Objective To observe the effect of atmospheric pollution on workers'α1-antitrypsin concentrationb in petrochemical enterprises.Methods During health examination,the workers were indivded into 3 groups according tO the length of job experience.Ⅰ group(<10 years)included 291 cases,aged 22-35 years old,Ⅱ group(10-20 years)included 634 cases,aged 33-45 years old,and Ⅲgroup(>20 years)included 427 cases,aged 44-55 years old.The normal control were individed int different group according to the status of petrochemical workers.,all the serum samples were detected on automatic biochemical analyzer,α1-antitrypsin levels were analyzed between the different groups.Results In group Ⅰ,the α1-AT concentration was 2.58±0.38 g/L(P>0.05)without significant difference compared with control.In group Ⅱ and Ⅲ,the concentration α1-AT were 2.02±0.43 g/L and 1.72±0.41 g/L respectively with significant differences compared with control.And the significant difference existed among the three groups of petrochemical workers..But the sigficant difference did not exist between different group of control people.Conclusion During the process of oil refining,the plants exhausted the polluted gas of nitrogen dioxide,olefins,alkanes,alcohols,carbonyl and other harmful substances,it influenced theα-AT levels of body.It is a good suggestion to decreas the petrochemical pollution,and to monitor the serum concentration of α-AT content among petrochemical workers,it might to reduce the incidence of lung disease of great significance.

Objective To improve the detection of G6PD heterozygotes in female patients by the optimum experimental factors of G6PD/6PGD specific value assay.Methods (1)Identifying the mutations of G6PD gene with the use of amplified refractory mutation system (ARMS).(2)Measuring the G6PD/6PGD specific value.Results According to the data analysed by statistics and ROC curve, the optimum experimental factors included that the incubation temperature was 37℃,the substrate concentrations were 0.78 mmol/L G6PNa2 and 0.195mmol/L NADP+, the reaction time was 10min.Conclusion The optimum experimental factors of G6PD/6PGD specific value assay may be used to improve the detection rate of G6PD heterozygotes in female patients.

Acute mveloid leukemia(AML) is a heterogeneous group of leukemias that arise from clonal transformation of hematopoietic precursors.This review summarizes classification of pediatric AML,more precise risk-group stratification,ongoing phase Ⅲ studies and minimal residual diseasse monitoring.In addition,we discuss the opportunities for innovative chemotherapy drugs in refractory AML and relapsed AML,such as gemtuzumab ozogamicin,liposomal daunorubicin,clofarabine,cladribine and lmatinib.Finally,the roles of allogeneic hematopoietic stem cell transplantation in pediatric AML are also discussed.

Objective To investigate the role of T follicular helper ( Tfh) cells in the immuno-pathogenesis of persistent immune thrombocytopenic purpura ( pITP) .Methods Twenty children with pITP and twenty healthy controls were enrolled in this study .The proportion of CD4+CXCR5+ICOShigh PD-1high T ( cTfh) cells and the expression of ICOSL on CD 19+B cells in peripheral blood of the patients and healthy subjects were analyzed by flow cytometry .The expressions of Bcl-6, c-Maf, IL-21 and ICOSL at mRNA level were detected by real-time PCR.The plasma concentrations of IL-2, IL-6 and IL-21 were determined by ELISA.Results (1) Compared with the healthy controls , the proportions of cTfh cells increased signifi-cantly in patients with pITP [(17.45±9.04) %vs.(7.57±2.57) %, P<0.05], but decreased with the treatment of dexamethasone (DEX) for 7 days [(5.93±1.64) %vs.(17.45±9.04) %, P<0.05].(2) The expression of Bcl-6, c-Maf and IL-21 at mRNA level in patients with pITP were higher than those in healthy controls (P<0.05).(3) Compared with healthy controls, the expression of ICOSL at mRNA and protein levels in CD19+B cells were significantly up-regulated in patients with pITP (P<0.05), which showed no significant changes after treatment with DEX (P>0.05).(4) The plasma concentration of IL-21 was remarkably elevated in patients with pITP , regardless of DEX treatment (P<0.05).But the plasma concentrations of IL-2 and IL-6 showed no significant changes compared with control group (P>0.05).Con-clusion The immunopathogenesis of persistent immune thrombocytopenic purpura might be associated with the hyper-activation of Tfh cells caused by excessive expression of ICOSL and IL-21.The persistent high expression of ICOSL and IL-21 might be one of the important factors resulting in the recurrence of pITP in children .

