1.Arterial switch operation under an integrated management mode of prenatal diagnosis-postnatal treatment for congenital heart disease: A single-center retrospective cohort study
Zirui PENG ; Jing LING ; Jiaxiong WU ; Runzhang LIANG ; Canxin WANG ; Jinxin LI ; Haiyun YUAN ; Shusheng WEN
Chinese Journal of Clinical Thoracic and Cardiovascular Surgery 2026;33(03):415-423
Objective To evaluate the impact of an integrated management mode of prenatal diagnosis-postnatal treatment for congenital heart disease (CHD) on perioperative and long-term outcomes of the arterial switch operation (ASO), and to analyze the efficacy of ASO in a single center. Methods This retrospective study analyzed the clinical data of 183 children who underwent ASO at Guangdong Provincial People’s Hospital from 2018 to 2024. The cohort included 106 (57.9%) patients of transposition of the great arteries with intact ventricular septum (TGA/IVS), 61 (33.3%) patients of transposition of the great arteries with ventricular septal defect (TGA/VSD), and 16 (8.7%) patients of Taussig-bing anomaly (TBA). Perioperative indicators were compared between 91 patients in the prenatal-postnatal integrated management group (an integrated group) and 92 patients in the traditional management group (a non-integrated group). Long-term survival and reoperation rates were analyzed using Kaplan-Meier curves. Results The overall perioperative mortality rate was 4.9% (9/183), showing a downward trend year by year. The primary cause of perioperative mortality was low cardiac output syndrome (LCOS), which occurred in 12 patients (6.6% incidence) with a mortality rate of 75.0%. The integrated group had a higher proportion of males (89.0% vs. 72.8%, P<0.05) and lower body weight [3.1 (2.7, 3.3) kg vs. 3.3 (3.0, 3.7) kg, P<0.05] compared to the non-integrated group. The age at surgery was significantly earlier in the integrated group [7 (3, 10) d vs. 14 (9, 48) d, P<0.05], and all children in the integrated group underwent ASO within the optimal surgical window (100.0% vs. 82.6%, P<0.05). Intraoperatively, cardiopulmonary bypass time [173 (150, 207) min vs. 186 (159, 237) min, P<0.05] and aortic cross-clamp time [100 (90, 117) min vs. 116 (97, 142) min, P<0.05] were significantly shorter in the integrated group. Although the integrated group had longer postoperative mechanical ventilation time [145 (98, 214) h vs. 116 (77, 147) h, P<0.05] and higher 48-hour maximum vasoactive inotropic score [15 (10, 21) points vs. 12 (8, 16) points, P<0.05], there was no statistically significant difference in the incidence of severe complications (LCOS, necrotizing enterocolitis, extracorporeal membrane oxygenation) or mortality rate (3.3% vs. 6.5%, P=0.51) between the two groups, despite earlier surgical intervention and a higher proportion of critically ill cases in the integrated group. The length of hospital stay in the emergency surgery group was significantly shorter than that in the elective surgery group [20 (15, 28) d vs. 25 (21, 30) d, P<0.05], suggesting that early surgery may be of potential benefit. A total of 163 patients were successfully followed up for a median of 4.7 years, with a 5-year survival rate of 95.1% and a freedom from reintervention survival rate of 95.1%. There were no late deaths, and the most common postoperative complication was pulmonary artery stenosis. Conclusion The integrated management model allowed critically ill children with lower body weights to safely undergo surgery, significantly optimizing the timing of surgery and shortening intraoperative times. The long-term risk of reoperation after ASO is primarily concentrated on pulmonary artery stenosis, necessitating long-term follow-up and monitoring.
