Adult ; Humans ; Male ; Maxillary Sinus ; Xanthogranuloma, Juvenile

12E7 Antigen ; Adult ; Antigens, CD ; metabolism ; Cell Adhesion Molecules ; metabolism ; Chondrosarcoma, Mesenchymal ; metabolism ; pathology ; surgery ; Diagnosis, Differential ; Hemangiopericytoma ; pathology ; Humans ; Lymphoma ; pathology ; Male ; Nasal Cavity ; Neuroectodermal Tumors, Primitive ; pathology ; Nose Neoplasms ; metabolism ; pathology ; surgery ; Vimentin ; metabolism ; Young Adult

Adrenal Glands ; Female ; Hematopoiesis, Extramedullary ; Humans ; Spherocytosis, Hereditary ; complications ; Young Adult

Objective To evaluate the long-term therapeutic effect of hyaluronic acid on adults with Kashia-Beck disease and the applicability of the joint dysfunction index.Methods A cluster-randomized,starch tablets-controlled trial was carried out depending on the joint dysfunction index of assessment for therapeutic efficacy on Kashin-Beck disease and self-assessment by patients of the Treatment Effect of Kaschin Beck Disease in Criterion.A total of 99 adults with Kashia-Beck disease were randomly divided into two groups:treatment group including 50 patients were intra-articular injected hyaluronic acid in knee weekly for 4 weeks,and the control group including 49 patients were treated with oral starch tablets for 3 months,2 tablets each time,3 times a day.We carried out 48 months follow-up and assessed the applicability of Kashia-Beck disease with treatment of hyaluronic acid.Results The improvement rates of joint dysfunction index were 80.0％ (40/50),83.0％ (39/47),86.4％ (38/44),85.7％(36/42),80.0％(36/45),and 47.1％(16/34) for 1 week and 1,2,3,6,48 months in treatment group,respectively,which were significantly higher than those of corresponding control group 25.0％ (12/48),28.3％ (13/46),33.3％ (16/48),25.0％ (10/40),30.0％ (12/40),and 21.4％ (6/28),and the differences were statistically significant between corresponding groups (x2=29.744,28.236,17.762,30.665,21.537,4.406; all P ＜ 0.05).Before treatment,the treatment group and control group in the joint dysfunction index scores were 7.59 ± 1.82 and 6.89 ± 1.97(t =1.837,P ＞ 0.05),respectively.After treatment,1 month follow up of the treatment group and control group in the joint dysfunction index scores were 3.44 ± 1.71 and 5.55 ± 2.34(t =4.972,P ＜ 0.01),respectively; 6 months follow up were 3.46 ± 2.78 and 6.10 ± 1.83(t =5.102,P ＜ 0.01),respectively; 48 months follow up were 5.13 ± 2.88 and 6.81 ± 3.07(t =2.219,P ＜ 0.05),respectively.The satisfaction of 85.1％ (40/47) in treatment group was significantly higher than that of 60.9％(28/46) in control group (Z =-4.012,P ＜ 0.01).Kappa value between joint dysfunction index and the overall evaluation of the treatment group after treatment was 0.526,which belong to moderate intensity of consistent degree (P ＜ 0.01).Conclusion The therapeutic effect of intra-articular injection of hyaluronic acid on adults with Kashia-Beck disease is significant and lasting,and the joint dysfunction index has better applicability to evaluate the long-term therapeutic effect.

Objective To evaluate the applicability of western Ontario and McMaster university osteoarthritis index (WOMAC) on Kaschin-Beck disease (KBD).Methods Clinical degree Ⅰ adult patients with KBD came from Yongshou and Linyou Counties in Shaanxi Province were investigated according to the historical diagnostic data and The Diagnosis Standard of Kashin-Beck Disease (GB 16003-1995),exclusion of other chronic diseases.Reliability of WOMAC was measured by retest reliability,1/2 coefficient and Cronbach'α reliability coefficient analysis; validity of the WOMAC was tested by the principal components,factor analysis and correlation analysis methods.Results Totally 200 adults patients with KBD were investigated,and 177 effective questionnaires were taken back (88.5 ％).Retest reliability was 0.754-0.853,1/2 coefficient was 0.886-0.971,and Cronbach'α reliability coefficient was 0.878-0.956.In three dimensions of WOMAC scale extracted a common factor,the cumulative variance contribution rate was 81.238％ through principal component factor.The pearson correlation coefficient between all items score of WOMAC and scale score and total score of WOMAC was more than 0.600.The differences of WOMAC score were not statistically significant in different ages,different grading of adult KBD patients (all P ＞ 0.05).Conclusion WOMAC scale used in KBD has good validity and reliability,but has low degree of differentiation.

Objective To investigate the improvement of quality of life of patient with Kashin-Beck disease (KBD) after knee joint cavity injection of hyaluronic acid.Methods Fifty KBD patients were selected in Yongshou County Shaanxi Province,and accepted knee joint cavity injection of hyaluronic acid (25 mg) for four times,one time a week.SF-36 health questionnaire was used to evaluate the quality of life of KBD patients,and the quality adjusted life years (QALYs) were calculated after 2 months.Results The total score of the quality of life of KBD patients was improved from 38.81 ± 17.39 to 49.35 ± 17.95 after knee joint cavity injection of hyaluronic acid.The scores of physiological function (PF),body pain (BP),general health (GH),energy (VT),social function (SF) and mental health (MH) in the eight dimensions of SF-36 scale were 54.42 ± 21.25,47.42 ± 20.80,48.00 ± 26.12,61.05 ± 19.14,68.09 ± 28.73 and 68.74 ± 14.85,respectively,after the treatment,which were higher than those before the treatment (41.51 ± 22.11,27.63 ± 11.78,38.76 ± 25.14,51.97 ± 18.14,57.89 ± 30.95 and 56.95 ± 20.47,t =-3.942,-6.344,-2.494,-2.785,-2.819 and-4.245,all P ＜ 0.05).The QALYs of KBD patients increased 0.051 ± 0.044 with 2 months followed up.Conclusion The quality of life (PF,BP,GH,VT,SF and MH) of KBD patients could be significantly improved after knee joint cavity injection of hyaluronic acid.

