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Allergic reactions to FIX have been associated with nephrotic syndrome and poor ITI outcome.3 Both our cases did, with case 2 having allergic reactions simultaneously with inhibitor development. This is consistent with previous observations that NS development was closely related to a significant decline in inhibitor titre.2 Interestingly, the NS in case 2 was preceded by a second allergic reactions to the ITI regimen and in case 1 by a fluctuation of inhibitor titres during ITI-rituximab. Patients with haemophilia B (HB) may develop factor IX (FIX) inhibitors with a cumulative incidence of 10.2% following FIX replacement therapy by 500 exposure days.1 In 50-60% cases, occurrence of severe allergic reactions may precede or occur simultaneously with the inhibitor development.2 The propensity for FIX to induce allergic reactions (compared to FVIII) may be related to the extravascular distribution of the smaller FIX molecules.3 Immune tolerance induction (ITI) therapy to eradicate FIX inhibitors has a poor success rate of only 13-31%.2,4 There is also a risk of developing nephrotic syndrome (NS) in 17-33% cases. Nephrotic syndrome in two haemophilia B children with inhibitor under low-dose immune tolerance induction combined with rituximab-based immunosuppressant protocol. [Extracted from the article]

A low-dose immune tolerance induction (ITI) protocol incorporating immunosuppressive agents in haemophilia A children with high-titre factor VIII inhibitor and poor-ITI prognostic risk These were all on good-ITI risk patients with inhibitor titre <10 BU at the initiation of ITI and historical peak inhibitor titre <200 BU. Nineteen haemophilia A inhibitor children with poor-ITI risk (historical peak inhibitor titre >=100 BU and/or inhibitor titre >=40 BU at ITI initiation) (Table 1) were enrolled from September 2016 through April 2019. Various ITI regimens achieved an overall success rates of 60%-80%.2,3 According to the UK guideline,4 low-dose ITI (50 IU/kg, every-other-day) was only suggested for using in patient with low-titre inhibitors (historical peak inhibitors <5 BU). [Extracted from the article]

The defect characteristic and magnetic properties of CuFe1-xCrxO2 (x = 0.0–0.4) compounds have been investigated in detail. The results demonstrate that Cr3+ ions enter into the CuFeO2 lattice sites and induce local lattice contraction but do not change the valance state of Cu+ and Fe3+ ions. We calculate the positron annihilation lifetime in the bulk and vacancy defect trapping states by the atomic superposition method in 3 × 3 × 1 CuFeO2 super-cell. Combined with theoretical calculation results, the positron annihilation results indicate that the positrons are mainly annihilated in vacancy clusters in CuFe1-xCrxO2. The substitution of Cr3+ for Fe3+ ions decreases the open volume and increases the concentration of vacancy defects but does not change the integral defect surroundings at annihilation sites. Magnetization measurements manifest that doping with Cr3+ reduces the antiferromagnetic transition temperature and promotes the formation of short-range magnetic correlation. Meanwhile, the enhanced short-range magnetic correlation in Cr3+-doped CuFeO2 is more readily driven by thermal activation energy and a faster dynamic behavior can be seen in time windows. [ABSTRACT FROM AUTHOR]

In this paper, M (Sc3+ and Zr4+)-doped CuFeO2 ceramics were prepared by using a solid-state reaction method. The effects of isovalent Sc3+ and nonisovalent Zr4+ substitution on the structure, defects, optical, and magnetic properties of CuFeO2 ceramics are comparatively investigated. The results demonstrated that all of the obtained samples are well crystallized, and M substitution leads to lattice structure distortion. The average grain sizes reduce with the addition of M. Positron annihilation lifetime results indicate that the vacancy clusters are present in all samples, and the Zr-substituted CFO sample has the maximum open volume and concentration of the defect among those three samples. Furthermore, the bandgap of the M-substituted samples becomes wider due to the broad bandgap of Sc2O3 and ZrO2. M substitution enhances the Urbach energy (EU) values in CFO system, which is in good consistent with the dependences of the defect concentration on the substitution elements. The magnetic measurements reveal that Sc and Zr substitution decrease the magnetic temperatures TN1 and TN2, which may be attributed to the consequence of the magnetic dilution effect and the change in the strength of exchange interaction. In addition, the defects are of major importance for regulating the magnetic properties of CFO ceramics. [ABSTRACT FROM AUTHOR]