Objective To observe the expression of leukocyte-associated immunoglobulin(Ig)-like receptor-1 (LAIR-1) on Treg cells in children with immune thrombocytopenia (ITP) and the level of soluble LAIR-1 (sLAIR-1),LAIR-2 in peripheral blood,and to discuss the possible role of LAIR in the pathogenesis of childhood ITP.Methods The levels of LAIR-1 on Treg cells of peripheral blood were measured in 36 children with ITP by using flow cytometry.Plasma levels of sLAIR-1 and LAIR-2 were measured by adopting enzyme-linked immunosorbent assay(ELISA).Real-time PCR was used to measure both LAIR-1 mRNA and LAIR-2 mRNA.Twenty-eight healthy children served as the healthy control group.Results The expression of Treg cells in children with ITP was significantly lower than that in the healthy control group [(2.05 ± 0.85) ％ vs (3.04 ± 1.03) ％,t =4.198,P ＜ 0.001].The expression rate of LAIR-1 on Treg cells and tyrosine phosphatase-2 (SHP-2) in children with ITP group had no statistically significant difference with the healthy control group [(71.18 ± 13.36) ％ vs (67.69 ± 13.07)％,t=1.045,P＞0.05;(1.20 ± 0.97) ％ vs (0.85 ±0.66)％;t=1.718,P＞0.05].The levels of sLAIR-1 and LAIR-2 in plasma in children with ITP group were increased significantly than those in the healthy control group [(20.53 ±4.32) μg/L vs (17.51 ± 5.15) μg/L,t =2.424,P ＜0.05;(5.83 ± 1.08) μg/L vs (5.19 ± 1.24) μg/L,t =2.267,P ＜ 0.05].The LAIR-1 mRNA expression level in children with ITP group was significantly increased compared with the healthy control group (t =2.851,P ＜ 0.05),but not the LAIR-2 mRNA expression level (t =1.715,P ＞ 0.05).Conclusions The expression of Treg cells in children with ITP is decreased,and it may be associated with the onset of ITP,and it may suggest that LAIR-1 does not play a leading role in Treg cells when ITP occurrs.And the levels of sLAIR-1,LAIR-2 in plasma are both increased,suggesting that LAIR-1,LAIR-2 may be one of the factors of immune disorders in children with ITP.

Objective To observe the effect of serum containing Dahuang Zhechong Pills on vascular endothelial growth factor ( VEGF) expression in K562 cells of chronic myelogenous leukemia ( CML) and to develop new antiangiogenic drugs for CML treatment. Methods Enzyme-linked immunosorbent assay ( ELISA) was used for the detection of serum VEGF content in 38 CML patients, 11 acute myelogenous leukemia patients and 9 healthy volunteers. SD rats were used for the preparation of serum containing Dahuang Zhechong Pills, and then ELISA was applied for the detection of VEGF content in K562 cells pretreated with serum containing Dahuang Zhechong Pills. Results Compared with the healthy volunteers, myelogenous leukemia patients at chronic phase and acute phase had high serum VEGF content ( P<0.05 or P<0.01) . Compared with blank serum control group, the secretion of VEGF in K562 cells pretreated with serum containing Dahuang Zhechong Pills was decreased obviously ( P<0.01) , and VEGF expression decreased with the increase of drug concentration. Conclusion VEGF content is increased in myelogenous leukemia patients at chronic phase and acute phase, which may be related to the pathogenesis, progress and prognosis of chronic myelogenous leukemia. Serum containing Dahuang Zhechong Pills can inhibit the expression of VEGF in chronic myelogenous leukemia K562 cells and decrease VEGF protein expression in concentration-dependent manner.

