The clinical characteristics and prognosis of patients with SAPHO syndrome--a real-world cohort study.

OBJECTIVES: We aimed to analyze the clinical characteristics and outcomes of patients with synovitis, acne, pustulosis, hyperostosis, and osteitis (SAPHO) syndrome. METHODS: The clinical records of 64 patients with SAPHO syndrome were collected, and the treatment and outcomes of 27 patients were followed up. The patients were divided into three groups according to the site of bone lesions: only anterior chest wall (ACW) involvement, only spinal involvement, and bone lesion involvement at both sites. The clinical characteristics and outcomes were compared. The clinical characteristics of the patients with and without peripheral joint involvement were compared. RESULTS: Among all patients, 31.25% (20/64) had only ACW involvement, 15.63% (10/64) had only spinal involvement, and 53.12% (34/64) had both ACW and spinal involvement. Peripheral joint involvement was observed in 25.00% (16/64) of the patients. Patients with only spinal involvement were older than those with only ACW involvement (p = 0.006). Patients with both ACW and spinal involvement were older than those with only ACW involvement (p = 0.002) and had a longer diagnosis delay (p = 0.015). Patients with peripheral joint involvement were younger than those without peripheral joint involvement (p = 0.028). During follow-up, 88.89% (24/27) of patients had good outcomes. Twenty-two patients were treated with non-steroidal anti-inflammatory drugs + Iguratimod (IGU), and the outcomes of 90.91% (20/22) improved. CONCLUSIONS: A relationship may exist between the sites of bone lesions and clinical characteristics of patients with SAPHO syndrome. The clinical outcomes of these patients may be good, and IGU may be effective in treating SAPHO syndrome. Key Points • This study is the first long-term follow-up on the effectiveness of iguratimod in treating patients with SAPHO. • This study revealed that patients with SAPHO and different bone lesion sites may present with different clinical characteristics.

An augmented diffusion algorithm with bidirectional communication for a distributed active noise control system.

Recent studies on diffusion adaptation for distributed active noise control (DANC) systems have attracted significant research interest due to their balance between computational burden and stability compared to conventional centralized and decentralized adaptation schemes. The conventional multitask diffusion FxLMS algorithm assumes that the converged solutions of all control filters are consistent to each other, which is unrealistic in practice hence results in inferior performance in noise reduction. An augmented diffusion FxLMS algorithm has been proposed to overcome this problem, which adopts a neighborhood-wide adaptation and node-based combination approach to mitigate the bias in the converged solution of the multitask diffusion algorithms. However, the improvement comes at the expense of a higher computational burden and communication cost. All existing DANC systems, including the multitask and augmented diffusion algorithms, assume one-way communication between nodes. By contrast, this paper proposes a bidirectional communication scheme for the augmented diffusion algorithm to further reduce the memory requirement, computational burden, and communication cost. Simulation results in the free field and with measured room impulse responses both demonstrate that the proposed augmented diffusion algorithm with bidirectional communication can achieve a faster convergence speed than that based on one-way communication with a lower memory, computation, and communication burden.

A study on coherence between virtual signal and physical signals in remote acoustic sensing.

Remote acoustic sensing can be used to estimate the error signals in human ears without placing any physical microphones there. In this paper, the coherence between the signals picked up by physical microphones over a sphere surface and the signal obtained at the sphere center is investigated. Based on the multiple channel coherence formulas in the time domain and frequency domain, the relationship between the coherence and the placement of physical microphones is analyzed by numerical simulations first, then the experimental results obtained in a reverberation chamber and a car cabin are presented to verify the simulation results. Finally, a placement of physical microphones for active control of road noise in car cabins is discussed. Both the numerical and experimental results show that an upper limit frequency exists for accurate sound pressure estimation at the center of a sphere with the sound pressure on the sphere surface. For a sufficiently complex sound field such as that in a reverberation room or in a car, half the wavelength of the upper limit frequency is about the average distance among the physical microphones.

Regulated dielectric loss based on core-sheath carbon-carbon hierarchical nanofibers toward the high-performance microwave absorption.

