Objective To study the prognosis of hepatoblastoma (HB) in children.Methods Retrospective analysis on the clinical data and follow-up of children with HB admitted from September 2010 to February 2015 and sum up the clinical outcome and prognosis.Results Thirty-six children with HB (23 males and 13 females) were enrolled. The median age on diagnosiswas 20 months. Allthe36 patients received follow-up, the median follow-up time was 37 months (7~71 months).(1) Of the 36 patients, 8 died. The prognosis of patients with stageⅠorⅡwas signiflcantly better than those with stage Ⅲ or Ⅳ(P<0.05). The prognosis of patients with fetal type was signiflcantly better than those with other subtypes (P<0.05). (2) Serum AFP level of 35 newly diagnosed cases increased, AFP level of survivors returned to normal after treatment. Of the 8 died patients, 3 patients had AFP elevated when relapsed, 5 cases had a persistently high AFP level after adjuvant chemotherapy. The levels of LDH, CRP, and AST at diagnosis had signiflcant influence on the prognosis (P<0.05).Conclusions Serum AFP can be a monitoring indicator for recurrence and prognosis of hepatoblastoma. POG / CCG staging, pathology classiflcation are key points when assessing the prognosis of hepatoblastoma. The prognosis of patients with high levels of LDH, CRP, and AST might be worse.

Objective To explore the relationship between X - linked spondyloepiphyseal dysplasia tarda (SEDL) gene escaping X chromosome inactivation( XCI) and SEDL phenotype. Methods RT - PCR was performed on total RNA which was isolated from blood samples of patients, female carriers and controls. Patients and female carriers were selected from the pedigree with SEDL caused by the mutation (IVS2 - 2A→C) of the gene. cDNA was analyzed by polyacrylamide gelelectrophoresis(PAGE). Results PAGE data indicateed that female carriers expressed both normal and mutant SEDL mRNA,meaning the SEDL gene escaping XCI. Family investigation showed carrier females in the SEDL pedigree presented no symptoms. Conclusions The SEDL gene escaping X chromosome in-activation is firstly identified from human body. This may explain that carrier females present no symptoms.

Objective:To investigate the effects of Helicobacter pylori on NLRP3 inflammasomes activation in THP-1 ( human monocytic cell line) -derived macrophages and evaluate the role of ROS.Methods:H.pylori strain SS1 was co-cultured with the THP-1-derived macrophages at a multiplicity of infection (MOI) of 1∶100 based on trial results with different MOIs (ratios of THP-1 cells to bacteria ranging from 1∶25 to 1∶200).The co-culture supernatants and THP-1 cells were collected at various time points (3 h,6 h,12 h,24 h) and cytokine production was quantitated using ELISA analysis.The generation of intracellular ROS was detected by FCM,and the mRNA transcript levels of NLRP3 and caspase-1 were measured by Real-time PCR.Western blot was employed to analyze the expression of active caspase-1 subunit ( p10).Then we observed the inhibitory effects of NAC and siRNA specific for NLRP3 on the ex-pression of NLRP3 inflammasome-related components and the secretion of cytokines induced by H.pylori.Results:We found that H.pylori SS1 induced IL-1βand IL-18 production in human acute monocytic leukemia cell line THP-1 in a time-and dose-dependent manner.We further showed that H.pylori could induce the mRNA expressions of NLRP3 and caspase-1 in THP-1 cells.Moreover, release of IL-1βand IL-18 from H.pylori-infected THP-1 cells was suppressed by the ROS scavenger NAC,which was an agent known to inhibit NLRP3 inflammasome formation.NAC administration also resulted in a significant decrease in the level of H.pylori-induced caspase-1 protein expression in THP-1 cells.Additionally,secretion of IL-1βand IL-18 in response to H.pylori infection was remarkably reduced by NLRP3-siRNA.Conclusion:The induction of IL-1βand IL-18 secretion by H.pylori strain SS1 in THP-1 cells could be mediated through activation of NLRP3 inflammasome via ROS signaling pathway, which may be involved in the host innate immune defence and the pathogenesis of the bacteria.

OBJECTIVETo observe the therapeutic efficacy of Modified Taohe Chengqi Granule (MTCG) combined mannitol for treating complicated edema in affected limbs of patients after tibiofibulas double fracture operation (TDFO).

METHODSTotally 64 TDFO patients complicated edema were randomly assigned to the treated group and the control group, 32 in each group. Those in the treated group took MTCG combined intravenous dripping of mannitol, while those in the control group received intravenous dripping of mannitol alone. The treatment course was 1 week. The clinical efficacy, the onset time, the swelling degree, and the pain index were observed and compared between the two groups.

RESULTSOne week after operation, the effective rate was 98.0% and the markedly effective rate was 87.5% in the treated group, while they were 78.0% and 56.9% respectively in the control group. Better results were obtained in the treated group, showing statistical difference when compared with the control group (P < 0.05). As for the onset time for swelling subsiding, it was (2.4 +/- 1.3) days in the treated group and (3.8 +/- 2.9) days in the control group. There was statistical difference between the two groups (P < 0.05). Better effects on the swelling subsiding degree and the pain index were obtained in the treated group, showing statistical difference when compared with the control group (P < 0.05).