2.Olfactory Receptors Expressed in The Intestine and Their Functions
Pei-Wen YANG ; Meng-Meng YUAN ; Ying ZHOU ; Peng LI ; Gui-Hong QI ; Ying YANG ; Zhong-Yi MAO ; Meng-Sha ZHOU ; Xiao-Shuang MAO ; Jian-Ping XIE ; Yi-Nan YANG ; Shi-Hao SUN
Progress in Biochemistry and Biophysics 2026;53(3):534-549
Olfactory receptors (ORs) form the largest superfamily of G protein-coupled receptors (GPCRs). Traditionally recognized for their role in the nasal olfactory epithelium, where they mediate the sense of smell, accumulating evidence has firmly established their ectopic expression in non-olfactory tissues, including the intestine, lungs, and kidneys. The intestine, as the primary site for nutrient digestion and absorption, harbors a highly complex chemical environment. To adapt to this environment, the gut employs a sophisticated network of “chemosensors” to monitor luminal contents and maintain homeostasis. Among these sensors, intestinal ORs have emerged as crucial functional components, serving as a molecular bridge that connects environmental chemical signals—such as food-derived odorants—to specific physiological responses. This discovery has significantly deepened our understanding of how dietary flavors and compounds influence intestinal physiology at the molecular level. This review systematically summarizes the expression profiles, ligand classification, and biological functions of ORs within the gastrointestinal tract. Studies indicate that intestinal ORs exhibit distinct spatial distribution patterns across different gut segments and display cell-type specificity, particularly within enterocytes and enteroendocrine cells. These receptors function as versatile sensors capable of recognizing a wide variety of ligands, including exogenous dietary components, gut microbiota metabolites such as short-chain fatty acids, and endogenous small molecules like azelaic acid. Upon activation by specific ligands, intestinal ORs trigger intracellular signaling cascades, primarily involving the AC-cAMP-PKA pathway or calcium influx channels. A major focus of this review is to elucidate the molecular mechanisms by which these receptors regulate the secretion of gut hormones. Activation of specific ORs in enteroendocrine cells has been shown to stimulate the release of hormones such as glucagon-like peptide-1 (GLP-1), peptide YY (PYY), and serotonin (5-HT), thereby modulating systemic energy metabolism, glucose homeostasis, and gastrointestinal motility. Furthermore, the review addresses the critical roles of ORs in immune regulation and pathology. Evidence suggests that specific ORs contribute to the maintenance of intestinal immune homeostasis and may offer protection against inflammation. Beyond their involvement in inflammatory responses, ORs such as Olfr78 have been shown to regulate the differentiation and function of intestinal endocrine cells. Similarly, Olfr544 has been demonstrated to alleviate intestinal inflammation by remodeling the gut microbiome and metabolome. These findings collectively suggest that specific ORs hold promise as therapeutic targets for mitigating intestinal inflammation and maintaining gut homeostasis. Additionally, the review explores the emerging role of ORs in cancer. Although OR expression is often downregulated in tumor tissues compared to normal mucosa, activation of specific ORs by certain ligands can inhibit tumor cell proliferation and migration and induce apoptosis via pathways such as MEK/ERK and p38 MAPK. Conversely, other receptors, such as OR7C1, may serve as biomarkers for cancer-initiating cells. In conclusion, intestinal ORs represent a vital component of the gut’s sensory network. The review also discusses the translational potential of these findings. By elucidating the precise pairing relationships between dietary components and specific ORs, novel therapeutic strategies could be developed. Intestinal ORs may thus emerge as promising targets for nutritional and pharmacological interventions in metabolic diseases, inflammatory bowel diseases, and malignancies.
