Child ; Child, Preschool ; Humans ; Macrophage Activation Syndrome ; etiology ; pathology ; Male ; Mucocutaneous Lymph Node Syndrome ; complications ; pathology

Abdominal Neoplasms ; metabolism ; pathology ; surgery ; Actins ; metabolism ; Angiomyolipoma ; metabolism ; pathology ; Desmin ; metabolism ; Diagnosis, Differential ; Humans ; Leiomyosarcoma ; metabolism ; pathology ; surgery ; Liposarcoma ; metabolism ; pathology ; surgery ; Male ; Middle Aged ; Neoplasms, Multiple Primary ; metabolism ; pathology ; surgery ; Retroperitoneal Neoplasms ; metabolism ; pathology ; surgery ; S100 Proteins ; metabolism

Aleutian mink disease parvovirus (AMDV) causes a persistent infection associated with immune complex disease, hypergammaglobulinemia, and high levels of antiviral antibodies. Despite the presence of an antibody, the virus is not cleared in vivo. Pre-existing antibodies may enhance viral infections, by Fc-receptor-mediated antibody-dependent enhancement (ADE), but the mechanism that underlies ADE has not been fully defined. Three models have been proposed, including: (1) interactions between antibody and FcR, complement C3 fragment and CR, or between C1q and C1qR, which promotes viral attachment to cells; (2) suppression of IFN-gamma-mediated host-cell antiviral gene expression by the upregulation of negative regulators of pathogen pattern recognition; and (3) the promotion of early IL-10 secretion. In addition, the role of cytokine IL-6 in ADE mediated disease development is discussed, to facilitate a better understanding of the pathogenesis of AMDV infection, as well as give insights into rational vaccine design approaches.
Aleutian Mink Disease ; immunology ; virology ; Aleutian Mink Disease Virus ; genetics ; immunology ; Animals ; Antibodies, Viral ; immunology ; Antibody-Dependent Enhancement ; Mink ; immunology ; virology

Child ; Child, Preschool ; Complement C3 ; Disease Progression ; Female ; Humans ; Intercellular Signaling Peptides and Proteins ; blood ; Lipid Metabolism ; Male ; Obesity ; blood

Objective To explore the effect of preoperative chemotherapy for children with malignant extragonadial germinoma. Methods Twenty patients with malignant extrogonadal germinoma had been treated with preoperative chemotherapy from Mar.2003 to Mar.2007.Protocol 1 of chemotherapy was to improve PEB:(cisplatin plus etoposide plus bleomycin A5);Protocol 2 was VAC(vincristine plus actinomycin D plus cyclophosphamide) the average time span of the chemotherapy was 16 weeks.Upon completion of chemotherapy,tumor resection was performed.The content of alpha fetoprotein(AFP) was determined before operation and compared with the content before chemotherapy.Re-gular follow-up procedure was taken after operation. Results All 20 patients received chemotherapy and underwent operation.The removal rate of resection operation was 100%,the process of operation was smooth.The volume of tumors in 14 patients decreased over 50% compared with those before chemotherapy.The response rate was 70%.The content of AFP decreased significantly in 15 patients(75%).Fifteen patients had been living without tumor for up to 2 years,for 5 cases the survival time went beyond 5 years. Conclusions Malignant extrogonadal ger-minoma in children grow infiltratively,bring detrimental effects to neighboring organs,and bring obstacles to resection operation.Chemotherapy for children with malignant extragonadal germinoma before operation can reduce volume of tumor and bleeding during operation,raise the resectability rate and lower the content of AFP.It will enhance the effectiveness of operation and improve prognosis.Therefore,it can be used as a conventional clinical method.

Objective To find the relationship between mutation of gamma 2 subunit of the gamma-aminobatyric acid type A receptor(GABRG2) and generalized epilepsy with febrile seizure plus(GEFS+).Methods Probands of 10 families with GEFS+ were selected,the GABRG2 gene were sequenced.Results We found a single nucleotide polymorphism site,and did not find the reported mutations.Conclusion GABRG2 mutation is not common in Hans of northern China.

