BackgroundTo assess the predictors for intravenous immunoglobulin (IVIG) resistance and coronary artery lesions (CALs) in Kawasaki disease (KD).MethodsA total of 560 KD patients were reviewed retrospectively, including 410 complete KD (cKD) and 150 incomplete KD (iKD) patients. The laboratory data were compared between the IVIG-resistant and IVIG-responsive groups, as well as between the coronary artery lesions (CALs+) and without coronary artery lesions (CALs-) groups.ResultsIn the cKD patients, C-reactive protein (CRP) levels had a sensitivity of 65.52% and a specificity of 62.7% for predicting IVIG-resistance at a cutoff point of >100 mg/L. When albumin <32 g/L, the sensitivity and specificity for predicting IVIG-resistance were 72 and 83.19%, respectively. N-terminal pro-brain natriuretic peptide (NT-proBNP) levels had a sensitivity of 73.91% and a specificity of 76.43% for predicting IVIG-resistance at a cutoff point of >1300 pg/ml. Interleukin-6 levels had a sensitivity of 76.19% and a specificity of 61.59% at a cutoff value of >45 pg/ml. Erythrocyte sedimentation rate (ESR) levels had a sensitivity of 53.26% and a specificity of 64.14% for predicting CALs at a cutoff point of >75 mm/h.In the iKD patients, the sensitivity and specificity for predicting IVIG-resistance were 80 and 54.1% when hemoglobin <110 g/L. When proportion of neutrophils >70%, the sensitivity and specificity for predicting IVIG-resistance were 68 and 66.94%, respectively. ESR levels had a sensitivity of 70.83% and a specificity of 65.81% for predicting IVIG-resistance at a cutoff point of >80 mm/h. NT-proBNP levels had a sensitivity of 78.57% and a specificity of 56.67% for predicting IVIG-resistance at a cutoff point of >360 pg/ml. Interleukin-6 levels had a sensitivity of 70.59% and a specificity of 66.28% at a cutoff value of >25 pg/ml. Interleukin-10 levels had a sensitivity of 64.71% and a specificity of 74.42% for predicting IVIG-resistance at a cutoff value of >8 pg/ml. ESR levels had a sensitivity of 61.82% and a specificity of 65.12% for predicting CALs at a cutoff point of >75 mm/h.ConclusionsThe white blood cell count, proportion of neutrophils, hemoglobin, CRP, ESR, albumin, NT-proBNP, interleukin-6 and 10 may be effective predictors for IVIG resistance and CALs in KD patients.

Antitumor immune responses are mediated primarily by T cells. Downregulation of the major histocompatibility complex (MHC) and the molecules that costimulate the immune response is associated with defective signaling by tumor cells for T-cell activation. In vitro treatment with a combination of cytokines significantly increased the expression of MHC class I and adhesion molecules on tumor cell surfaces. When tumor cells were first incubated with a bispecific monoclonal antibody that binds antigen on tumor cells to CD28 on T cells, the modified tumor cells become immunogenic and are able to stimulate naive T cells, generating tumor-specific cytotoxic T cells in vitro. Immunization with the modified tumor cells elicits an immune response mediated by CD8+ T cells. This response protected against a challenge with parental tumor cells and cured established tumors. The approach was effective in both low immunogenic and nonimmunogenic tumor model systems. Modification of tumor cells with this two-step procedure may provide a strategy for development of tumor vaccines that is effective for cancer immunotherapy.

A scandium(iii)-catalyzed electrophilic alkene difunctionalization reaction with the synergistic formation of two C–S bonds has been developed. This reaction features a broad substrate scope, simple procedures, 100% atom economy, and a reversed regioselectivity compared to previously studied alkene sulfonylation methods.

