Objective: To investigate the differential expression of Yes-associated protein(YAP) in the healthy control group and in patients with rheumatoid arthritis(RA), and to explore its correlation with the progression of the disease. Methods: Three cases each of synovial membranes from the healthy control group and RA group were selected for RNA-sequence detection to identify differentially expressed genes. Data and laboratory indicators from 50 healthy control cases and 150 RA patients who met the criteria were collected. ELISA was used to detect the expression of YAP in the serum of each group, and then its correlation with other laboratory indicators in RA patients was analyzed. The receiver operating characteristic curve was applied to explore the diagnostic efficacy of the related risk factors for RA. Results: Expression of 14-3-3-gama, which regulated the disease process, was significantly down-regulated in the synovial membranes of RA patients compared to normal synovial membranes; YAP expression was significantly up-regulated in rheumatoid arthritis fibroblast-like synoviocytes compared to healthy controls and was correlated with haematological sedimentation, C-reactive protein, interleukin(IL)-1, IL-4, IL-6, IL-10, rheumatoid factor, anti-cyclic citrullinated polypeptide antibody, interferon alpha, tumour necrosis factor alpha, protein electrophoresis α1 globulin, protein electrophoresis alpha2 globulin, disease activity scores, disease activity index, and patient-reported outcome had correlations(P0.05); multifactor Logistic regression analysis showed that YAP expression was an independent influencing factor for the prognosis of RA patient. Conclusion YAP is differentially expressed between the healthy control group and RA group, which shows a significant positive correlation with the disease activity of RA, potentially becoming a new target for clinical treatment of RA.

In 2024, significant advancements have been achieved in the field of pediatric rheumatic and immunological diseases, spanning key conditions such as juvenile idiopathic arthritis (JIA), systemic lupus erythematosus (SLE), and autoinflammatory diseases (AIDs). These developments encompass the indepth elucidation of disease pathogenesis, the expanded application of biomarkers, continuous updates to clinical practice guidelines, and the development of novel targeted therapies. These advances have markedly enhanced the precision and effectiveness of clinical diagnosis and treatment while laying a solid foundation for improving long-term patient outcomes. This article highlights the key research breakthroughs and significant progress in pediatric rheumatic and immunological diseases in 2024, aiming to advance clinical understanding, promote early recognition, and enable accurate diagnosis to optimize outcomes for affected children.

Objective:To analyze the clinical characteristics, treatment, response to treatment, prognosis, and the importance of early recognition and treatment of pediatric systemic lupus erythematosus (SLE) complicated with thrombotic microangiopathy (TMA).Methods:A retrospective summary of the clinical data of 5 children diagnosed with SLE complicated by TMA at Beijing Children′s Hospital, Capital Medical University, from November 2024 to January 2025.Results:Among the 5 children (1 boy and 4 girls, male-to-female ratio of 1∶4), the age of onset ranged from 11 years and 9 months to 14 years and 9 months. All cases had acute onset, rapid disease progression, severe illness, and involvement of multiple organs and systems. The disease activity of SLE was moderately to severely active when TMA was diagnosed. During the course of TMA, all 5 children exhibited varying degrees of hemolytic anemia, thrombocytopenia, renal dysfunction, and proteinuria. Elevated sC5b-9 levels were observed in all 5 children, with 3 showing severely reduced ADAMTS13 activity and 2 with elevated ADAMTS13 inhibitors. Two children had elevated sC5b-9, severely reduced ADAMTS13 activity, and elevated ADAMTS13 inhibitors simultaneously. Four children had newly diagnosed SLE with TMA and achieved stable condition within 2-3 weeks after aggressive treatment, including methylprednisolone pulse therapy, immunosuppressive agents, biologics, plasma infusion, plasma exchange, or dialysis. One child, who had been diagnosed with SLE for 8 years and had irregular oral medication for half a year, suddenly developed TMA with refractory hemolytic anemia and severe thrombocytopenia. This child responded poorly to methylprednisolone pulse therapy, cyclosporine, and eculizumab but showed improvement with plasma exchange. However, the condition was prone to relapse when the interval between plasma exchanges was prolonged. The child eventually responded well to low-dose rituximab and was discharged after 6 weeks of combined treatment. Four children were diagnosed with TMA within 1 week of admission and achieved stable condition after 3 months of follow-up, with no anemia or thrombocytopenia, negative proteinuria, normal complement C3 and C4 levels, and an SLE disease activity score of 0. One child, who had been treated with high-dose glucocorticoid, multiple immunosuppressive agents, and biologics at another hospital, was transferred and diagnosed with TMA 2 weeks after admission. The condition gradually stabilized after treatment with methylprednisolone pulse therapy, immunosuppressive agents, biologics, and plasma exchange. However, at 3-month follow-up, the child still had alopecia and proteinuria, with an SLE disease activity score of 6, indicating mild disease activity.Conclusion:For children with moderate to severe active SLE, especially those with acute onset, rapid disease progression, and prominent manifestations of anemia, thrombocytopenia, renal dysfunction, and poor response to high-dose methylprednisolone pulse therapy, the risk of TMA should be vigilantly monitored. Early diagnosis and aggressive treatment are crucial.