3.The Regulatory Effects and Mechanisms of Piezo1 Channel on Chondrocytes and Bone Metabolic Dysregulation in Osteoarthritis
Yan LI ; Tao LIU ; Yu-Biao GU ; Hui-Qing TIAN ; Lei ZHANG ; Bi-Hui BAI ; Zhi-Jun HE ; Wen CHEN ; Jin-Peng LI ; Fei LI
Progress in Biochemistry and Biophysics 2026;53(3):564-576
Osteoarthritis (OA), a highly prevalent degenerative joint disease worldwide, is defined by articular cartilage degradation, abnormal bone remodeling, and persistent chronic inflammation. It severely compromises patients’ quality of life, and currently, there is no radical cure. Abnormal mechanical stress is widely regarded as a core driver of OA pathogenesis, and the exploration of mechanical signal perception and transduction mechanisms has become crucial for deciphering OA’s pathophysiological processes. Piezo1, a key mechanosensitive cation channel belonging to the Piezo protein family, has recently gained significant attention due to its pivotal role in mediating cellular responses to mechanical stimuli in joint tissues. This review systematically examines Piezo1’s expression patterns, regulatory mechanisms, and pathological functions in OA, with a particular focus on its dual roles in modulating chondrocyte homeostasis and bone metabolism disorders, while also delving into the underlying molecular signaling pathways and potential therapeutic implications. Piezo1, consisting of approximately 2 500 amino acids and forming a unique trimeric propeller-like structure, is widely expressed in chondrocytes, osteocytes, mesenchymal stem cells, and synovial cells. It exhibits permeability to cations such as Ca2+, K+, and Na+, and directly responds to membrane tension changes induced by mechanical stimuli like fluid shear stress and mechanical overload. In OA patients and animal models, Piezo1 expression is significantly upregulated, especially in cartilage regions subjected to abnormal mechanical stress (e.g., human temporomandibular joint cartilage). This overexpression is closely associated with aggravated cartilage degeneration, increased chondrocyte apoptosis, accelerated cellular senescence, and intensified inflammatory responses. Mechanical overload and pro-inflammatory cytokines (e.g., IL-1β) are key inducers of Piezo1 upregulation: IL-1β activates the PI3K/AKT/mTOR signaling pathway to enhance Piezo1 expression, forming a pathogenic positive feedback loop that inhibits chondrocyte autophagy, promotes apoptosis, and further accelerates joint degeneration. Mechanistically, Piezo1 mediates OA progression through multiple interconnected pathways. When activated by mechanical stress, Piezo1 triggers excessive Ca2+ influx, leading to endoplasmic reticulum stress (ERS) and mitochondrial dysfunction, which directly induce chondrocyte apoptosis. This process involves the activation of downstream signaling cascades such as cGAS-STING and YAP-MMP13/ADAMTS5. YAP, a transcriptional regulator, upregulates the expression of matrix metalloproteinase 13 (MMP13) and aggrecanase (ADAMTS5), thereby accelerating cartilage matrix degradation. Additionally, Piezo1-driven Ca2+ overload promotes the accumulation of reactive oxygen species (ROS) and upregulates senescence markers (p16 and p21), accelerating chondrocyte senescence via the p38MAPK and NF-κB pathways. Senescent chondrocytes secrete senescence-associated secretory phenotype (SASP) factors (e.g., IL-6, IL-1β), further amplifying joint inflammation. In terms of bone metabolism, Piezo1 maintains joint homeostasis by promoting the differentiation of fibrocartilage stem cells into chondrocytes and balancing bone formation and resorption through regulating the FoxC1/YAP axis and RANKL/OPG ratio. Therapeutically, targeting Piezo1 shows promising potential. Preclinical studies have demonstrated that Piezo1 inhibitors (e.g., GsMTx4) can reduce joint damage and alleviate pain in OA mice. Simultaneously, siRNA-mediated co-silencing of Piezo1 and TRPV4 (another mechanosensitive channel) decreases intracellular Ca2+ concentration, inhibits chondrocyte apoptosis, and promotes cartilage repair. Conditional knockout of Piezo1 using Gdf5-Cre transgenic mice alleviates cartilage degeneration in post-traumatic OA models by downregulating MMP13 and ADAMTS5 expression. Despite existing challenges, such as off-target effects of inhibitors, inefficient local drug delivery, and interindividual genetic variability, strategies like developing selective Piezo1 antagonists, optimizing targeted nanocarriers, and combining Piezo1-targeted therapy with physical therapy provide viable avenues for clinical translation. The authors propose that Piezo1 serves as a critical therapeutic target for OA, and future research should focus on deciphering its context-dependent regulatory networks, developing tissue-specific intervention strategies, and validating their efficacy and safety in clinical trials to address the unmet medical needs of OA patients.