As a response to stealth technology and electromagnetic pollution, microwave absorbing materials have attracted the attention of many research scholars. However, achieving effective absorption with a low filling level is still a challenge in the harsh environment. Here, an emerging carbon-carbon composite fiber with a core-sheath structure is cleverly tailored for high-performance microwave absorber by tuning the dielectric loss. Reasonable engineering heterogeneous interfaces and conductive paths give rise to a synergistic effect of the impedance matching, conductive loss, polarization loss and multiple scattering. The obtained CR-800 achieves the maximum reflection loss of -51.91 dB, effective absorbing bandwidth of 4.82 GHz, and radar cross section (RCS) reduction value of 41.5 dBm2. Furthermore, the composites own superior environmental adaptation with stable absorbing properties in the harsh environment benefited from great environmental resistance of carbon materials. Given this, the core-sheath carbon-carbon composite fibers are expected to be a candidate for radar stealth technology and electromagnetic pollution.

Integration of efficient microwave absorption and shielding in a multistage composite foam with progressive conductivity modular design.

Ultra-efficient electromagnetic interference (EMI) shielding composites with excellent microwave absorbing properties are the most desirable solution for eliminating microwave pollution. However, integrating absorbing and electromagnetic shielding materials is a difficult challenge because they have different design strategies. In this work, the compatibility of high absorption and shielding capability based on progressive conductivity modular design was realized. Reduced graphene oxide@ferroferric oxide/carbon nanotube/tetraneedle-like ZnO whisker@silver/waterborne polyurethane (rGO@Fe3O4/CNT/T-ZnO@Ag/WPU) multistage composite foams with aligned porous structures were fabricated, which exhibited an excellent average EMI SE > 92.3 dB and remarkable microwave absorption performance with reflection loss < -10 dB in the frequency range of 8.2-18.0 GHz. The average shielding effectiveness of reflection (SER) and reflectivity (R) are as low as 0.065 dB and 0.015, respectively. Besides, the correlations between the morphology and structure of the composite foam and the electromagnetic wave attenuation mechanism were established via electromagnetic simulation. Significantly, the integration of efficient absorbing and shielding materials was realized for the first time. Such composite foams with electromagnetic wave absorption and shielding characteristics are light weight and structurally designable with an adjustable shielding mechanism, and exhibit low filler consumption and high performance. They display promising applications in demanding electromagnetic environments. Our work provides a new strategy to design ultra-efficient EMI shielding materials with reliable absorption-dominated features.

Magnetic coupling N self-doped porous carbon derived from biomass with broad absorption bandwidth and high-efficiency microwave absorption.

Nowadays, developing microwave absorption materials (MAMs) with thin thickness, wide-frequency effective absorption bandwidth (EAB) and strong absorbing capacity is an urgent requirement to tackle the increasingly serious electromagnetic radiation issue. Herein, we report a novel high-performance MAMs by growing Fe3O4 nanoparticles on activated porous carbon derived from egg white via a facile carbonization and subsequent hydrothermal approach. The resultant composite features three-dimensional hierarchical porous carbon embedded with Fe3O4 nanoparticles. Benefiting from the balanced impedance matching and the multi-loss that involve the conductive loss, dielectric loss, dipolar/interfacial polarization loss and magnetic loss, the prepared composite achieves a minimum reflection loss (RL) of -43.7 dB at 9.92 GHz and a broad EAB (RL < -10 dB) of 7.52 GHz (6.24-13.76 GHz) at a thin thickness of 2.5 mm and a low filler content of 20 wt%. This work provides new insights for exploring novel magnetic coupling porous carbon derived from biomass with high-efficiency microwave absorption performance.

Possible effects of chemokine-like factor-like MARVEL transmembrane domain-containing family on antiphospholipid syndrome.