Objective:To evaluate the efficacy and safety of nebulized inhalation of recombinant human interferon (IFN) α1b injection in the treatment of respiratory syncytial virus (RSV) associated lower respiratory tract infections (pneumonia and bronchiolitis) in children.Methods:A randomized, double-blind, parallel, placebo-controlled add-on design was used.Children with pneumonia or bronchiolitis aged 2 months to 5 years who tested positive for RSV antigen within 72 hours of onset from 30 clinical trial sites including Beijing Children′s Hospital, Capital Medical University between February 2021 and December 2022 were included in this study and randomly divided into 2 groups at a ratio of 1∶1 based on a stratified-block method.Both groups received basic treatments such as cough control, asthma relieving, expectorant treatment, fever reduction, oxygen therapy, etc.The experimental group received additional nebulized inhalation of IFN α1b injection at a dose of 2.0 μg/(kg·time), twice a day.The control group received nebulized inhalation of placebo twice a day.Clinical efficacy was evaluated based on indicators such as the duration of clinical symptoms and signs, and the Kaplan-Meier method was used to calculate the median and 95% CI of the duration of clinical symptoms and signs.The Log-rank test was used to compared data between groups.Safety was assessed through the incidence of adverse reactions and laboratory tests, and the Chi-square test was used to analyze the difference between groups. Results:There were 123 children in the experimental group and 122 children in the control group.The median durations of all the 5 clinical symptoms and signs [including shortness of breath, wheezing, dyspnea (visible retractions), decreased transcutaneous oxygen saturation, and abnormal mental state] in the experimental group after treatment were slightly shortened than those in the control group [2.7 d(95% CI: 1.9-3.0 d)] vs.[2.9 d(95% CI: 2.6-3.6 d), P=0.027].The improvement in dyspnea (retractions) was especially pronounced in the experimental group, with a relief rate of 50.0% (0, 100%) on the first day of administration[compared with 0 (0, 50.0%) in the control group ( Z=2.002, P=0.025)].The median duration of dyspnea in the experimental group was nearly 1 day shorter than that in the control group [1.0 d(95% CI: 0.7-1.7 d) vs.1.8 d(95% CI: 1.0-2.5 d), P=0.046].There were no significant difference in hospital stay [6.0(5.0, 8.0) d vs.6.5(5.0, 8.0) d, Z=0.675, P=0.500], oxygen therapy duration [32.0(14.0, 96.3) h vs.39.0 (24.0, 83.2) h, Z=0.094, P=0.925], the recovery rate from clinical symptoms during treatment [(105/106, 99.1%) vs.(96/101, 95.0%)], and recurrence rate [(0/106, 0) vs.(2/101, 2.0%)] between the 2 groups (all P>0.05).However, the above-mentioned four indicators in the experimental group showed a trend of clinical benefits.The quantitative virus detection results showed that the RSV viral load in both groups decreased after treatment compared to before treatment.After 2 days of treatment, the decline rate of RSV viral load from the baseline was 0.90 lg copies/(mL·d) in the experimental group and 0.25 lg copies/(mL·d)in the control group, with a statistically significant difference ( P<0.05).Furthermore, there was no statistically significant difference in the incidence of adverse reactions between the 2 groups ( P>0.05).Importantly, no drug-related serious adverse reactions occurred in both groups. Conclusions:The nebulized inhalation therapy of IFN α1b demonstrates efficacy and safety in treating pediatric RSV associated lower respiratory tract infections.It particularly offers outstanding clinical therapeutic value for severe children.

