Clinical analysis of 10 cases of multi-center tumor necrosis factor receptor-associated periodic syndrome / 中华儿科杂志

Objective: To summarize the clinical characteristics of tumour necrosis factor receptor-associated periodic syndrome (TRAPS) in children. Methods: The clinical manifestations, laboratory tests, genetic testing and follow-up of 10 children with TRAPS from May 2011 to May 2021 in 6 hospitals in China were retrospectively analyzed. Results: Among the 10 patients with TRAPS, including 8 boys and 2 girls. The age of onset was 2 (1, 5) years, the age of diagnosis was (8±4) years, and the time from onset to diagnosis was 3 (1, 7) years. A total of 7 types of TNFRSF1A gene variants were detected, including 5 paternal variations, 1 maternal variation and 4 de novo variations. Six children had a family history of related diseases. Clinical manifestations included recurrent fever in 10 cases, rash in 4 cases, abdominal pain in 6 cases, joint involvement in 6 cases, periorbital edema in 1 case, and myalgia in 4 cases. Two patients had hematological system involvement. The erythrocyte sedimentation rate and C-reactive protein were significantly increased in 10 cases. All patients were negative for autoantibodies. In the course of treatment, 5 cases were treated with glucocorticoids, 7 cases with immunosuppressants, and 7 cases with biological agents. Conclusions: TRAPS is clinically characterized by recurrent fever accompanied by joint, gastrointestinal, skin, and muscle involvement. Inflammatory markers are elevated, and autoantibodies are mostly negative. Treatment mainly involves glucocorticoids, immunosuppressants, and biological agents.

Expression of GATA-3 in Pulmonary Tissue of Asthmatic Mice and Inhibitory Effect of Dexamethsone on It / 实用儿科临床杂志

Objective To explore the expression of GATA-3 in pulmonary tissue of asthmatic mice and the inhibitory effect of dexamethsone(Dex)on it.Methods The Blab/c mice asthma model was induced by ovalbumin(OVA) with classic method.Twenty-four male mice were randomly divided into control group,asthmatic group and Dex treated group.The expression of GATA-3 protein was measured by immunohistochemical staining.The expression of GATA-3 mRNA was measured by reverse transcription-polymerase chain reaction(RT-PCR).The level of IL-4 in mice spleen CD4 T cell was measured by flow cytometry.The airway inflammation was evaluated by HE staining.Results The percentages of postive GATA-3,GATA-3 mRNA and IL-4 protein of asthmatic group were significantly higher than those of control group(P

Expression of CD 86 on monocyte and B cell surface, the level of T(H)-derived cytokine and their correlation in children with asthma / 中华儿科杂志

<p><b>OBJECTIVE</b>To explore the role of T lymphocytes activation co-stimulation pathway and T lymphocyte subset activation in asthma pathogenesis.</p><p><b>METHODS</b>The blood samples were taken from 35 asthma children (including 22 male and 13 female, age 11 months-9 years) and 31 normal children (including 19 male and 12 female, age 8 months-12 years). Direct immunofluorescence flow cytometry was used to detect the CD86 mean fluorescence intensity (MFI) on CD(14)(+) cell, CD(19)(+) cell percentage and CD19 and CD86 double positive cell percentage in PBMC activated by LPS. ELISA was used to detect the levels of IL-4, IL-13, IFN-gamma in culture supernatants of PBMC stimulated with PHA and plasma total IgE level.</p><p><b>RESULTS</b>(1) The CD86 MFI on CD(14)(+) cell in asthma group was elevated significantly (31.8 +/- 9.2 vs 23.5 +/- 6.4, P < 0.01). (2) CD(19)(+) cell percentage and CD19 and CD86 double positive cell percentage in PBMC stimulated with LPS in asthma group were increased significantly (13.5 +/- 4.0 and 4.6 +/- 2.0 vs. 8.2 +/- 3.0 and 2.3 +/- 1.4, respectively, P < 0.01). The plasma total IgE level [186.6 (64.3 - 723.6)] was higher than that of control's [41.95 (0.9 - 115.2)]. (3) The levels of IL-4 and IL-13 in supernatants stimulated with PHA increased significantly [34.2 (2.8 - 70.0) and 1 239.0 (378.3 - 2 929.0) vs. 9.7 (2.0 - 46.2) and 683.2 (128.8 - 1 560.8) respectively, P < 0.01, P < 0.05]; and the level of IFN-gamma decreased significantly [12.16 (1.6 - 44.8) vs. 26.7 (2.1 - 99.5) P < 0.05]. (4) In asthma group there was a significantly positive correlation of CD86 MFI on CD(14)(+) cell with CD19 and CD86 double positive cell percentage (r = 0.644), there was a significantly positive correlation of CD(19)(+)CD(86)(+)cells percentage with plasma total IgE (r = 0.537); there was a significantly positive correlation of CD(19)(+)cells percentage with the levels of IL-4 and IL-13 (r = 0.607, 0.540 respectively); a significantly positive correlation of CD(19)(+)CD(86)(+) percentage cells with the levels of IL-4 and IL-13 was found (r = 0.617, 0.678, respectively).</p><p><b>CONCLUSIONS</b>(1) The expression of CD86 on APC cells increased in children with acute asthma. (2) The response to mitogens of B lymphocyte and T(H2) but not T(H1) lymphocyte were increased significantly. The results suggest that CD86 and imbalance of T(H) subset may play an important role in asthma pathogenesis.</p>