Objective:To explore the histogenesis and differentiation of dermatofibroma (DF) and dermatofibrosarcoma protuberans (DFSP). Methods: Clinical information and microscopic characteristics of 26 cases of DF and 26 cases of DFSP were investigated. The immunohistochemical study was performed on microarray sections by a panel of antibodies including FactorⅩⅢa, HLA-DR, CD34, CD14, S-100, MSA, and Ki67. Probe was labeled by in vitro transcription. The mRNA expression levels of TGF-? and bFGF were investigated by in situ hybridization. Results: All cases showed positive for Factor ⅩⅢa,HLA-DR and CD34 to different extent. The medians of positive rates in DF were FactorⅩⅢa 90%, HLA-DR 70%, and CD34 5%, and in DFSP were FactorⅩⅢa 10%, HLA-DR 5%, and CD34 80%. CD14 was positive in 3 cases of DF and 1 case of DFSP. S-100 was positive in 6 cases of DFSP and 2 cases of DF. MSA was positive in 5 cases of DFSP and 3 cases of DF. In all cases, positive rate of Ki67 was less than 5%. The mRNA expression levels of TGF-? was elevated in DF in comparison with DFSP. Conclusion: Both DF and DFSP can differentiate to dendritic cells (DC) in different degree. Considering the character of microscopic features and immunohistochemical phenotype, cells of DF are much similar to mature DC, while those of DFSP much similar to immature dermal reserve cell (DRC). The differences of cell differentiation between DF and DFSP result in different prognosis. DF is a benign tumor, while DFSP a low grade malignant tumor. The different expression of FactorⅩⅢa and CD34 may be helpful to differential diagnosis of DF and DFSP.

Objective:Since malignant fibrous histiocytoma (MFH) may be taken as an undifferentia-ted pleomorphic sarcoma (UPS), this study was conducted to reassess 33 previously diagnosed MFH cases in the past 10 years based on the latest WHO concept. And then to search for the clinicopathological features, probably tumorigenesis, and the line of differentiation of the remaining MFH/UPS cases.Methods: Thirty-three cases in tissue microarray were studied by immunohistochemistry with panels of neurogenic, myogenic, and lipogenic antibodies. Three expertise pathologists reevaluated the slides separately. Results: Among the 33 cases, 17 cases (51.5%) of MFH had their diagnoses changed, including 5 leiomyosarcomas, 3 malignant peripheral nerve sheath tumors, 1 fibrosarcoma, 1 inflammatory myofibrosarcoma, 1 giant cell tumor and 1 angiomatoid fibrous histiocytoma. The remaining 16 cases (48.5%) were finally diagnosed as MFH/UPS, among which patients were mainly old adults (median age: 63 years; range: 38 to 76 years). The median tumor size was 6.0 cm (range: 3.0 to 14.0 cm), 8 cases (50%) located in lower limb and 5 cases (31.3%) located in thigh. These tumors had marked cytological and nuclear pleomorphism. Immunohistochemistry showed that Vimentin was strongly positive in all 16 MFH/UPS (100%), Muscle-specific actin was variously positive in 8 cases (50%) and 1 case focally expressed Desmin. Eleven cases (68.8%) variously expressed CD68 (KP1) and 7 cases (43.8%) expressed CD68 (PG-M1), which were much higher than leiomyosarcoma, malignant peripheral nerve sheath tumor and liposarcoma with significant difference. Moreover, Ki67 expression rates were from 10% to 100%, including 14 cases more than 50% and 11 cases more than 70%. However, only 2 cases (12.5%) showed P53 positive. Conclusion: MFH/UPS often show marked histological pleomorphism, and the diagnosis must be made by exclusion of other definitive sarcomas, especially myogenic and neurogenic sarcoma. Only Vimentin was always expressed in MFH/UPS, while some of the tumors were positive for myogenic antigen and CD68. It was suggested that MFH/UPS might arise from primary mesenchymal cells, and some cases exhibited fibroblastic and/or myofibroblastic features. In addition, histiocytic phenotypic marker did have more expression in MFH/UPS than in other sarcomas. MFH/UPS still had certain clinicopathological characteristics.

The biochemical mechanism of degrading keratins by S.fradiae var S-221 was primarily studied.The compounds (Na_ 2 SO_ 4 , Na_ 2 SO_ 3 and sulfdryl acohol), which respecitively enhance specific activity of keratinase, activate keratinase intensively and mainly act on the disulfide bonds reductase in the keratinase, Na_ 2 SO_ 3 activates intensively both disulfide bonds reductase and polypeptide hydrolytase at 0.01 mol/L, whereas Na_ 2 S_ 2 O_ 3 , which acts on the disulfide bonds reductase, inhibits keratinase.On the condition that substrate, keratins exists, S.fradiae var S-221 is induced to produce exo-keratinase, which is a multiproteinase, containing disulfide bonds reductase, which is a key enzyme degrading keratins, then, with polypeptidic, hydrolytase, graduately hydrolyzates denatured keratins into polypeptides, oligopeptides and free amino acids, so that keratins have been decomposed completely.Sulfur in the keratins was transferred into sulfhydryl compounds, H_ 2 S and sulfates in the course of keratinolysine.