Objective:To evaluate the efficacy and safety of nebulized inhalation of recombinant human interferon (IFN) α1b injection in the treatment of respiratory syncytial virus (RSV) associated lower respiratory tract infections (pneumonia and bronchiolitis) in children.Methods:A randomized, double-blind, parallel, placebo-controlled add-on design was used.Children with pneumonia or bronchiolitis aged 2 months to 5 years who tested positive for RSV antigen within 72 hours of onset from 30 clinical trial sites including Beijing Children′s Hospital, Capital Medical University between February 2021 and December 2022 were included in this study and randomly divided into 2 groups at a ratio of 1∶1 based on a stratified-block method.Both groups received basic treatments such as cough control, asthma relieving, expectorant treatment, fever reduction, oxygen therapy, etc.The experimental group received additional nebulized inhalation of IFN α1b injection at a dose of 2.0 μg/(kg·time), twice a day.The control group received nebulized inhalation of placebo twice a day.Clinical efficacy was evaluated based on indicators such as the duration of clinical symptoms and signs, and the Kaplan-Meier method was used to calculate the median and 95% CI of the duration of clinical symptoms and signs.The Log-rank test was used to compared data between groups.Safety was assessed through the incidence of adverse reactions and laboratory tests, and the Chi-square test was used to analyze the difference between groups. Results:There were 123 children in the experimental group and 122 children in the control group.The median durations of all the 5 clinical symptoms and signs [including shortness of breath, wheezing, dyspnea (visible retractions), decreased transcutaneous oxygen saturation, and abnormal mental state] in the experimental group after treatment were slightly shortened than those in the control group [2.7 d(95% CI: 1.9-3.0 d)] vs.[2.9 d(95% CI: 2.6-3.6 d), P=0.027].The improvement in dyspnea (retractions) was especially pronounced in the experimental group, with a relief rate of 50.0% (0, 100%) on the first day of administration[compared with 0 (0, 50.0%) in the control group ( Z=2.002, P=0.025)].The median duration of dyspnea in the experimental group was nearly 1 day shorter than that in the control group [1.0 d(95% CI: 0.7-1.7 d) vs.1.8 d(95% CI: 1.0-2.5 d), P=0.046].There were no significant difference in hospital stay [6.0(5.0, 8.0) d vs.6.5(5.0, 8.0) d, Z=0.675, P=0.500], oxygen therapy duration [32.0(14.0, 96.3) h vs.39.0 (24.0, 83.2) h, Z=0.094, P=0.925], the recovery rate from clinical symptoms during treatment [(105/106, 99.1%) vs.(96/101, 95.0%)], and recurrence rate [(0/106, 0) vs.(2/101, 2.0%)] between the 2 groups (all P>0.05).However, the above-mentioned four indicators in the experimental group showed a trend of clinical benefits.The quantitative virus detection results showed that the RSV viral load in both groups decreased after treatment compared to before treatment.After 2 days of treatment, the decline rate of RSV viral load from the baseline was 0.90 lg copies/(mL·d) in the experimental group and 0.25 lg copies/(mL·d)in the control group, with a statistically significant difference ( P<0.05).Furthermore, there was no statistically significant difference in the incidence of adverse reactions between the 2 groups ( P>0.05).Importantly, no drug-related serious adverse reactions occurred in both groups. Conclusions:The nebulized inhalation therapy of IFN α1b demonstrates efficacy and safety in treating pediatric RSV associated lower respiratory tract infections.It particularly offers outstanding clinical therapeutic value for severe children.

In 2024, significant advancements have been achieved in the field of pediatric rheumatic and immunological diseases, spanning key conditions such as juvenile idiopathic arthritis (JIA), systemic lupus erythematosus (SLE), and autoinflammatory diseases (AIDs). These developments encompass the indepth elucidation of disease pathogenesis, the expanded application of biomarkers, continuous updates to clinical practice guidelines, and the development of novel targeted therapies. These advances have markedly enhanced the precision and effectiveness of clinical diagnosis and treatment while laying a solid foundation for improving long-term patient outcomes. This article highlights the key research breakthroughs and significant progress in pediatric rheumatic and immunological diseases in 2024, aiming to advance clinical understanding, promote early recognition, and enable accurate diagnosis to optimize outcomes for affected children.