4.Clinical Efficacy of Janus Kinase Inhibitors in Combination with Chinese Herbal Medicine for Rheumatoid Arthritis:A Retrospective Study and A Meta-analysis
Chenguang ZHAN ; Shengqin YANG ; Xin LI ; Yu WEN ; Peng ZHANG ; Xingrui YAN ; Haifang DU ; Maojie WANG ; Xiaodong WU ; Liyan MEI ; Xiumin CHEN ; Yanlin LI ; Runyue HUANG
Journal of Traditional Chinese Medicine 2026;67(5):534-543
ObjectiveTo evaluate the efficacy and safety of Janus kinase (JAK) inhibitors combined with Chinese herbal medicine (CHM) in treating rheumatoid arthritis (RA). MethodsClinical data from 169 RA patients were retrospectively collected. Among them, 71 cases received JAK inhibitors as the control group, while 98 cases received JAK inhibitors plus CHM as the observation group, both treated for 24 weeks. The rheumatoid factor (RF), cyclic citic peptide antibody (anti-CCP), erythrocyte sedimentation rate (ESR), C-reactive protein (CRP), and white blood cell count (WBC) were recorded before and after treatment. Databases including CNKI, Wanfang, VIP, PubMed and Web of Science were searched from inception till August 31st, 2025 for randomized controlled trials (RCTs) on the combined use of JAK inhibitors and CHM for RA. The methodological quality of the included studies was evaluated using the risk of bias assessment tool. Meta-analyses were performed for RF, anti-CCP, ESR, CRP, 28-joint disease activity score (DAS28), overall clinical effective rate, and incidence of adverse events. Sensitivity analysis were also performed. ResultsThe retrospective study demonstrated that after treatment, ESR, CRP, and anti-CCP levels decreased in the observation group, while ESR and CRP levels decreased in the control group (P<0.05). Moreover, ESR and RF levels in the observation group were lower than those in the control group (P<0.05). A total of 9 RCTs involving 770 patients were included in the meta-analysis. The results indicated that the JAK inhibitors plus CHM group was superior to the JAK inhibitors group in reducing RF (MD=-8.97, 95%CI -15.01 to -2.94, P=0.004), CRP (MD=-3.34, 95%CI -3.82 to -2.86, P<0.001), ESR (MD=-5.33, 95%CI -7.98 to -2.69, P<0.001), and DAS28 score (MD=-0.54, 95%CI -0.74 to -0.34, P<0.001), as well as in improving the overall clinical effective rate (OR=4.53, 95%CI 2.55 to 8.03, P<0.001). No statistically significant differences were observed between groups in anti-CCP levels (SMD=-2.08, 95%CI -4.41 to 0.24, P=0.080) or incidence of adverse events (OR=0.93, 95%CI 0.55 to 1.57, P=0.790). ConclusionThe combination of JAK inhibitors and CHM demonstrates remarkable efficacy in treating RA, contributing to improved disease activity and reduced inflammatory markers with a favorable safety profile.
5.A study on the application of predictive extracorporeal membrane oxygenation intraoperative support in high-risk liver transplant recipients
Zhiying LEI ; Xuyong SUN ; Yuanyuan MO ; Jianhui DONG ; Ning WEN ; Rong PENG ; Juhua ZENG ; Silin LI
Organ Transplantation 2026;17(4):618-624
Objective To investigate the clinical safety and efficacy of predictive extracorporeal membrane oxygenation (ECMO) support during liver transplantation in high-risk recipients. Methods A single-center retrospective study was conducted. A total of 35 liver transplant recipients who underwent predictive ECMO assistance during surgery at the Second Affiliated Hospital of Guangxi Medical University from January 2021 to April 2025 were included. Baseline characteristics, ECMO application, ECMO operating parameters and hemodynamic conditions during ECMO operation were analyzed. The cumulative survival rate was calculated using the Kaplan-Meier method and survival curves were plotted. Results Veno-arterial ECMO in 35 recipients was established after induction of general anesthesia. The median ECMO operation time was 8.5 h, and the success rate of weaning from ECMO was 97%. The cardiac index (CI) during the non-liver period and the new liver period was higher than that before cannulation, and CI increased during the non-liver period and before weaning (all P<0.001). The mean invasive arterial pressure and central venous pressure remained stable. No ECMO-related complications occurred in the recipients. The average survival time was (25 ± 3) months. The main causes of death were severe pulmonary infection and multiple organ failure. Conclusions For liver transplant recipients with severe cardiopulmonary dysfunction, hemodynamic disorders, or multiple organ failure risk factors, implementing predictive ECMO support during liver transplantation through active risk prediction is a safe and effective circulatory guarantee strategy.
