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The article discusses the crystal structure of the title compound di(potassium 2-(2-ethoxy-2-oxoethoxy)-5-(7-(2-ethoxy- 2-oxoethoxy)-4-oxo-4H-chromen-3-yl) benzenesulfonate) (C23H21KO11S). Topics discussed include the source of material, experimental data, and a study on daidzein and its derivatives. Also provided are tables which show information on data collection, atomic coordinates and displacement parameters.

Background/Aims: Thiamine-responsive megaloblastic anemia syndrome is a rare autosomal recessive disorder resulting from mutations in SLC19A2, and is mainly characterized by megaloblastic anemia, diabetes, and progressive sensorineural hearing loss. Methods: We study a Chinese Zhuang ethnicity family with thiamine-responsive megaloblastic anemia. The proband of the study presented with anemia and diabetes, similar to his late brother, as well as visual impairment. All clinical manifestations were corrected with thiamine (30 mg/d) supplementation for 1–3 months, except for visual impairment, which was irreversible. The presence of mutations in all exons and the flanking sequences of the SLC19A2 gene were analyzed in this family based on the proband’s and his brother’s clinical data. Computer analysis and prediction of the protein conformation of mutant THTR-1. The relative concentration of thiamine pyrophosphate in the proband’s whole blood before and after initiation of thiamine supplement was measured by high performance liquid chromatography (HPLC). Results: Gene sequencing showed a homozygous mutation in exon 6 of the SLC19A2 gene (c.1409insT) in the proband. His parents and sister were diagnosed as heterozygous carriers of the c.1409insT mutation. Computer simulation showed that the mutations caused a change in protein conformation. HPLC results suggested that the relative concentration of thiamine pyrophosphate in the proband’s whole blood after thiamine supplement was significantly different (P=0.016) from that at baseline. Conclusions: This novel homozygous mutation (c.1409insT) caused the onset of thiamine-responsive megaloblastic anemia in the proband. [ABSTRACT FROM AUTHOR]

Objective: To compare indirect immunofluorescence assay (IIFA) and enzyme-linked immunosorbent assay (ELISA) for detecting antinuclear antibodies (ANA) and anti-double-stranded DNA antibodies (anti-dsDNA)., Methods: A total of 125 serum samples were obtained from patients with established or suspected autoimmune disease, and 82 samples were used for ANA detection and 57 for anti-dsDNA detection using both IIFA and ELISA. Fourteen samples were examined for both ANA and anti-dsDNA. In cases where discrepancy occurred in the results by the two methods, extractable nuclear antigens were detected using immunoblotting., Results: The positivity rate of ANA detected by IIFA and ELISA was significantly different (87.8% and 73.17%, respectively, P<0.01), but the positivity rate of anti-dsDNA was similar between IIFA and ELISA (77.19% and 71.93%, respectively, P>0.05). The percent agreement between the two testing methods with different cutoff values of ANA and anti-dsDNA showed significant differences (P<0.01), and for some uncommon patterns, the percent agreement of the two methods was lowered in ANA detection but remained unchanged for anti-dsDNA with different ANA patterns. High percent agreements of the two methods were obtained with the cutoff ANA titer of 1:100 and the cutoff anti-dsDNA value of weak positivity, but they demonstrated a significant difference in testing low-titer ANA and anti-dsDNA., Conclusion: IIFA is more sensitive than ELISA in detecting the total ANA and anti-dsDNA. ELISA prescreening combined with IIFA can obtain the information of the nuclear pattern and allow the observation of the titer alterations. The combination of two or more testing methods can greatly enhance the accuracy of the results.

Objective: To evaluate the feasibility of URIT-12 hemoglobin analyzer for fast measurement of hemoglobin concentration., Methods: Hemoglobin concentration was detected in 100 random blood samples using URIT-12 hemoglobin analyzer and Coulter LH-750 hematology analyzer., Results: The two analyzers showed good correlation of the results (r=0.994) without significant difference between them (P>0.05). The linear range of URIT-12 hemoglobin analyzer was 46-240 g/L, and in the repeated measurements (20 times) of 3 batches of blood samples with low, moderate and high hemoglobin concentrations, the within-batch coefficient of variation of URIT-12 hemoglobin analyzer, from low to high concentrations, were 2.13%, 2.17%, and 2.33%, respectively. In the measurement of 4 batches of high-fat, high-bilirubin, high-globin and high-white-blood-cell blood samples, the interference rate of the former 3 samples were all less than 4% by the two devices, but that of the fourth sample was 10% by URIT-12 hemoglobin analyzer and 7% by Coulter LH-750 analyzer., Conclusion: The results detected by URIT-12 hemoglobin analyzer have high accuracy and precision and is easy to operate, fast-testing and portable.

Objective: To establish a method for evaluating the activity of von Willebrand factor cleaving protease (vWF-cp) and evaluate its clinical application., Methods: Purified von Willebrand factor polymer was isolated by gel filtration from human fresh-frozen plasma as the enzyme substrate. SDS-PAGE, Western blotting, and luminographic detection were used to evaluate vWF-cp activity of 60 healthy adults, 28 patients with cerebral infarction (CI) and 7 with thrombotic thrombocytopenic purpura (TTP)., Results: In the subjects involved, the method for evaluating vWF-cp activity had intra- and inter-batch coefficient of variation(CV) of 4.81% (n=8) and 8.63% (n=6), respectively. According to this method, the plasma vWF-cp activity in the 60 healthy adults was significantly higher than that in the CI patients [(86.53-/+17.49)% vs (77.15-/+16.72)%, P<0.05]. In TTP patients before plasma replacement, the vWF-cp activity was (9.06-/+7.17)% and increased significantly to (47.00-/+6.27)% 24 h after plasma replacement, respectively, but still significantly lower than that of healthy adults (P<0.01), whereas in the convalescent stage, the activity approached the normal level [(83.18-/+8.83)%, P>0.05]., Conclusions: According to the described method, which allows accurate vWF-cp activity measurement with good sensitivity, specificity and reproducibility, vWF-cp activity is lower in CI patients and even more so in TTP patients than that of healthy adults. Plasma replacement can effectively increase the vWF-cp activity in TTP patients.