Abstract?AIM: To investigate the influence of propylene glycol mannite sulfate ( PGMS ) on the expression of tumor necrosis factor -α( TNF-α) and interleukin-1β( IL-1β) , in diabetic retinopathy by a rat model, to study the mechanism of PGMS against diabetic retinopathy, and provide a reliable theoretical and experimental evidence for the PGMS to be applied to clinical prevention and treatment of diabetic retinopathy.?METHODS: Male Wistar rats were randomized into 4 groups, normal control group, diabetic control group and PGMS in group, the PGMS in groups included the doses of 50mg/kg and 100mg/kg. 1% streptozotocin ( STZ) of 60 mg/kg was intraperitoneally injected in rats to establish the diabetic models. The PGMS with the doses of 50mg/kg and 100mg/kg were used to gavage in different groups of models for 12wk.Twelve weeks later, the animals were sacrificed and retinas were isolated. The aqueous humors and serums were taken, expressions of TNF-αand IL-1βprotein in retinas, aqueous humors and serums were detected by enzyme-linked immunosorbent assay ( ELISA) , respectively.The location and the expression of TNF-αand IL-1βprotein in retina tissue was detected by immunohistochemistry.?RESULTS: Twelve weeks after the use of PGMS, the level of blood glucose was not changed.ELISA showed that the expression of TNF-αand IL-1βprotein in serum and retina was significantly increased in diabetic control group than in normal control group(P<0.05), but in the groups which PGMS was given reduced, lower than those in diabetes mellitus( DM) group, especially as the concentration of PGMS increased ( P<0.05 ).But the levels of aqueous humor's TNF-αand IL-1βproteins in PGMS group were not reduced.Immunohistochemistry showed that the TNF -α protein was almost not expressed in normal control group. But the TNF-αprotein was highly expressed in diabetic control group. The expression mainly located in the ganglion cell layer, the inner plexiform layer, outer plexiform layer and pigment epithelium. The TNF-αprotein was weakly expressed at the group of 50mg/kg PGMS, the TNF-αprotein was almost not expressed at the group of 100mg/kg PGMS.When the normal control group was detected, the IL-1βprotein was weakly expressed in the outer plexiform layer.But the IL-1βprotein was also highly expressed in diabetic control group.The expression mainly located in the inner plexiform layer, outer plexiform layer and pigment epithelium. The IL -1βprotein was weakly expressed at the group of 50mg/kg and 100mg/kg PGMS.?CONCLUSION:PGMS can treat the diabetic retinopathy by downregulating the expressions of TNF-αand IL-1βin early diabetic retinopathy.PGMS maybe have a good control effect on early diabetic retinopathy.

Background The pathogenesiof age-related maculadegeneration (AMD) iassociated with the senility of human retinal pigmenepithelium (RPE) cells.Seeking drug to arresRPE cell senility iof significance fothe prevention and treatmenof AMD.Research showed thathe lycium barbarum polysaccharide (LBP) can delay senility,buitinfluence on RPE cell aging iunclear.Objective Thistudy wato discusthe protective effecand mechanism of LBP on RPE cell aging.MethodPorcine retinal neural epithelial layewaisolated,and photoreceptooutesegmen(POS) waextracted by density gradiencentrifugation and marked by FITC.The POwathen co-cultured with RPE cellin the medium containing 0.01,0.10 and 1.00 g/L LBP fo24 hours.The areof fluorescence,representing the amounof POphagocytosed by RPE cells,wameasured undethe fluorescenmicroscope to evaluate the influence of LBP on the phagocytifunction of RPE cells.The POS-induced RPE lipofuscin-uptake cell model waestablished by co-culturing human RPE cellwith porcine POfo3 weeks.The RPE-POco-culture cell model waincubated in medium containing 0.01,0.10 o1.00 g/L LBP,and the autofluorescence caused by lipofuscin up-taken into RPE cellwadetected with flow cytometry.cell counting kiwaused to assescell proliferation and viability (value) 24,48 and 72 hourafteculturing.ResultPorcine POpresented athin rodundethe lighmicroscope and appeared abilayedisc-like structureundethe transmission electron microscope,and itFITC-labeled yellow-green autofluorescence waobserved undethe fluorescenmicroscope.No POwaup-taken into the RPE cellin the normal control group,buthe areof POphagocytosed by RPE cellwagradually enlarged with increasing doseof LBP,showing significandifference among the group(F =21.425,P =0.006).Compared with the POcontrol group,the phagocytosed areincreased avariouconcentrationof LBP+POgroup(P＜0.01).Flow cytometry showed thathe autofluorescence value in the POcontrol group wamore highethan thaof the normal control group.Athe LBP dose increased,the autofluorescence value in the RPE celldeclined gradually and iwaneathe normal value in the 1.00 g/L LBP+ POgroup.The rate of proliferation of the lipofuscin RPE cellvaried with the increase of doseof LBP with the maximal value in the normal RPE group and minimal value in the lipofuscin RPE group,and the rate of proliferation of the lipofuscin RPE cellascended with increasing doseof LBP until neathe normal value in the 1.00 g/L LBP + lipofuscin RPE cellgroup (P＞0.05).ConclusionLBP enhance the anti-aging effecof human RPE cellby strengthening the phagocytiability to POand the ability to remove lipofuscin and by heightening the proliferation of human RPE cells.

Animals ; Cells, Cultured ; DNA ; analysis ; Flow Cytometry ; methods ; Fluorescence ; G1 Phase ; Resting Phase, Cell Cycle

Objective To study the effects of angiotensin converting enzyme inhibitor benazepri1 on apoptosis and the expression of Fas and FasL in the kidney of rats with adriamycin-indued nephritic glomeruosclerosis.Methods After uninephrectomy and the injection of adriamycin induced rats model with glomerulosclerosis, benazapril(6 mg/kg) was delivered daily by gavage to the rats in therapeutic groups for 12 weeks.Apoptosis was examined by means of terminal-deoxynucleotidyl trans ferase mediated d-UTP nick end label ling(TUNEL) and immunohistochemistry was utlized to detect the expression of Fas and FasL.Software of pathological analysis quantitated the level of Fas and FasL.Results Compared with those of the control group, the kidney of model group had moresevere glomerulosclerosis, much more apoptotic cells and higher level of exprssion of Fas and FasL. The degree of glomeruloscleroais, the nuxner of apoptotic cells and the level of expression of Fas and FasL were ameliofated by benazepril treatment.Conclusion Benazepril may suppress the excessive apoptosis of kidney cell by lowering the expression of the protin correlatng apoptosis Fas and FasL,so as to postpone the process of glomeruosclerosis.