6.Discriminating Tumor Deposits From Metastatic Lymph Nodes in Rectal Cancer: A Pilot Study Utilizing Dynamic Contrast-Enhanced MRI
Xue-han WU ; Yu-tao QUE ; Xin-yue YANG ; Zi-qiang WEN ; Yu-ru MA ; Zhi-wen ZHANG ; Quan-meng LIU ; Wen-jie FAN ; Li DING ; Yue-jiao LANG ; Yun-zhu WU ; Jian-peng YUAN ; Shen-ping YU ; Yi-yan LIU ; Yan CHEN
Korean Journal of Radiology 2025;26(5):400-410
Objective:
To evaluate the feasibility of dynamic contrast-enhanced MRI (DCE-MRI) in differentiating tumor deposits (TDs) from metastatic lymph nodes (MLNs) in rectal cancer.
Materials and Methods:
A retrospective analysis was conducted on 70 patients with rectal cancer, including 168 lesions (70 TDs and 98 MLNs confirmed by histopathology), who underwent pretreatment MRI and subsequent surgery between March 2019 and December 2022. The morphological characteristics of TDs and MLNs, along with quantitative parameters derived from DCE-MRI (K trans , kep, and v e) and DWI (ADCmin, ADCmax, and ADCmean), were analyzed and compared between the two groups.Multivariable binary logistic regression and receiver operating characteristic (ROC) curve analyses were performed to assess the diagnostic performance of significant individual quantitative parameters and combined parameters in distinguishing TDs from MLNs.
Results:
All morphological features, including size, shape, border, and signal intensity, as well as all DCE-MRI parameters showed significant differences between TDs and MLNs (all P < 0.05). However, ADC values did not demonstrate significant differences (all P > 0.05). Among the single quantitative parameters, v e had the highest diagnostic accuracy, with an area under the ROC curve (AUC) of 0.772 for distinguishing TDs from MLNs. A multivariable logistic regression model incorporating short axis, border, v e, and ADC mean improved diagnostic performance, achieving an AUC of 0.833 (P = 0.027).
Conclusion
The combination of morphological features, DCE-MRI parameters, and ADC values can effectively aid in the preoperative differentiation of TDs from MLNs in rectal cancer.
7.Value of Repeat Renal Biopsy in the Treatment and Prognosis of Patients With Severe Lupus Nephritis.
Maheshati QIAOWAKE ; Wen-Ling YE ; Wei YE ; Yu-Bing WEN ; Gang CHEN ; Peng XIA ; Ke ZHENG ; Hang LI ; Li-Meng CHEN ; Xue-Mei LI
Acta Academiae Medicinae Sinicae 2025;47(5):801-810
Objective To investigate the value of repeat renal biopsy in the treatment and prognosis of nephrotic syndrome(NS)and acute kidney injury(AKI)following immunosuppressive therapy in patients with lupus nephritis(LN). Methods A retrospective analysis was conducted for the clinicopathological data and follow-up records of LN patients undergoing repeat renal biopsy at Peking Union Medical College Hospital from January 1,2009 to December 31,2021. Results A total of 76 patients(55 females,72.4%)were included in this study,with the mean age at the first biopsy being(29.0±10.4)years,the median inter-biopsy interval of 4.0(2.0,7.0) years,and the median total follow-up duration of 7.5(5.0,13.8)years.Pathological transformation occurred in 46(60.5%)patients,and 2 patients had comorbid diabetic nephropathy.At repeat renal biopsy,50(65.8%) patients presented NS.These patients demonstrated lower estimated glomerular filtration rate(eGFR)(P<0.001),higher chronicity index(CI)(P=0.029),and higher complement C3(P<0.001)and C4(P<0.001)levels than those with NS at the first renal biopsy(n=50).Among the 28(36.8%) patients with AKI at repeat renal biopsy,8(28.6%)experienced acute exacerbation of chronic renal insufficiency.These patients exhibited higher serum creatinine level(P=0.002),C4 level(P=0.033),CI(P=0.042),and prevalence of thrombotic microangiopathy(P=0.046)than the patients showing AKI at the first renal biopsy(n=16),while the activity index(AI)showed no significant difference(P=0.051).Over 50% of NS and AKI patients underwent treatment modifications post-repeat renal biopsy,with clinical remission rates comparable to those after the first renal biopsy(both P>0.05).Elevated CI(≥5,P=0.001)and serum creatinine(≥140 μmol/L,P<0.001)at repeat renal biopsy were identified as independent risk factors for poor prognosis.The patients with AKI at repeat renal biopsy had higher incidence of endpoint events than the non-AKI patients(P=0.015).Neither AKI at the first renal biopsy nor NS at both biopsies had significant associations with prognosis. Conclusions Repeat renal biopsy reveals not only sustained high disease activity but also accelerates chronic progression in LN patients,which underscore its critical role in guiding the therapy for severe LN post-immunosuppression.AKI,CI≥5,and serum creatinine ≥140 μmol/L at repeat renal biopsy are strongly associated with poor prognosis.