Comparative pharmacokinetics of Kogenate FS and Kovaltry in 14 Chinese paediatric patients with haemophilia A: A single-centre study Although the CL of Kogenate FS and Kovaltry showed no statistical difference in our study, the CL of Kovaltry showed a decreasing trend when compared with Kogenate (3.35 vs 3.78 mL/kg/h). Keywords: factor VIII; haemophilia A; haemorrhage; paediatric patients; pharmacokinetics EN factor VIII haemophilia A haemorrhage paediatric patients pharmacokinetics e287 e290 4 03/23/21 20210301 NES 210301 Dear Editor, Haemophilia A is an X-linked inherited bleeding disease caused by a deficiency in the activity of coagulation factor VIII (FVIII). According to the World Federation of Hemophilia (WFH), the management of haemophilia A involves prophylaxis as standard of care, especially for patients with severe haemophilia A.1 However, significant interindividual variability is observed within the patients of haemophilia A in response to treatment. [Extracted from the article]

The polycrystalline CuFeO2 (CFO) and CuFe1-xMxO2 (M = Ti, Hf, Zr, M-CFO) samples are synthesized by solid-state reaction method. The doping effect of tetravalent nonmagnetic transition metal M4+ ions on the microstructure, defect evolution, and magnetic and magnetocaloric properties of the CFO system are comparatively investigated. The substitution of M4+ for Fe3+ increases lattice parameters, changes the bond length of Fe/Cu-O and bond angle of Fe-O-Fe, and improves the microstructure to some extent. Positron annihilation spectroscopy results indicate that M4+ doping induces the agglomeration of small-sized vacancy defects manifested as the increase in vacancy-type defect size and the suppression of inherent defect concentration. Magnetic measurements show that the antiferromagnetic stability of CFO system is clearly affected by M4+ doping, and Ti4+ doping has a more noticeable impact than Hf4+ and Zr4+ doping. Meanwhile, the coexistence of weak ferromagnetism and antiferromagnetism is detected in all doped samples, while Ti-doped system exhibits extraordinary magnetic properties. Especially for Ti-CFO2 (x = 0.03) sample, the maximum magnetization reaches as high as 11.81 emu/g at 0.5 T, which enhanced one order of magnitude than that of undoped CFO. Isothermal M-H data at different temperatures show that entropy change (ΔSM) and refrigerant capacity (RC) for CFO and M-CFO2 samples are significantly weakening with M4+ substitution in bulk CFO. The maximum ΔSM = 4.79 J·kg−1 K−1 and RC = 12.79 J·kg−1 for undoped CFO are obtained near the transition temperature TN2 = 12 K, with the applied fields up to 6 T. The possible reasons for the above observations are discussed in detail. [ABSTRACT FROM AUTHOR]

GdMnO3 (GMO) ceramics were prepared by solid-state reaction method, the structure, defects, dielectric, and magnetic properties of GMO ceramics sintered at different temperatures have been studied. Characterization by XRD indicates that impurity phase-free GMO can be synthesized at 1350 °C. SEM measurements show that the grain size of GMO increases and the microstructure becomes denser with increasing sintering temperature. Positron annihilation results reveal that the vacancies concentration decreases when sintering temperature increases from 1300 to 1350 °C, and then increases when sintering temperature increases from 1350 to 1400 °C. Dielectric measurements indicate that the GMO ceramic sintered at 1350 °C presents higher dielectric constant and smaller dielectric loss. Magnetic properties measurements show that the magnetic properties of GMO can be modulated by sintering temperature. The relation between structure and properties implies that the cation vacancy defect has remarkable effects on the dielectric and magnetic properties of GMO. [ABSTRACT FROM AUTHOR]

Objective: This study aims to investigate the association of circulating T follicular helper (cTfh) cells and T follicular regulatory (cTfr) cells with acute myeloid leukemia (AML) patients. Methods: A total of 22 newly diagnosed, untreated AML patients as well as 26 healthy controls were enrolled. Percentages of cTfh and cTfr cells were detected using flow cytometry. Results: Compared to healthy controls, a significantly higher percentage of cTfr cells was observed in AML patients (4.10 ± 11.18 vs. 0.63 ± 0.38%) (p < 0.05). In addition, a significantly lower cTfh/cTfr ratio was found in the AML patients' group when compared to the control group (9.04 ± 9.19 vs. 11.66 ± 5.68) (p < 0.05). A lower level of plasma IL-2 and TGF-β1 was found in AML patients. Based on the complete remission (CR) response after one cycle of inductive chemotherapy, patients were divided into two groups at sample collection: AML with and without CR. Significantly lower percentages of cTfr cells and a higher cTfh/cTfr ratio were found in the group of AML patients with CR than in the AML patients without CR. Conclusion: There was a significantly higher percentage of cTfr cells in AML patients. cTfr cells may have a potential association with the pathogenesis of AML patients. [ABSTRACT FROM AUTHOR]