Purpose Idiopathic pulmonary fibrosis (IPF) is a chronic, progressive fibrotic lung disease of unknown cause with a variable course. Acute exacerbations of IPF (AE-IPF) is sudden accelerations of the disease or a superimposed idiopathic acute injury significantly reducing lung function. To examine the serum concentrations of Progranulin (PGRN) and activin A in patients with AE-IPF in a pilot study. Methods Twenty-one patients with AE-IPF were compared with 23 patients with stable IPF as a control group. Serum PGRN and activin A levels, arterial blood gas measurements, and lung function were determined in these two groups. Results Peripheral blood PGRN and activin A levels in patients with AE-IPF were 83.7 + 10.0 and 14.2 ± 1.7 ng/ml (mean + SD), respectively; higher than those in the control group 61.0 + 5.8 and 5.8 + 1.0 (p < 0.001). PGRN and activin A levels were significantly negatively correlated with carbon monoxide diffusion capacity r = − 0.857 (p < 0.001) and r = − 0.757 (p < 0.001). Conclusion Progranulin (PGRN) and activin A may be involved in the pathogenesis of AE-IPF. They may be possible markers of disease activity in AE-IPF.

Measurement of the level of a specific protein can be an important parameter to discern as that can change and reflect disease status. A number of methods have been developed to quantitate the level of a protein, some amenable to high throughput screening. A method is described to measure the total level of the tumor suppressor p53 using scintillation proximity assay (SPA) beads and radiolabeled streptavidin. Three different cell extracts were used, with one used to develop the standard curve for the amount of p53. This method allows the specific detection of p53 in the range of 50 to 300 pg in 10 μl of an extract. While this detection is less than what can be detected by commercially available enzyme linked immunosorbant assay (ELISA) kits, the SPA compares favorably on time required and cost. This new assay also has the potential to be coupled with measurements for p53 DNA binding, a unique aspect of this approach.

Three new ester glycosides, named as Caesateroside A (1), Caesateroside B (2) and Caesateroside C (3) were obtained from the seeds of Caesalpinia sappan. The new structures of compounds 1-3 were elucidated by analyzing their 1D NMR, 2D NMR and HR-ESI-MS spectra. Compounds 1-3 showed weak-moderate cytotoxicity against Hela and HepG-2 human cancer cell lines.

Purpose To examine the serum concentrations of Progranulin (PGRN) and activin A in patients with acute exacerbations of idiopathic pulmonary fibrosis (AE-IPF) in a pilot study. Methods Twenty-one patients with AE-IPF were compared with 23 patients with stable idiopathic pulmonary fibrosis (IPF) as a control group. Serum PGRN and activin A levels, arterial blood gas measurements and lung function were determined in these two groups. Students t-test was used to compare the differences in PGRN and activin A levels between the two groups, and Spearman correlation coefficient was used to examine the relationship between serum PGRN and activin A levels with carbon monoxide diffusion capacity in patients with IPF. Results Peripheral blood PGRN and activin A levels in patients with AE-IPF were 83.7+10.0 and 14.2+1.7 ng/ml (mean+SD), respectively, higher than those in the control group 61.0+5.8, and 5.8+1.0 (p<0.001). PGRN and activin A levels were significantly negatively correlated with carbon monoxide diffusion capacity (r=-0.857 p<0.001) and r=-0.757 (p<0.001). Conclusion: PGRN and activin A may be involved in the pathogenesis of AE-IPF. They may be possible markers of activity in AE-IPF.

Background Noonan syndrome (NS) is a relatively rare inherited disease. Typical clinical presentation is important for the diagnosis of NS. But the initial presentation of NS could be significant variant individually which results in the difficult of working diagnosis. Here we report a rare neonatal case of NS who presented with refractory thrombocytopenia as the initial manifestation. Case presentation This was a preterm infant with refractory thrombocytopenia of unknown origin transferred from obstetric hospital at 6 weeks of age. During hospitalization, typical phenotypes of NS in addition to thrombocytopenia were observed, such as typical facial characteristics, short stature, atrial septal defect, cryptochidism, coagulation defect and chylothorax. Genetic testing showed a pathogenic variant at exon 2 of the PTPN11 gene with c.124A > G (p.T42A). Respiratory distress was deteriorated with progressive chylothorax. Chest tube was inserted for continuous draining. Chemical pleurodesis with erythromycin was tried twice, but barely effective. Finally, parents decided to withdraw medical care and the patient died. Conclusions Thrombocytopenia could be the first symptom of Noonan syndrome. After ruling out other common causes of thrombocytopenia, NS should be considered as the working diagnosis.