CONCLUSIONSMTCG combined mannitol could obviously abate the edema in affected limbs of patients after TDFO. It was a better treatment method for managing edema in the peri-operative period of orthopedics.

Adult ; Drugs, Chinese Herbal ; therapeutic use ; Edema ; drug therapy ; Female ; Fibula ; injuries ; Fractures, Bone ; surgery ; Humans ; Male ; Mannitol ; therapeutic use ; Middle Aged ; Postoperative Complications ; drug therapy ; Tibia ; injuries ; Treatment Outcome ; Young Adult

OBJECTIVETo explore whether PI3K/Akt/mdm2 signalling pathway affect the sensitivity of gastric cancer cell line SGC7901 cells to doxorubicin.

METHODSGastric cancer cell line SGC7901 cells were exposed to doxorubicin and specific PI3K inhibitor wortmannin. Cell apoptosis was detected using flow cytometry. PI3K activity was detected by radioactive immunoprecipitation-kinase assay. Western blotting was employed to evaluate the expressions of PI3K-p85, pAkt-S473, Akt, pmdm2-S166 and p53.

RESULTSThe level of apoptosis in gastric cancer SGC7901 cells treated with doxorubicin was gradually increasing. wortmannin enhanced its effects significantly. PI3K activity and the expression of pAkt-S473 increased in a time-dependent manner, pmdm2-S166, p53 were also increased wortmannin inhibited phosphorylation of mdm2 and improved the p53 expression.

CONCLUSIONPI3K/Akt/mdm2 signalling pathway can be activated by doxorubicin and suppress apoptosis by promoting phosphorylation of mdm2. PI3K inhibitor wortmannin can enhance sensitivity of gastric cancer cells to chemotherapy.

Androstadienes ; pharmacology ; Antibiotics, Antineoplastic ; pharmacology ; Apoptosis ; drug effects ; Cell Line, Tumor ; Doxorubicin ; pharmacology ; Drug Resistance, Neoplasm ; Enzyme Activation ; Humans ; Phosphatidylinositol 3-Kinases ; antagonists & inhibitors ; metabolism ; Phosphorylation ; Protein Kinase Inhibitors ; pharmacology ; Proto-Oncogene Proteins c-akt ; metabolism ; Proto-Oncogene Proteins c-mdm2 ; metabolism ; Signal Transduction ; Stomach Neoplasms ; metabolism ; pathology ; Tumor Suppressor Protein p53 ; metabolism

OBJECTIVETo study the changes of body composition in females patients with human immunodeficiency virus (HIV)-related lipodystrophy (LD) syndrome (HIV-LD).

METHODSTotally 25 female patients who were treated in our hospital from January 2002 to December 2009 were divided into LD group and non-LD group based on the existence of LD. All these patients were receiving highly active antiretroviral therapy (HAART). In addition, 12 healthy women were set as the controls. Total and regional body composition were measured by dual X-ray absorptiometry in all three groups.

RESULTSThe fat mass (FM) was correlated negatively with the duration of HAART (r=-0.431, P=0.029). Multiple linear regression analysis showed that FM had positive correlation with weight and negative correlation with lean mass (LM) (r = - 0. 973, P =0. 000). Total, trunk and leg FM were significantly lower in LD patients than that in controls (P <0.05).Meanwhile, total, trunk and leg bone mineral contents were statistically lower in LD patients than that in controls (P <0. 05). Lumbar bone mineral density of LD patients was lower than that of non-LD patients and controls, and there was significant difference between LD patients and controls (P = 0. 001). LM of LD patients was higher than that of non-LD patients but without statistical difference (P > 0. 05).

CONCLUSIONSThe peripheral and central FM and bone mineral contents remarkably decrease in female patients with HIV-LD. How-ever, HIV-LD patients tend to have higher LM than non-LD patients. .

Adipose Tissue ; metabolism ; Adult ; Body Composition ; physiology ; Bone Density ; physiology ; Female ; HIV-Associated Lipodystrophy Syndrome ; metabolism ; Humans ; Middle Aged ; Young Adult

Adult ; Humans ; Male ; Pulmonary Embolism ; diagnosis ; surgery ; Retroperitoneal Neoplasms ; diagnosis ; surgery

OBJECTIVETo investigate the effects of T cell receptor (TCR) BD2-BJ2 gene rearrangement on the complementary-determining region (CDR) 3 of TCR beta chain (TCR BV CDR3) in Jurkat cells.

METHODSTCR BV gene subfamilies were detected by RT-PCR in Jurkat cells during proliferation and after induction with non-specific T cell activators and SEA, respectively. To determine the clonality of TCR BV subfamilies and the lengths of CDR3, the PCR products were analyzed by TCR GeneScan technique, and the sequences of CDR3 were further analyzed by DNA sequencer.