Humans
;
Lupus Nephritis/drug therapy*
;
Female
;
Retrospective Studies
;
Adult
;
Male
;
Prognosis
;
Biopsy
;
Kidney/pathology*
;
Acute Kidney Injury/pathology*
;
Nephrotic Syndrome/pathology*
;
Glomerular Filtration Rate
;
Young Adult
;
Immunosuppressive Agents/therapeutic use*
;
Middle Aged
8.Bibliometric and Visual Analysis of the Application of in situ Simulation in Medical Field.
Peng-Xia SUN ; Di JIANG ; Shu-Ya LI ; Yan SHI ; Shao-Wen HU ; Jing CHEN ; Fan LI
Acta Academiae Medicinae Sinicae 2025;47(5):830-842
Objective To analyze the research status of in situ simulation in the medical field and explore its hotspots and trends. Methods Relevant literature was searched in China National Knowledge Infrastructure and Web of Science core collection from the inception to February 2024.CiteSpace 6.3.R1 was used to analyze the authors,institutions,and keywords and draw visual knowledge maps. Results A total of 25 Chinese articles and 438 English articles were included.Only 14 English articles were from China.In Chinese articles,the authors with the largest number of articles were Dai Hengmao and Liu Shangkun,and the institution with the largest number of articles was Tongji Hospital affiliated to Tongji Medical College of Huazhong University of Science and Technology.There was little cooperation between the authors and institutions.In English articles,the author and institution with the largest number of articles was Auerbach Marc and Yale University,respectively,and the cooperation between authors and institutions was close.Emergency medicine,emergency event handling,and on-the-job training were the keywords with high frequency in Chinese articles.Patient safety,medical education,and cardiac arrest were the keywords with high frequency in English articles.A total of 4 clusters were generated for Chinese keywords and 13 clusters for English keywords. Conclusions The application of in situ simulation in the medical field is still in the initial stage,and the development is not balanced at home and abroad.The number of articles published and the cooperation between authors and institutions in China obviously lags behind those abroad.Treatment and care of emergency critical patients,emergency event handling and skill training,identification of latent safety threats,improvement of readiness,and promotion of medical quality improvement are the future research hotspots and research trends in this field.
Bibliometrics
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Humans
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China
;
Simulation Training
;
Education, Medical
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Emergency Medicine/education*
9.Optimization of Laser-Induced Breakdown Spectroscopy Parameters for Phosphorus Detection in Water Using Orthogonal Experiment
Xin-Yan YANG ; Xin WANG ; Wen-Wen ZHOU ; Yi-Heng LI ; Peng ZHU ; Bin WANG ; Xian-Feng ZHENG
Chinese Journal of Analytical Chemistry 2025;53(11):1921-1930
Aerosol-assisted plasma amplification laser-induced breakdown spectroscopy(LIBS)was employed for phosphorus detection in water.To address the multivariate coupling effects in LIBS and nebulization sampling system,an orthogonal experiment was employed to systematically optimize the key experimental parameters.Using the orthogonal experimental design,the parametric effects of laser energy(output voltage),signal acquisition delay,liquid velocity,and gas velocity on the signal to background ratio(SBR)of P I 213.618 nm were evaluated,and the optimal conditions were achieved,including laser energy of 86 mJ,delay time of 3 μs,gas velocity of 1.05 mL/min,and liquid velocity of 60 μL/min,which were in agreement with the control-variable optimization results.Moreover,the SBR response trends at P I 213.618 nm with all experimental parameters was strong in consistency with control-variable optimization results,which demonstrated the validity of the orthogonal array experimental design.This study established an efficient and accurate parameter optimization methodology for complex LIBS systems,significantly advancing the application of LIBS in environmental monitoring.
