Williams-Beuren syndrome in pediatric T-cell acute lymphoblastic leukemia: A rare case report and review of literature.

Background: Williams-Beuren syndrome (WBS) is a rare genetic disorder caused by hemizygous microdeletion of contiguous genes on chromosome 7q11.23. Although the phenotype features extensive heterogeneity in severity and performance, WBS is not considered to be a predisposing factor for cancer development.

Measurement of the morphological data of primary teeth in northwest China.

Objective: This study aims to digitally obtain the morphological data of children's primary teeth in northwest China and evaluate the reliability of digitally obtaining the anatomical morphological data of primary teeth.

Methods: A total of 308 extracted primary teeth and cone-beam computed tomography (CBCT) images of 407 primary teeth were collected in northwest China. Electronic digital Vernier callipers (accuracy: 0.

[New methods to detect autophagic flux].

Autophagy is a crucial biological process of eukaryotes, which is involved in cell growth, survival and energy metabolism, while the premise of the autophagy function is activated autophagic flux. It has been confirmed that impaired autophagic flux promotes pathogenesis of many chronic inflammatory diseases, especially cancer, neurodegenerative disease and tissue fibrosis, therefore the analysis of autophagic flux state is important for revealing autophagy function and the mechanism of autophagy related diseases. Given that autophagy is a dynamic process with multiple steps, it is very hard to observe the real state of autophagic flux.

[Research advances in the pathogenesis, diagnosis and treatment of hemophagocytic lymphohistiocytosis in children].

Hemophagocytic lymphohistiocytosis (HLH), or hemophagocytic syndrome (HPS), is characterized clinically by abrupt onset and progressive deterioration and even death. HLH is much more prevalent in children, and is potentially fatal if early diagnosis is not made and appropriate HLH-directed therapy not instituted. Increasing genetic defects and underlying diseases or causative factors have been identified to be closely implicated in the pathogenesis of HLH.

[Establishment and application of TLR2 receptor-based cell screening model].

TLR2 activity plays an important role in the pathogenesis of autoimmune diseases, tumor carcinogenesis and cardio-cerebrovascular diseases. To establish a TLR2 receptor-based cell screening model, NF-kappaB promoter-driven luciferase reporter plasmids were transfected into human embryonic kidney cells (HEK293) stably expressing human TLR2 and co-receptors CD14, TLR1 and TLR6. Single clones were then isolated and characterized.

[DEDD decreases Smad3 activity, promotes tumor cell apoptosis and inhibits proliferation].

DEDD is a member of the death-effector domain protein family. DEDD inhibits the Smad3 mediated transcriptional activity and participates in the regulation of apoptosis. In this study, how the death-effector domain of DEDD participates in the regulation of Smad3 activity and apoptosis has been further investigated.

[Experimental study of donor natural killer cells reducing graft-versus-host disease and enhancing engraftment].

The purpose of this study was to investigate whether pretransplant infusion of reactive natural killer cells (NK cells) from donor or recipient can reduce graft-versus-host disease (GVHD) and enhance engraftment in bone marrow transplantation (BMT). Recipient BALB/c mice were divided into 4 groups after received 6.5 Gy total-body irradiation (TBI): control group 1 was treated with nothing, control group 2 received BMT alone, experiment group 1 received BMT and autoreactive NK cells, experiment group 2 received BMT and alloreactive NK cells.

[Immune regulatory effect of human bone marrow mesenchymal stem cells on T lymphocyte].

To investigate the immune regulatory effects of human bone marrow mesenchymal stem cells on alloantigen T lymphocyte in vitro, human MSCs were isolated and expanded from bone marrow cells, and identified with cell morphology, and the phenotypes were assessed by immunohistochemistry and flow cytometry. As the stimulation factor of T lymphocytes proliferation, either PHA or dendritic cells isolated from cord blood were cocultured with CD2(+) T lymphocytes from peripheral blood mononuclear cells by magnetic beads with or without MSC in 96-well plats for seven days. T cell proliferation was assessed by [(3)H]-thymidine incorporation using a liquid scintillation counter.

[Construction of eukaryotic expression vector of siRNA specific to bcr/abl fusion gene].

Objective: To construct eukaryotic expression vector of siRNA specific to bcr/abl and to initially investigate the effect of recombinant plasmid on bcr/abl and P210 protein expression in K562 cells.

Methods: siRNA (small interfering RNA) was designed according to the Tuschl's principle of RNAi-based medicine, and was converted into cDNA coding expression of shRNA (small hairpin RNAs)of siRNA for bcr/abl fusion gene. The cDNA was synthesized and inserted into plasmid pTER.

[A clinicopathological study of 96 cases of lymphoblastic lymphoma].

Objective: To investigate the clinicopathological and immunohistochemical features of lymphoblastic lymphoma (LBL).

Methods: A retrospective clinicopathological study of 96 cases LBL was carried out. Immunohistochemical staining was used for the characterization and immunophenotyping.