Inhibition of respiratory syncytial virus replication in cultured cells by RNA-cleaving DNAzyme / 中华儿科杂志

<p><b>OBJECTIVE</b>DNAzyme/Deoxyribozyme is another novel molecular biological tool following the ribozyme. DNAzyme consists of a 15-nucleotide (nt) internal loop as its catalytic domain and two flanking substrate-recognition domains of 7 to 8 nt each which is complementary to substrate. The RNA substrate is cleaved at a particular phosphodiester located between an unpaired purine and a paired pyrimidine residue. DNAzyme has been applied in fields such as viral infectious disease, tumor, cardiovascular disease and genetic disease. But there is no report about using DNAzyme for anti-respiratory syncytial virus purpose. To observe the inhibitory effects of RNA-cleaving DNAzymes on respiratory syncytial virus (RSV) replication in cultured cells.</p><p><b>METHODS</b>Anti-RSV RNA-cleaving DNAzyme DZ604 was designed to target the RSV genome at the start of the NS2 gene in an effort to inhibit the RNA replication. Microscope and electron microscope were used to observe the effects of anti-RSV genomic RNA DNAzyme on cytopathogenic effect (CPE) and ultrastructural change of 9HTE cell induced by RSV infection. Viral plaque forming reduction assay and MTT assay were used to detect the anti-RSV activity and protective function for RSV infected 9HTE cells of DNAzyme.</p><p><b>RESULTS</b>Anti-RSV genomic RNA DNAzyme (DZ604) significantly improved CPE of RSV-infected 9HTE cells. The time to appearance of CPE and of total CPE was delayed by using DZ604 in a dose-dependent manner. At a 5 micro mol/L concentration of DZ604, CPE of 9HTE cells induced by RSV infection at 10 and 1 multiplicity of infection (MOI) was not improved. At smaller MOI (0.1, 0.01, 0.001, 0.0001) of RSV infection, CPE of 9HTE cells was significantly lightened by DZ604 at the same concentration. DZ604 also significantly improved ultrastructural change of 9HTE cells at early stage of RSV infection. Reduction in RSV yield was 85.56% and 8.33% at concentrations of 5 micro mol/L and 0.25 micro mol/L of DZ604. DZ604 inhibited RSV yield in a dose-dependent manner (P < 0.05). Non-specific DNAzyme did not have anti-RSV activity (P > 0.05).</p><p><b>CONCLUSION</b>Anti-RSV genomic RNA DNAzyme designed and synthesized in our laboratory was capable of inhibiting respiratory syncytial virus replication specifically in cultured cells. Our data indicated that DNAzymes could be useful for the prevention against respiratory syncytial virus infection.</p>

Expressions of GATA-3 and Th2 Cytokines in Asthmatic Mice and Their Significances / 实用儿科临床杂志

Objective To investigate the expressions and significances of GATA-3 and Th2 cytokines in asthmatic mice.Methods Sixteen BALB/c mice were randomly divided into control group and asthmatic group.The pathological changes in trachial and pulmonary tissue were observed with HE staining method.The GATA-3 mRNA expression in pulmonary tissue was messured with reverse transcription polymerase chain reaction(RT-PCR).And the IL-5,IL-13 proteins in pulmonary tissue were detected with enzyme linked immunosorbent assay(ELISA).Results HE staining showed infiltration of a great deal of inflammatory cells around the bronchial wall in asthmatic group,while there was no obvious infiltration in control group;Compared with control group,the expressions of GATA-3 mRNA and IL-5,IL-13 protein in pulmonary tissue increased significantly(Pa

The relationship of expression of CD_(30) on CD4~+ cell surface and level of Thelper cell 2 derived cytokine and plasma total immunoglobulin E in asthmatic children / 实用儿科临床杂志

Objective To explore the role of CD 30 on CD4 + T lymphocyte and T lymphocyte subset activation in asthma pathogenesis.Methods Twenty seven asthma active attack patients,16 acute upper respiratory infection patients and 19 control children were randomly selected. Direct immunofluorescence flow cytometry was used to detect the CD 30 positive percentage on CD4 +cell; ELISA was used to detect the levels of interleukin(IL) 4 and IL 13 in culture supernatants of peripheral blood mononuclear cells(PBMC) and plasma total immunoglobulin E(IgE) level.Results Compared with control group and acute upper respiratory infection group, in asthmatic children the CD4 +CD 30 + percentage on CD4 + cell was elevated significantly; 2.The levels of IL 4 and IL 13 in supernatants of cultured lymphocyte were increased significantly, the plasma total IgE level was increased significantly;3.There was a significant positive correlation of CD4 +CD 30 + cell percentage with the levels of IL 4 and IL 13 in culture supernatants and plasma total IgE.Conclusions The cell that could produce Th2 derived cytokine may consist of CD 30 +cell;When CD 30 L combined with CD 30 on CD4 + cells ,it might result in Th2 cell proliferation ,develop and release Th2 derived cytokine; IL 4 and IL 13 could induce B cell production and secrete more IgE. It is suggested that CD 30 and imbalance of Th2 subset may play an important role in asthma pathogenesis.