Objective:To analyze the clinical characteristics, treatment, response to treatment, prognosis, and the importance of early recognition and treatment of pediatric systemic lupus erythematosus (SLE) complicated with thrombotic microangiopathy (TMA).Methods:A retrospective summary of the clinical data of 5 children diagnosed with SLE complicated by TMA at Beijing Children′s Hospital, Capital Medical University, from November 2024 to January 2025.Results:Among the 5 children (1 boy and 4 girls, male-to-female ratio of 1∶4), the age of onset ranged from 11 years and 9 months to 14 years and 9 months. All cases had acute onset, rapid disease progression, severe illness, and involvement of multiple organs and systems. The disease activity of SLE was moderately to severely active when TMA was diagnosed. During the course of TMA, all 5 children exhibited varying degrees of hemolytic anemia, thrombocytopenia, renal dysfunction, and proteinuria. Elevated sC5b-9 levels were observed in all 5 children, with 3 showing severely reduced ADAMTS13 activity and 2 with elevated ADAMTS13 inhibitors. Two children had elevated sC5b-9, severely reduced ADAMTS13 activity, and elevated ADAMTS13 inhibitors simultaneously. Four children had newly diagnosed SLE with TMA and achieved stable condition within 2-3 weeks after aggressive treatment, including methylprednisolone pulse therapy, immunosuppressive agents, biologics, plasma infusion, plasma exchange, or dialysis. One child, who had been diagnosed with SLE for 8 years and had irregular oral medication for half a year, suddenly developed TMA with refractory hemolytic anemia and severe thrombocytopenia. This child responded poorly to methylprednisolone pulse therapy, cyclosporine, and eculizumab but showed improvement with plasma exchange. However, the condition was prone to relapse when the interval between plasma exchanges was prolonged. The child eventually responded well to low-dose rituximab and was discharged after 6 weeks of combined treatment. Four children were diagnosed with TMA within 1 week of admission and achieved stable condition after 3 months of follow-up, with no anemia or thrombocytopenia, negative proteinuria, normal complement C3 and C4 levels, and an SLE disease activity score of 0. One child, who had been treated with high-dose glucocorticoid, multiple immunosuppressive agents, and biologics at another hospital, was transferred and diagnosed with TMA 2 weeks after admission. The condition gradually stabilized after treatment with methylprednisolone pulse therapy, immunosuppressive agents, biologics, and plasma exchange. However, at 3-month follow-up, the child still had alopecia and proteinuria, with an SLE disease activity score of 6, indicating mild disease activity.Conclusion:For children with moderate to severe active SLE, especially those with acute onset, rapid disease progression, and prominent manifestations of anemia, thrombocytopenia, renal dysfunction, and poor response to high-dose methylprednisolone pulse therapy, the risk of TMA should be vigilantly monitored. Early diagnosis and aggressive treatment are crucial.

The core components of the Hippo signaling pathway encompass upstream regulatory molecules,core kinase cascade complexes,and downstream transcriptional regulation complexes.This pathway modulates cellular behaviors by influencing the effector molecules of its core components and plays a pivotal role in immune regulation.Effector molecules,such as Yes-associated protein(YAP),transcriptional coactivator with PDZ-binding motif(TAZ),transcriptional enhanced associate domain transcriptional factor(TEAD),monopolar spindle-one binder(MOB1),large tumor suppressor(LATS),can stimulate fibroblast-like synovial cell migration and invasion in rheumatoid arthritis,regulate osteoarthritis disease progression,promote pathological new bone formation in ankylosing spondylitis,sustain submandibular gland development while delaying Sjogren's syndrome progression,mediate alpha-smooth muscle actin in systemic sclerosis,and refine the regulation of target genes associated with pulmonary fibrosis.This article provides an overview of the regulatory mechanisms involving Hippo signaling pathway-related effector molecules in the pathogenesis and progression of rheumatic immune system diseases,to serve as a reference for exploring novel therapeutic targets of rheumatic immune system diseases.