10.Diagnostic value of MS score in macrophage activation syndrome associated with systemic juvenile idiopathic arthritis
Lingling GENG ; Yue PENG ; Duomei SHI ; Li WANG ; Xianyan TANG ; Xinran WEN ; Wenhua ZHANG ; Xiaoqing LI
International Journal of Pediatrics 2025;52(7):476-480
Objective:To explore the diagnostic value of the macrophage activation syndrome/systemic juvenile idiopathic arthritis(MS)score in macrophage activation syndrome(MAS)associated with systemic juvenile idiopathic arthritis(sJIA),and to provide a reference for clinical work.Methods:This study was a retrospective case-control analysis,conducted on the patients initially diagnosed as sJIA-associated with MAS and admitted into the Department of Rheumatology and Immunology of Children's Hospital Affiliated to Xi 'an Jiaotong University from July 1st,2016 to June 30th,2023. All of the patients met the diagnostic criteria for patients with MAS associated with sJIA according to the 2016 European Alliance of Associations for Rheumatology(EULAR)/American College of Rheumatology(ACR)/Pediatric Rheumatology International Trials Organization(PRINTO)standards. The basic information at baseline,clinical manifestations,and auxiliary examination results were collected. The MS score was applied to re-evaluate the children diagnosed as sJIA-associated with MAS. When the MS score ≥-2.1,the possibility of sJIA with MAS was high. Thirty cases of sJIA without MAS were randomly selected as the control group.Results:There were 28 cases in the MAS group,including 13 males(46.43%)and 15 females(53.57%),with an average age of(7.51±4.01)years. Compared with the control group,the MAS group were significantly more likely to have high fever( χ2=8.539, P=0.003),hepatomegaly( χ2=11.621, P<0.001),splenomegaly( χ2=11.710, P<0.001)and neurological involvement( χ2=27.619, P<0.001),with the differences being statistically significant. Meanwhile,there were statistically significant differences between the two groups in terms of white blood cell count( Z=-4.001, P<0.001),neutrophil count( Z=-3.659, P<0.001),platelet count( Z=-4.687, P<0.001),albumin level( Z=-4.018, P<0.001),alanine aminotransferase( Z=-3.846, P<0.001),aspartate aminotransferase( Z=-5.932, P<0.001),lactate dehydrogenase( Z=-6.150, P<0.001),triglycerides( Z=-5.874, P<0.001),fibrinogen( Z=-5.808, P<0.001),ferritin( Z=-5.280, P<0.001),erythrocyte sedimentation rate( Z=-3.971, P<0.001),ferritin/erythrocyte sedimentation rate( Z=-5.433, P<0.001),reduction of two-line cells in blood( χ2=11.408, P<0.001)and the presence of hemophagocytosis in bone marrow smears( χ2=28.260, P<0.001). Moreover,there was a statistically significant difference in MS scores between the two groups( Z=-6.148, P<0.001),with higher MS scores in the MAS group. Nevertheless,this study showed the median MS scores of both groups ≥-2.1. Conclusion:The MS score was significant to a certain degree as reference for the diagnosis of MAS,and this study showed that the MS score in the MAS group was significantly higher than the control group. However,the median MS scores in both groups were no less than -2.1. This might be related to the influence of factors during the assessment,which made it necessary to optimize the cutoff values of the MS score. Therefore,prospective studies should be carried out on the role of MS score in early identification of MAS.

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