; Leukemia, Myeloid, Acute ; diagnosis ; genetics ; immunology ; Male ; Middle Aged ; Mutation ; Nuclear Proteins ; genetics ; Young Adult

OBJECTIVETo explore etiologic fraction (EF) and interaction of serum hepatitis B surface antigen (HBsAg) carriage and other risk factors for primary hepatocellular carcinoma (PHC) in Wenzhou, Zhejiang, China.

METHODS1:1 matched case-control study was carried out in Wenzhou, with 180 cases of PHC and 180 controls. EF and interactions of serum positive HBsAg [HBsAg(+)] and other risk factors for PHC were analyzed by Mantel-Haenszel stratified method and conditional multiple logistic regression.

RESULTSSerum HBsAg(+), poor economic status during the past five years, preferring intake of pickled vegetables, history of PHC in their first-degree relatives, and negative life events all were risk factors for PHC, with EFs of 0.728, 0.245, 0.224, 0.084, and 0.234, respectively. There existed interactions of HBsAg(+) with other risk factors, including poor economic status during the past five years, preferring intake of pickled vegetables, history of PHC in their first-degree relatives, and negative life events, with etiologic fractions attributable to interaction [EF (A x B)] of 0.770, 0.630, 0.848, and 0.627, and indices of interaction of 0.789, 0.638, 0.852, and 0.634, respectively.

CONCLUSIONSMain risk factor for PHC in Wenzhou, Zhejiang, China could include HBsAg(+), poor economic status during the past five years, preferring intake of pickled vegetables, history of PHC in their first-degree relatives, and negative life events. HBsAg(+) plus any of the following factors, such as poor economic status during the past five years, preferring intake of pickled vegetables, history of PHC in their first-degree relatives, and negative life events, could increase the risk of PHC.

Adult ; Aged ; Aged, 80 and over ; Carcinoma, Hepatocellular ; etiology ; Female ; Hepatitis B Surface Antigens ; blood ; Humans ; Liver Neoplasms ; etiology ; Logistic Models ; Male ; Middle Aged

OBJECTIVETo evaluate the discrepancy between pre- and post-transplant diagnosis of end-stage dilated cardiomyopathy, a pre-transplantation diagnosis was compared with the diagnosis made after macroscopic and microscopic examination of the explanted hearts in 40 cardiac transplant recipients who had undergone cardiac transplantation at our institute.

METHODSPre-operation echocardiograms were obtained in all patients and coronary angiogram was obtained in 9 patients who had significant risk factors for coronary heart disease (CHD). CHD was considered present when there was a 75% reduction in cross-sectional luminal area of >or= 1 major coronary artery. Idiopathic dilated cardiomyopathy (IDC) was diagnosed when ventricular dilation and global reduction in ventricular systolic function were present in the absence of any identifiable cause. IDC patients with an alcohol consumption of > 100 g/day during the last 12 months before the onset of congestive heart failure were classified as having alcoholic cardiomyopathy. The pathological diagnosis of arrhythmogenic right ventricular cardiomyopathy was formulated in the presence of gross/or histological evidence of regional or diffuse transmural fatty or fibrofatty infiltration of the right ventricular free wall.

RESULTSBefore transplantation, 45.0%, 17.5%, 17.5% and 7.5% of patients were classified as IDC, CHD, alcoholic cardiomyopathy and hypertrophic cardiomyopathy. Post-transplant CHD diagnosis was made in all patients with a pre-transplant diagnosis of CHD. Post-transplant CHD diagnosis was also established in 4 patients with a pre-transplant diagnosis of IDC, in 4 patients with presumptive alcoholic cardiomyopathy, in 1 patient with hypertensive cardiomyopathy and in 1 patient with a pre-transplant diagnosis of aortic valve disease. Post-transplant arrhythmogenic right ventricular cardiomyopathy diagnosis was made in 6 patients with a pre-transplant diagnosis of IDC or KaShan disease. Post-transplant giant cell myocarditis diagnosis was made in 1 patient with a pre-transplant diagnosis of IDC.

CONCLUSIONPost-transplant CHD diagnosis is significantly higher than that of pre-transplant (42.5% vs. 17.5%, P < 0.05). Part of these patients might benefit from bypass surgery or PCI. Therefore, "in-depth" search for a heart failure cause, especially the coronary angiography examination, should be conducted in all heart transplantation candidates due to heart failure, regardless of their clinical presentation.