Objective:To analyze the clinical characteristics of children with systemic lupus erythematosus (SLE) combined with thrombotic microangiopathy (TMA), and clarify the clinical outcomes and related risk factors of pediatric patients through their treatment and follow-up.Methods:This was a single-center retrospective case-control study. Children diagnosed with SLE combined with TMA between January 2017 and January 2023 at Beijing Children′s Hospital, Capital Medical University, were selected as the TMA group, and SLE children without TMA were selected as the control group.According to the prognosis, children in the TMA group were further divided into the good prognosis group and the poor prognosis group.The data of the children were collected, including age, gender, SLE disease activity, clinical presentations at the time of diagnosis and at the time of thrombosis, laboratory examinations, treatment strategies, prognosis, and follow-up results.The chi-square test and Z-test were used for comparison of count data.The t-test was used for comparison of metrological pairing data.The Fisher′s exact test was used to compare the differences between the 2 groups in categorical variables.The univariate Logistic regression was used to analyze the risk factors of poor prognosis. Results:There were 29 cases in the TMA group, and the incidence of TMA accounted for 2.53% of SLE patients; 33 cases were in the control group.The age at diagnosis of TMA was 13 years and 5 months (ranging from 9 years, 1 month and 5 days to 17 years and 4 months).The common clinical manifestations in order of prevalence were renal involvement (28 cases, 96.55%), hematologic involvement (26 cases, 89.66%), serous effusion (17 cases, 58.62%), rash (13 cases, 44.82%), and neurologic involvement (12 cases, 41.38%).Pleurisy or pericarditis, renal involvement and neurological involvement occurred more often in the TMA group than in the control group (17 cases vs.3 cases, 28 cases vs.10 cases, 12 cases vs.3 cases), and the TMA group showed less facial rash and arthritis than the control group (13 cases vs.25 cases, 4 cases vs.17 cases), and the differences were statistically significant (all P<0.05).The Systemic Lupus Erythematosus Disease Activity Index score in the TMA group [(24.14±9.42) scores] was significantly higher than that in the control group [(10.18±9.42) scores], and the difference was statistically significant ( t=3.233, P<0.05).The hemoglobin level, platelet count, and complement C3 level of the children in the TMA group were significantly lower than those in the control group, whereas the double stranded DNA antibody, lactate dehydrogenase, D-dimer, urea, creatinine, ferritin level, and urine protein quantitation were significantly higher than those in the control group, and the differences were statistically significant (all P<0.05).In the TMA group, 5 cases had decreased ADAMTS13 activity, and 5 cases had significantly increased complement C5b9.A total of 15 cases (51.72%) in the TMA group underwent renal biopsy, and 13 of them had combined renal TMA.In the TMA group, 28 patients (96.6%) received hormone therapy, 17 patients received plasma exchange, and 12 patients were treated with immunosuppressants and biologics; 19 patients (65.5%) improved, and 10 patients (34.5%) gave up the treatment due to deterioration of the disease.The urea level and peripheral blood fragmented erythrocyte rate in the good prognosis group were significantly lower than those in the poor prognosis group [(13.18±4.39) mmol/L vs.(21.16±10.14) mmol/L, t=2.975, P=0.006; 8/17 (47.06%) vs.7/7 (100%), χ2=5.929, P=0.015].The univariate Logistic regression analysis showed that the fragmented erythrocyte, ADAMTS13 activity and urea were the independent risk factors for poor prognosis (all P<0.05). Conclusions:SLE patients with moderate-to-severe disease activity, especially children with hemolytic anemia, thrombocytopenia, and renal dysfunction as prominent manifestations, should be alert to the risk of TMA.Early diagnosis and treatment are crucial.

Objective:To explore the current status and training needs of nurses' knowledge, belief, and behavior regarding the good limb positioning in stroke patients with hemiplegia.Methods:From November to December 2023, convenience sampling was used to select 1 708 nurses from 30 ClassⅢ hospitals in 17 provinces across the country, who worked in departments such as Neurology and Rehabilitation. The self-designed General Information Questionnaire, Knowledge, Belief and Behavior Questionnaire for Putting Good Limb Position in Clinical Nurses, and self-designed Training Status and Needs Questionnaire for Good Limb Positioning were used for online surveys. Multiple linear regression was used to analyze the influencing factors of nurses' knowledge, belief, and behavior in good limb positioning.Results:A total of 1 622 valid questionnaires were collected. Nurses scored (135.64±25.93) on the Knowledge, Belief and Behavior Questionnaire for Putting Good Limb Position in Clinical Nurses, with the lowest score rate of 65.65% in the knowledge subscale and the highest score rate of 88.16% in the belief subscale. Multiple linear regression analysis showed that education level, working years, department, and whether they received training were the influencing factors of nurses' knowledge, belief, and behavior in good limb positioning ( P<0.05). 92.23% (1 496/1 622) of nurses were willing to receive training on good limb positioning, and 70.10% (1 137/1 622) of nurses participated in training on good limb positioning. Conclusions:Nurses generally have a moderate level of knowledge, belief, and behavior towards the good limb positioning, with room for improvement in their knowledge and behavior. However, their belief is relatively positive. Nursing managers should actively carry out training on good limb positioning based on the different characteristics and needs of nurses, improve their ability to good limb positioning.