ObjectiveTo investigate the alteration of Fc gamma receptors expressed on monocytes in childhood acute immune thrombocytopenia (aITP).Methods27 patients with aITP and 25 health controls were enrolled in this study.The expression of FcγR Ⅰ and FcγRⅢ on monocytes surface was determined by flow cytometry.Real-time PCR was performed to detect the levels of FcγR Ⅱ a and FcγR.Ⅱ b mRNA.The plasma concentration of IL-4 and IFN-γwas analyzed by ELISA.ResultsThe expression of FcγR I and FcγRⅢ on monocytes was significantly highet in ITP pationts than thatinhealth control group.Transcription levels of FcγR Ⅱ a mRNA and the ratio of FcγR Ⅱ a/Ⅱ b mRNA on monocytes were significantly elevated,while the inhibitory FcγR Ⅱ b mRNA was significant lower in aITP patients (P＜0.05) ; in comparison with healthy controls. After dexamethasone treatment,the stimulatory FcγR Ⅰ,FcγRⅢ and FcγR Ⅱ a mRNA were significantly decreased while inhibitory FcγR Ⅱ b mRNA was increased.(2)The higher levels of plasma IFN-y and lower IL-4 were to be found in aITP patients compared with healthy controls.Correlation analysis showed that the level of plasma IFN-γwas positively correlated with the elevated expression of FcγR Ⅱ a mRNA on monocytes,while the level of IL-4 was positively correlated with FcγRⅡ b mRNA.Conclusion The over expression of inhibitory FcγRs and the down-regulation of inhibitory FcγR Ⅱb mRNA on monocytes,and the imbalance of stimulatory and inhibitory FcγR might be involve in immunological pathogenesis of the acute ITP.The alteration levels of plasma pro-inflammatory cytokine IFN-γand anti-inflammatory cytokine IL-4 might be involved in imbalance expression of FcγR in acute ITP.Dexamethasone therapy could shift the balance between stimulatory and inhibitory FcγR toward inhibitory FcγR Ⅱ b in aITP patients.

Objective To study on chronic myelogenous leukemia on the basis of protein interaction network to further explore its development mechanism.Methods Chronic myelogenous leukemia-related genes were screened from Online Mendelian Inheritance in Man database (OMIM) of genetic.After text mining by Cytoscape software and Agilent Literature Search,the protein interaction networks of chronic myelogenous leukemia were established.Then the molecular complexes contained in the network were analyzed by Clusterviz plug.The biological pathways of molecular complexes were enriched based on DAVID.Results There were 79 chronic myelogenous leukemia genes in OMIM.The protein-protein interaction network of chronic myelogenous leukemia contained 638 nodes,1 830 edges and maybe 5 molecular complexes.Conclusions Pathways underlying complexes 1 are involved in cytokines and inflammation,cytokines-receptor binding,cytokine receptor signaling.Complexes 3 has relation to complex biological behavior of the tumors and other broad relevance,which can provide the bioinformatic foundation for further understanding the development mechanisms of chronic myelogenous leukemia.