Adolescent ; Adult ; Cardiomyopathy, Dilated ; diagnosis ; pathology ; surgery ; Heart Failure ; diagnosis ; pathology ; Heart Transplantation ; Humans ; Male ; Middle Aged ; Prognosis ; Retrospective Studies ; Stroke Volume

OBJECTIVETo establish serum proteome fingerprinting predictive models and search for proteins associated with colorectal cancer.

METHODSThirty-six randomly selected colorectal cancer patients and 36 cases with hernia or gall bladder diseases scheduled for elective operation were enrolled as cancer group and control group respectively. Peripheral venous blood samples were collected before the operations. Special serum protein or peptide fingerprint was investigated by using surface enhanced laser desorption/ ionization-time of flight-mass spectrometry (SELDI-TOF-MS) measurement after blood sample had been treated with weak cation exchange protein chip (CM10) for each case. The obtained data were analyzed by Biomarker Wizard software to screen serum proteome tumor markers and set up diagnosis predictive model for colorectal cancer. Blind validation of the model with 44 healthy controls and 88 colorectal cancer patients were carried out by using Biomarker Patterns Software.

RESULTSIn comparing colorectal cancer group with control group, 5 specific protein peaks (P < 0.05) were found. The predictive model had a sensitivity of 100% and a specificity of 97.2%. A sensitivity of 71.6% and a specificity of 72.7% was got with the blind validation. The specific protein peaks with a mass-to-charge ratio (m/z) of 8908 and 13,707 showed in all the results and it showed their strong relationship with colorectal cancer.

CONCLUSIONSThe predictive models built by the differences of serum proteome fingerprint could be a very useful diagnostic tool in colorectal cancer. Proteins with m/z of 8908 and 13,707 would possibly be the tumor markers of colorectal cancer.

Biomarkers, Tumor ; blood ; Blood Proteins ; analysis ; Colorectal Neoplasms ; blood ; diagnosis ; Female ; Humans ; Male ; Middle Aged ; Peptide Mapping ; Proteomics ; methods ; Sensitivity and Specificity ; Spectrometry, Mass, Matrix-Assisted Laser Desorption-Ionization

OBJECTIVETo investigate the clinical, pathological and immunohistochemical features of vulvar intraepithelial neoplasia (VIN).

METHODSAccording to the 2004 modified terminology of International Society for the Study of Vulvovaginal Diseases (ISSVD), the cases were diagnosed as VIN from patients who had performed vulvar biopsy in Beijing Wuzhou Women's Hospital from February 2009 to December 2011, which were reclassified as usual VIN and differentiated VIN. The clinical and pathological studies were conducted respectively. MaxVision immunohistochemical staining was used to detect the expression of Ki-67, p16 and p53 proteins.

RESULTSThere were 20 cases of VIN in 237 patients, and the incidence of VIN was 8.4% in all of contemporary vulvar biopsy. In 17 cases of usual VIN, mean age was 29.6 years, the lesion typically presented with atypical cells involving almost all layers of the epithelium, which was equivalent to the high-grade squamous intraepithelial neoplasia of cervix. Immunohistochemistry for Ki-67 and p16 was strongly positive in usual VIN. High risk human papillomavirus (HPV) detection was also positive. The incidence of differentiated VIN was less than usual VIN, and there were only 3 cases in this study. In differentiated VIN, patients aged over 50 years, with mean of 53.7 years, and the lesion most commonly presented with lichen sclerosis background. There were epithelial thickening and extending, and parakeratosis, and atypia was strictly confined to the basal and parabasal layers of the epithelium where the cells enlarged with abundant eosinophilic cytoplasm, presented with prominent nucleoli, increased cellularity and abnormal keratinization. In differentiated VIN, p53 was strongly positive, Ki-67 and p16 immunohistochemical expression was confined to the basal layer only.

CONCLUSIONSVIN is a precursor of invasive squamous cell carcinoma of the vulva. The modified terminology of ISSVD classifies VIN as high-grade lesions. Definitive pathological diagnosis of VIN plays an important role in its timely treatment and the prevention of vulvar carcinoma.

Adult ; Carcinoma in Situ ; metabolism ; pathology ; virology ; Cyclin-Dependent Kinase Inhibitor p16 ; metabolism ; Female ; Humans ; Immunohistochemistry ; Ki-67 Antigen ; metabolism ; Middle Aged ; Papillomavirus Infections ; pathology ; Tumor Suppressor Protein p53 ; metabolism ; Vulvar Neoplasms ; metabolism ; pathology ; virology ; Young Adult