ABSTRACT: Antiphospholipid syndrome (APS) is a systemic autoimmune disease defined by thrombotic or obstetrical events and persistent antiphospholipid antibodies (aPLs). Chemokine-like factor-like MARVEL transmembrane domain-containing family (CMTM) is widely expressed in the immune system and may closely related to APS. This review aimed to systematically summarize the possible effects of CMTM on APS. Publications were collected from PubMed and Web of Science databases up to August 2020. CKLF, CKLFSF, CMTM, antiphospholipid syndrome, immune cells, and immune molecules were used as search criteria. Immune cells, including neutrophil, dendritic cells (DCs), T-cells, B-cells, and inflammatory cytokines, play an important role in the development of APS. Chemokine-like factor 1 (CKLF1) has a chemotactic effect on many cells and can affect the expression of inflammatory cytokines and adhesion molecules through the nuclear factor-kB (NF-kB) pathway or mitogen-activated protein kinase (MARK) pathway. CKLF1 can participate in the maturation of DCs, T lymphocyte activation, and the activation of neutrophils through the MAPK pathway. CMTM1 may act on Annexin A2 by regulating Ca2+ signaling. CMTM2 and CMTM6 are up-regulated in neutrophils of APS patients. Some CMTM family members influence the activation and accumulation of platelets. CMTM3 and CMTM7 are binding partners of B-cell linker protein (BLNK), thereby linking B cell receptor (BCR) and activating BLNK-mediated signal transduction in B cells. Moreover, CMTM3 and CMTM7 can act on DCs and B-1a cell development, respectively. CMTM may have potential effects on the development of APS by acting on immune cells and immune molecules. Thus, CMTM may act as a novel prognostic factor or immunomodulatory treatment option of APS.

Clinical features associated with pregnancy outcomes in women with positive antiphospholipid antibodies and previous adverse pregnancy outcomes: a real-world prospective study.

OBJECTIVE: This study aims to analyze factors related to pregnancy outcomes in women with positive antiphospholipid antibodies and previous adverse pregnancy outcomes (APOs) prospectively. METHODS: Patients' characteristics were described. Factors associated with obstetric complications were analyzed using logistic regression analysis. RESULTS: A total of 128 females with 73.4% non-criteria obstetric antiphospholipid syndrome (NC-OAPS) were included. APOs accounted for 38.3%, of which 65.3% were fetal losses. Live birth rates in criteria OAPS and NC-OAPS were similar (76.5% and 74.5%). For the whole patients, antinuclear antibody (ANA) titer ≥ 1:160 (OR 5.064, 95% CI (1.509, 16.995), P = 0.009) was a risk factor for APOs and low molecular weight heparin (LMWH) use (OR 0.149, 95% CI (0.029, 0.775), P = 0.024)) was a protective factor. Age (OR 1.159, 95% CI (1.011, 1.329), P = 0.034) and previous APOs ≥ 3 times (OR 3.772, 95% CI (1.14, 12.435), P = 0.029) were risk factors for fetal loss, and LMWH use (OR 0.068, 95% CI (0.009, 0.486), P = 0.007) was a protective factor. Regular rheumatology visits was a protective factor for APOs and fetal loss (OR 0.085, 95% CI (0.024, 0.306), P < 0.001; OR 0.019, 95% CI (0.004, 0.104), P < 0.001). The proportion of it decreased in APOs and fetal loss groups (53.1% and 28.1%). Glucocorticoid use was a risk factor for APOs in NC-OAPS and higher serum C3 levels in the first gestational trimester was a protective factor for fetal loss (OR 3.703, 95% CI (1.402, 9.777), P = 0.008; OR 0.041, 95% CI (0.002, 0.947), P = 0.046). CONCLUSION: Age, APO history, ANA titer, LWMH and glucocorticoid use, serum C3 levels, and regular rheumatology visits were related to obstetric complications. Key Points • This was one of the few prospective studies focused on patients with positive antiphospholipid antibodies and previous adverse pregnancy outcomes. The majority were NC-OAPS patients. • The study first evaluated the impact of rheumatologists' monitoring based on individual disease assessments on pregnancy outcomes. The live birth proportion was similar in patients with criteria OAPS and NC-OAPS when treated. • Age, APO history (≥ 3 times), ANA titer (≥ 1:160), LMWH use, glucocorticoid use, and serum C3 were factors related to obstetric complications.

Change of serum B-cell activating factor level in patients with positive antiphospholipid antibodies and previous adverse pregnancy outcomes and its significance.

