[Clinical characteristics and labial gland pathological features in children with systemic lupus erythematosus complicated by Sjögren's syndrome]. / å„¿ç«¥ç³»ç»Ÿæ€§çº¢æ–‘ç‹¼ç–®åˆå¹¶å¹²ç‡¥ç»¼åˆå¾çš„ä¸´åºŠç‰¹ç‚¹åŠå”‡è ºç— ç†ç‰¹å¾åˆ†æž.

OBJECTIVES: To study the clinical manifestations, laboratory features, and labial gland pathological features in children with systemic lupus erythematosus (SLE) complicated by Sjögren's syndrome (SS). METHODS: A retrospective analysis was conducted on 102 children with SLE who underwent labial gland biopsies at Renji Hospital, Shanghai Jiao Tong University School of Medicine from January 2013 to December 2022. The children were divided into two groups based on the presence of SS: the SLE with SS group (SLE-SS; 60 children) and the SLE-only group (42 children). According to the focus score (FS) of the labial glands, children in the SLE-SS group were further subdivided into FS≥4 subgroup (26 children) and FS<4 subgroup (34 children). The clinical data of the groups were compared. RESULTS: Compared to the SLE-only group, children in the SLE-SS group had less skin and mucosal involvement, were more likely to have positive anti-SSA and anti-SSB antibodies, and had higher levels of rheumatoid factor (P<0.05). There was no significant difference in treatment protocols between the two groups (P>0.05). Compared to the FS<4 subgroup, the FS≥4 subgroup had more frequent musculoskeletal involvement (P<0.05), but there was no significant difference in SLE disease activity or other major organ involvement between the subgroups (P>0.05). CONCLUSIONS: Children with SLE complicated by SS are less likely to have skin and mucous membrane involvement and exhibit specific serological characteristics. The SLE-SS children with an FS≥4 are more likely to experience musculoskeletal involvement. However, FS is not associated with disease activity or other significant organ damage.

Quantum Size Effect to Induce Colossal High-Temperature Energy Storage Density and Efficiency in Polymer/Inorganic Cluster Composites.

Polymer dielectrics need to operate at high temperatures to meet the demand of electrostatic energy storage in modern electronic and electrical systems. The polymer nanocomposite approach, an extensively proved strategy for performance improvement, encounters a bottleneck of reduced energy density and poor discharge efficiency beyond 150 °C. In this work, a polymer/metal oxide cluster composite prepared based on the "site isolation" strategy is reported. Capitalizing on the quantum size effect, the bandgap and surface defect states of the ultrasmall inorganic clusters (2.2 nm diameter) are modulated to markedly differ from regular-sized nanoparticles. Experimental results in conjunction with computational simulation demonstrate that the presence of ultrasmall inorganic clusters can introduce more abundant, deeper traps in the composite dielectric with respect to conventional polymer/nanoparticle blends. Unprecedented high-temperature capacitive performance, including colossal energy density (6.8 J cm-3 ), ultrahigh discharge efficiency (95%) and superior stability at different electric field frequencies, are achieved in these polymer/cluster composites up to 200 °C. Along with the advantages in material preparation (inexpensive precursors and one-pot synthesis), such polymer/inorganic cluster composite approach is promising for high-temperature dielectric energy storage in practical power apparatus and electronic devices.

Evaluation of the 2019 EULAR/ACR classification criteria for systemic lupus erythematosus in children and adults.