Objective To investigate the changes of CD4 +CD25highFoxp3 +regulatory T (Treg) cells and their significance in immune escape of childhood B-cell acute lymphocytic leukemia ( B-ALL ) . Methods Forty-two children with B-ALL and twenty-eight age-matched healthy children were enrolled in this study.Flow cytometry analysis was performed to evaluate the proportion of CD 4 +CD25high Foxp3 +Treg cells as well as CD4 +CD25high ICOS+Foxp3 +and CD4 +CD25high ICOS-Foxp3 +subsets in peripheral blood samples.The expression of associated molecules including IL-10, TGF-β, IL-35, TGF-βRII, ICOS and CD28 at protein level were also measured by flow cytometry analysis .The transcription level of Smad3/4, TIEG1 and Itch by CD4 +T cells were determined by quantitative real-time PCR.The concentration of TGF-βin plasma was detected by enzyme-linked immunosorbent assay.Results (1)The proportion of CD4 +CD25highFoxp3 +Treg cells in children with B-ALL were significantly higher than those of health subjects (P<0.05).The proportion of both ICOS +Foxp3 +and ICOS -Foxp3 +subsets were increased in comparison with those of control group (P<0.05), while the ratio of ICOS +Foxp3 +to ICOS-Foxp3 +was decreased (0.73 ±0.21 vs 1.87 ±0.59, P<0.05).(2) The expression of Foxp3, TGF-β, IL-10 and IL-35 by ICOS+Foxp3 +Treg cells and the expression of membrane bound TGF-βby ICOS -Foxp3 +Treg cells were significantly increased in children with B-ALL (P<0.05).However, the expression of Foxp3 by ICOS -Foxp3 +Treg cells showed no significant difference between the two groups (P>0.05).(3)The concentra-tion of TGF-βin plasma from children with B-ALL were higher than those from control group [ ( 25 .83 ± 12.65) ng/ml vs (8.59 ±5.73) ng/ml, P<0.05].The expression of TGF-βRII and its associated mole-cules (Smad3/4, TIEG1 and Itch) by CD4 +T cells were significantly up-regulated.Moreover, an increased expression of ICOS and CD28 by CD4 +CD25highFoxp3 +Treg cells were also observed in children with B-ALL (P<0.05).Conclusion The hyper-activity of TGF-β, ICOS and CD28 signaling might be closely associ-ated with the increased proportion of CD4 +CD25high Foxp3 +Treg cells and the imbalance of its subsets in children with B-ALL.

Objective: To investigate the clinical features and genetic characteristics of cases with X-linked immunodeficiency with magnesium defect, Epstein-Barr virus (EBV) infection, and neoplasia (XMEN). Methods: Characteristics of clinical material, immunological data and gene mutation of two cases with XMEN in the same family in China were retrospectively analyzed. The related reports literature were searched by using search terms'MAGT1 gene’or'XMEN’. Results: The proband, a 2-year-eight-month old boy, was admitted due to 'Urine with deepened color for two days and yellow stained skin for one day’. He had suffered from recurrent upper respiratory tract infection and sinusitis previously. Hemoglobin level was 38 g/L. The absolute count of reticulocytes was 223.2×10⁹/L. Urobilinogen level was 38 μmol/L (3-16 μmol/L). Coomb's test was positive. Both total (77.2 μmol/L) and indirect bilirubin (66 μmol/L) levels were elevated. There was an inverted CD4⁺/CD8⁺T cell ratio (0.89). The gene sequencing results showed MAGT1 gene c.472delG, p.D158Mfs*6 mutation. His 1-year-6-month old brother, was also identified to have MAGT1 gene c.472delG, p.D158Mfs*6 mutation.The younger brother mainly suffered from recurrent upper respiratory tract infection, accompanied by an inverted CD4⁺/CD8⁺T cell ratio (0.45), an elevated ratio and number of total B cells (45.7%). A total of 7 reports were retrieved including 11 male cases caused by MAGT1 gene mutation. These 11 cases were characterized by EBV viremia (11 cases), recurrent upper respiratory tract infection, otitis media or sinusitis (10 cases), secondary neoplasia diseases (8 cases), reduction of CD4⁺/CD8⁺ T cell ratio (7 cases),and autoimmune thrombocytopenia or hemolytic anemia (2 cases). Conclusion XMEN often manifests as male onset, recurrent upper respiratory tract infection, otitis media or sinusitis, EBV viremia, lymphoproliferative disease or lymphoma, autoimmune diseases and reduction of CD4⁺/CD8 ⁺T cell ratio. NKG2D expression in NK cells is significantly reduced, and gene sequencing analysis shows a pathogenic mutation in MAGT1 gene.