BACKGROUND: B-cell activating factor (BAFF) is vital for B cell survival. Serum BAFF levels are elevated in thrombotic antiphospholipid syndrome, but little is known about levels in patients with positive antiphospholipid antibodies (aPLs) and previous adverse pregnancy outcomes (APOs). We aimed to analyze serum BAFF concentrations of these patients in early pregnancy along with different pregnancy outcomes. METHODS: Thirty-six pregnant patients positive for aPLs and previous APOs (patient group), 25 healthy pregnant females (HP group) and 35 healthy non-pregnant females (HNP group) from the Peking University Third Hospital, between October 2018 and March 2019, were enrolled in this study. Serum of HNP and serum of patients as well as HP in the first gestational trimester were collected. Enzyme-linked immunosorbent assay kits were used to measure serum BAFF and interferon-alpha (IFN-α) concentrations. Cytometric bead array analysis was used to measure serum concentrations of cytokines. The patient group was further divided into APOs and non-APOs (NAPOs) group, fetal loss and live birth group according to pregnancy outcomes. The Mann-Whitney U-test was used to assess significance between and within groups. Spearman rank-order was used to evaluate correlation coefficients between BAFF and related cytokines. RESULTS: The serum BAFF level in HP group was significantly lower than HNP group (245.24 [218.80, 265.90] vs. 326.94 [267.31, 414.80] pg/mL, Z = -3.966, P < 0.001). The BAFF level was obviously elevated in patient group compared to that in HP group (307.77 [219.86, 415.65] vs. 245.24 [218.80, 265.90] pg/mL, Z = -2.464, P = 0.013). BAFF levels in APOs group tended to be higher than that in NAPOs group (416.52 [307.07, 511.12] vs. 259.37 [203.59, 375.81] pg/mL, Z = -2.718, P = 0.006). Compared to HP group, concentrations of IFN-α, interleukin (IL-6) and tumor necrosis factor were higher in patient group (33.37 [18.85, 48.12] vs. 13.10 [6.85, 25.47] pg/mL, Z = -2.023, P = 0.043; 39.16 [4.41, 195.87] vs. 3.37 [2.92, 3.90] pg/mL, Z = -3.650, P < 0.001; 8.23 [2.27, 64.46] vs. 1.53 [1.25, 2.31] pg/mL, Z = -3.604, P < 0.001, respectively). Serum BAFF levels had a positive correlation with the concentrations of both IL-6 and IL-10 (IL-6: r = 0.525, P = 0.002; IL-10: r = 0.438, P = 0.012). CONCLUSIONS: Serum BAFF levels are increased in patients with positive aPLs and previous APOs as compared to healthy pregnant females and tend to be higher in individuals with current APOs. The BAFF levels have a positive correlation with serum IL-6 and IL-10.

Risk factors for pregnancy failure in patients with antiphospholipid antibody positivity and prior pregnancy losses: A retrospective study.

OBJECTIVES: To analyze the risk factors associated with pregnancy failure in patients with positive antiphospholipid antibodies (aPL) and prior pregnancy losses, with or without a diagnosis of antiphospholipid syndrome. METHODS: We retrospectively reviewed the medical records of all the pregnant patients with positive aPL that visited the Peking University Third Hospital on an inpatient or outpatient basis from 2010 to 2019. According to the pregnancy outcome during this study, patients were divided into successful and unsuccessful groups. The clinical characteristics, laboratory tests and treatments were collected. RESULTS: In total, 105 patients were included. 74/105 (70.48 %) patients were in the successful group and 31/105 (29.52 %) patients were in the unsuccessful group. In univariate analysis, the patients with successful pregnancies had a significant prevalence of low-dose aspirin (LDA) prior to pregnancy (P = 0.049) and heparin plus LDA plus hydroxychloroquine (HCQ) and/or steroids (P = 0.037). Triple aPL positivity (P = 0.026), prior pregnancy losses ≥3 (P = 0.036), maternal age at pregnancy ≥35 years (P = 0.001) and no treatment during pregnancy (P < 0.001) were significantly prevalent in patients with unsuccessful pregnancies. In multivariate analysis, prior pregnancy losses ≥3 (P = 0.039; odds ratio (OR) 4.5; 95 %CI 1.3, 14.3), maternal age at pregnancy ≥35 years (P = 0.019; OR 4.3; 95 %CI 1.1, 18.5) and no treatment (P < 0.001; OR 0.03; 95 %CI 0.004, 0.2) were independent risk factors for subsequent pregnancy losses. CONCLUSION: The risk factors affecting pregnancy outcomes for patients with aPL positivity and prior pregnancy losses included prior pregnancy losses ≥3, maternal age at pregnancy ≥35 years and no treatment during pregnancy.

Chemokine-like factor-like MARVEL transmembrane domain-containing family in autoimmune diseases.