RESULTSNo new TCR BV subfamilies were found in Jurkat cells, a monoclonal BV8(+)cell line, either during cell proliferation or after stimulation with different treatments, nor were any differences found in CDR3 size or sequences.

CONCLUSIONTCR BD2-BJ2 rearrangement in Jurkat cells may not play a role in modification of TCR BV CDR3 domains or the consequent antigen immunorecognition of BV CDR3, but the possibility of TCR modification can not be excluded.

Amino Acid Sequence ; Base Sequence ; Complementarity Determining Regions ; genetics ; Gene Rearrangement, beta-Chain T-Cell Antigen Receptor ; genetics ; Humans ; Immunologic Factors ; genetics ; Jurkat Cells ; Leukemia, T-Cell ; genetics ; immunology ; pathology ; Molecular Sequence Data ; Receptors, Antigen, T-Cell ; genetics ; T-Lymphocytes ; immunology ; metabolism

OBJECTIVETo study the roles of follicular helper T (Tfh) cells and follicular regulatory T (Tfr) cells in the pathogenesis of Henoch-Schönlein purpura (HSP) in children.

METHODSPeripheral blood samples were collected from 40 HSP children and 25 healthy controls. The percentages of Tfh and Tfr cells were measured by flow cytometry; the mRNA expression levels of Bcl-6, c-MAF, Blimp-1, and PD-1 in peripheral blood were measured by real-time polymerase chain reaction.

RESULTSCompared with the controls, the children with HSP had significantly increased percentage of Tfh cells and Tfh/Tfr ratio but a significantly reduced percentage of Tfr cells in the peripheral blood (P<0.05). Compared with the controls, the children with HSP had significantly increased mRNA expression of Bcl-6 and c-MAF but significantly reduced mRNA expression of Blimp-1 in CD4+ T cells (P<0.05), and had significantly increased mRNA expression of PD-1 but significantly reduced mRNA expression of Blimp-1 in CD4+CD25+ regulatory T cells (P<0.05).

CONCLUSIONSAbnormal percentages of Tfh and Tfr cells may be involved in the pathogenesis of HSP in children, and over-expression of Bcl-6, c-MAF, and PD-1 mRNA and inhibited expression of Blimp-1 mRNA may be considered as important reasons for abnormal percentages of Tfh and Tfr cells.

Adolescent ; Child ; DNA-Binding Proteins ; genetics ; Female ; Humans ; Male ; Programmed Cell Death 1 Receptor ; genetics ; Proto-Oncogene Proteins c-bcl-6 ; Proto-Oncogene Proteins c-maf ; genetics ; Purpura, Schoenlein-Henoch ; etiology ; immunology ; T-Lymphocytes, Helper-Inducer ; immunology ; T-Lymphocytes, Regulatory ; immunology

OBJECTIVETo measure the expression of lymphocyte function-associated antigen-3 (CD58) in childhood B-lineage acute lymphoblastic leukemia (B-ALL) and to explore the feasibility of CD58 as an indicator for minimal residual disease (MRD) detection in childhood B-ALL.

METHODSEighty-seven children diagnosed with B-ALL between January 2014 and September 2014 were enrolled, and 20 hospitalized children who had no tumor or blood disease and had normal bone marrow cell morphology served as the control group. The expression features of CD58 in bone marrow samples from the two groups (at diagnosis, on day 15 of induction chemotherapy) were analyzed by four-color flow cytometry (FCM). Quantitative real-time polymerase chain reaction (qRT-PCR) and FCM were used to detect MRD in B-ALL patients on day 33 of induction chemotherapy.

RESULTSThe mean fluorescence intensity of CD58 expression in the 87 B-ALL cases (91±33) was significantly higher than that in the 20 controls (14±6) (P<0.01); CD58 was over-expressed in 44 of the B-ALL cases. In the B-ALL children, the expression of CD58 on day 15 of induction chemotherapy (105±22) was not significantly different from that at diagnosis (107±26) (P>0.05). In the 44 B-ALL patients with CD58 over-expression, FCM showed 9 MRD(+) cases and 35 MRD(-) cases, while qRT-PCR showed 11 MRD(+) cases and 33 MRD(-) cases; 42 cases (95%) showed consistent results of the two tests, so there was no significant difference between the two methods in detecting MRD (P>0.05).

CONCLUSIONSCD58 is over-expressed and stable in children with B-ALL, and it can be considered as an indicator for MRD detection in childhood B-ALL.

Adolescent ; CD58 Antigens ; analysis ; Cell Lineage ; Child ; Child, Preschool ; Feasibility Studies ; Female ; Humans ; Induction Chemotherapy ; Infant ; Male ; Neoplasm, Residual ; diagnosis ; Precursor B-Cell Lymphoblastic Leukemia-Lymphoma ; drug therapy ; immunology