OBJECTIVE: To evaluate the performance of the 2019 European League Against Rheumatism/American College of Rheumatology (EULAR/ACR-2019) classification criteria for systemic lupus erythematosus (SLE) compared with the ACR-1997 and Systemic Lupus International Collaborating Clinics (SLICC) 2012 criteria in childhood-onset SLE (cSLE) and adult-onset SLE (aSLE) patients. METHODS: We conducted a retrospective study of SLE patients (221 children and 221 adults) and controls (214 children and 214 adults) with defined rheumatic diseases to establish each ACR-1997, SLICC-2012, and EULAR/ACR-2019 criterion fulfilled. Demographic, clinical, and laboratory features were evaluated through chart review. RESULTS: For cSLE, sensitivities of ACR-1997, SLICC-2012, and EULAR/ACR-2019 criteria were 63.3%, 94.6%, and 98.2%, with specificities 99.5%, 98.6%, and 93.5%, respectively. For aSLE, sensitivities of ACR-1997, SLICC-2012, and EULAR/ACR-2019 criteria were 72.9%, 96.8%, and 99.1%, with specificities 97.2%, 92.5%, and 90.2%, respectively. When including only ANA positive patients, receiver operating characteristics analysis demonstrated that the cutoff value for EULAR/ACR-2019 criteria in cSLE and aSLE patients was 13 (sensitivity, 92.2%; specificity, 93.1%) and 10 (sensitivity, 99.1%; specificity, 85.1%), respectively. Twelve cSLE patients and seven aSLE patients only met the EULAR/ACR-2019 criteria, among whom eleven and four cases had single organ involvement, respectively. CONCLUSION: The EULAR/ACR-2019 criteria showed similar sensitivity to cSLE and aSLE patients and was more sensitive than ACR-1997 and SLICC-2012 criteria, allowing earlier recognition of patients with single or major organ involvement. The adoption of a EULAR/ACR total score ≥ 13 in this study, instead of the initially proposed ≥ 10 points, could further improve the diagnostic performance of the EULAR/ACR-2019 criteria in cSLE. Key Points • Sensitivity of the EULAR/ACR-2019 criteria was high in both cSLE and aSLE patients. • The EULAR/ACR-2019 criteria allowed earlier recognition of patients with single or major organ damage. • The adoption of a EULAR/ACR total score ≥13 could further improve the diagnostic performance of the EULAR/ACR-2019 criteria in cSLE.

Polymer Nanocomposites with High Energy Density Utilizing Oriented Nanosheets and High-Dielectric-Constant Nanoparticles.

The development of high-energy-density electrostatic capacitors is critical to addressing the growing electricity need. Currently, the widely studied dielectric materials are polymer nanocomposites incorporated with high-dielectric-constant nanoparticles. However, the introduction of high-dielectric-constant nanoparticles can cause local electric field distortion and high leakage current, which limits the improvement in energy density. In this work, on the basis of conventional polymer nanocomposites containing high-dielectric-constant nanoparticles, oriented boron nitride nanosheets (BNNSs) are introduced as an extra filler phase. By changing the volume ratios of barium titanate (BT) and BNNSs, the dielectric property of polymer nanocomposites is adjusted, and thus the capacitive energy storage performance is optimized. Experimental results prove that the oriented BNNSs can suppress the propagation of charge carriers and decrease the conduction loss. Using poly(vinylidene fluoride-co-hexafluoropropylene) (P(VDF-HFP)) as the polymer matrix, the P(VDF-HFP)/BNNS/BT nanocomposite has a higher discharged energy density compared with the conventional nanocomposite with the freely dispersed BT nanoparticles.

Polymer/molecular semiconductor all-organic composites for high-temperature dielectric energy storage.

Dielectric polymers for electrostatic energy storage suffer from low energy density and poor efficiency at elevated temperatures, which constrains their use in the harsh-environment electronic devices, circuits, and systems. Although incorporating insulating, inorganic nanostructures into dielectric polymers promotes the temperature capability, scalable fabrication of high-quality nanocomposite films remains a formidable challenge. Here, we report an all-organic composite comprising dielectric polymers blended with high-electron-affinity molecular semiconductors that exhibits concurrent high energy density (3.0 J cm-3) and high discharge efficiency (90%) up to 200 °C, far outperforming the existing dielectric polymers and polymer nanocomposites. We demonstrate that molecular semiconductors immobilize free electrons via strong electrostatic attraction and impede electric charge injection and transport in dielectric polymers, which leads to the substantial performance improvements. The all-organic composites can be fabricated into large-area and high-quality films with uniform dielectric and capacitive performance, which is crucially important for their successful commercialization and practical application in high-temperature electronics and energy storage devices.