The chemokine-like factor (CKLF)-like MARVEL transmembrane domain-containing family (CMTM) is widely expressed in the immune system. Abnormal expression of CMTM is associated with the development of various diseases. This article summarizes the relevant research on the role of the CMTM family in immune disorders. This information will increase our understanding of pathogenesis and identify promising targets for the diagnosis and treatment of autoimmune diseases. The CMTM family is highly expressed in peripheral blood mononuclear cells. CKLF1 may be involved in the development of arthritis through its interaction with C-C chemokine receptor 4. CKLF1 is associated with the pathogenesis of lupus nephritis and psoriasis. Both CMTM4 and CMTM5 are associated with the pathogenesis of systemic lupus erythematosus. CMTM1, CMTM2, CMTM3, and CMTM6 play a role in rheumatoid arthritis, systemic sclerosis, Sjögren syndrome, and anti-phospholipid syndrome, respectively. The CMTM family has been implicated in various autoimmune diseases. Further research on the mechanism of the action of CMTM family members may lead to the development of new treatment strategies for autoimmune diseases.

Highly Bendable and Durable Waterproof Paper for Ultra-High Electromagnetic Interference Shielding.

An efficient electromagnetic interference (EMI) shielding paper with excellent water repellency and mechanical flexibility has been developed, by assembling silver nanowires (AgNWs) and hydrophobic inorganic ceramic on the cellulose paper, via a facile dip-coating preparation. Scanning electron microscope (SEM) observations confirmed that AgNWs were interconnected and densely coated on both sides of the cellulose fiber, which endows the as-prepared paper with high conductivity (33.69 S/cm in-plane direction) at a low AgNW area density of 0.13 mg/cm2. Owing to multiple reflections and scattering between the two outer highly conductive surfaces, the obtained composite presented a high EMI shielding effectiveness (EMI SE) of up to 46 dB against the X band, and ultrahigh specific EMI SE of 271.2 dB mm-1. Moreover, the prepared hydrophobic AgNW/cellulose (H-AgNW/cellulose) composite paper could also maintain high EMI SE and extraordinary waterproofness (water contact angle > 140°) by suffering dozens of bending tests or one thousand peeling tests. Overall, such a multifunctional paper might have practical applications in packaging conductive components and can be used as EMI shielding elements in advanced application areas, even under harsh conditions.

Gradient Structure Design of Flexible Waterborne Polyurethane Conductive Films for Ultraefficient Electromagnetic Shielding with Low Reflection Characteristic.

Highly efficient electromagnetic shielding materials entailing strong electromagnetic wave absorption and low reflection have become an increasing requirement for next-generation communication technologies and high-power electronic instruments. In this study, a new strategy is employed to provide flexible waterborne polyurethane composite films with an ultra-efficient electromagnetic shielding effectiveness (EMI SE) and low reflection by constructing gradient shielding layers with a magnetic ferro/ferric oxide deposited on reduced graphene oxide (rGO@Fe3O4) and silver-coated tetraneedle-like ZnO whisker (T-ZnO/Ag) functional nanoparticles. Because of the differences in density between rGO@Fe3O4 and T-ZnO/Ag, a gradient structure is automatically formed during the film formation process. The gradient distribution of rGO@Fe3O4 over the whole thickness range forms an efficient electromagnetic wave absorption network that endows the film with a strong absorption ability on the top side, while a thin layer of high-density T-ZnO/Ag at the bottom constructs a highly conductive network that provides an excellent electromagnetic reflection ability for the film. This specific structure results in an "absorb-reflect-reabsorb" process when electromagnetic waves penetrate into the composite film, leading to an excellent EMI shielding performance with an extremely low reflection characteristic at a very low nanofiller content (0.8 vol % Fe3O4@rGO and 5.7 vol % T-ZnO/Ag): the EMI SE reaches 87.2 dB against the X band with a thickness of only 0.5 mm, while the shielding effectiveness of reflection (SER) is only 2.4 dB and the power coefficient of reflectivity ( R) is as low as 0.39. This result means that only 39% of the microwaves are reflected in the propagation process when 99.9999998% are attenuated, which is the lowest value among the reported references. This composite film with remarkable performance is suitable for application in portable and wearable smart electronics, and this method offers an effective strategy for absorption-dominated EMI shielding.