1.A Case of Multidisciplinary Treatment for Inflammatory Myofibroblastic Tumor Complicated by ANCA-Associated Vasculitis
Shaoying WANG ; Linyi PENG ; Ke ZHENG ; Zhiwei WANG ; Dachun ZHAO ; Xia ZHANG ; Lin ZHAO ; Wenhui WANG ; Weiqing WANG ; Zhenzhen ZHU ; Jin XU ; Min SHEN
JOURNAL OF RARE DISEASES 2026;5(1):43-51
A 51-year-old male presented with nasal obstruction, followed by progressive hearing loss and blurred vision. Imaging identified space-occupying lesions in the paranasal sinuses, orbits, and paraspinal regions, while laboratory tests confirmed positive anti-proteinase 3 anti-neutrophil cytoplasmic antibody(PR3- ANCA) immunoglobulin G (IgG)and markedly elevated serum IgG4. Despite treatment with corticosteroids, immunosuppressants, and radiotherapy, the patient exhibited steroid dependency with relentless disease progression. Following multidisciplinary consultation, a diagnosis of inflammatory myofibroblastic tumor (IMT) coexisting with ANCA- associated vasculitis (AAV) was favored, though IgG4-related disease remained a critical differential. Ultimately, profound immunosuppression precipitated a severe herpesvirus infection, leading to disseminated intravascular coagulation and multiple organ dysfunction syndrome. This case underscores the rarity and diagnostic complexity of concurrent IMT and AAV, highlights the therapeutic dilemma of balancing primary disease control against fatal opportunistic infections, and emphasizes the critical role of multidisciplinary collaboration in the diagnosis and treatment of complex diseases.
2.Analysis of Clinical Prognostic Characteristics in Patients with Primary Sjögren's Syndrome-Related Renal Fanconi Syndrome
Xiaoxiao SHI ; Yuan DONG ; Jiahe JIANG ; Peng XIA ; Shuo ZHANG ; Yubing WEN ; Dong XU ; Fengchun ZHANG ; Limeng CHEN
Medical Journal of Peking Union Medical College Hospital 2026;17(2):358-369
Renal Fanconi syndrome (FS) is a rare renal manifestation of primary Sjögren's syndrome (pSS). This study aims to analyze the clinical and prognostic characteristics of patients with pSS-associated renal FS (pSS-FS) and provide insights for clinical management. Patients diagnosed with pSS-FS via renal biopsy at Peking Union Medical College Hospital from 1993 to 2024 were enrolled. Data collected included age, sex, clinical symptoms (xerostomia, xerophthalmia, skin purpura, arthralgia, polyuria, and systemic symptoms), laboratory findings [serum immunoglobulin G (IgG) and IgM, complement (C3, C4), antinuclear antibody, anti-Sjögren's syndrome-associated antigen A antibody (SSA), anti-SSB antibody, 24-hour urinary protein quantification, tubular proteinuria, serum creatinine, serum electrolytes], treatment, and follow-up information. Systematic assessments included the EULAR Sjögren's Syndrome Disease Activity Index (ESSDAI) score, pulmonary involvement (including non-infectious interstitial pneumonia, pulmonary fibrosis, pulmonary hypertension, etc.), hematological involvement (anemia, leukopenia, thrombocytopenia), etc. Efficacy evaluations encompassed improvements in immunological parameters, renal function, and tubular function. Group comparisons were performed using chi-square/Fisher's exact tests, A total of 38 patients with pSS-FS were included, with 37(97.4%) being female. The median age at pSS diagnosis was 43(37, 57) years. Xerostomia (76.3%) and xerophthalmia (71.1%) were the predominant clinical symptoms. The most common renal tubular dysfunctions were generalized aminoaciduria (96.9%), tubular proteinuria (96.0%), and hypokalemia (94.7%). The median eGFR was 52.57(32.04, 76.10)mL/(min·1.73 m2), with 60.5% (23/38) of patients having an eGFR below 60 mL/(min·1.73 m2).After six months of immunosuppressive therapy, including moderate-to-high-dose glucocorticoids, significant improvements were observed in immunological parameters (improvement rate: 69.2%), renal tubular function (89.5%), and renal function (44.4%). Following immunosuppressive treatment, the median eGFR increased from 54.95(33.06, 76.10)mL/(min·1.73 m2) to 65.56(56.24, 83.58)mL/(min·1.73 m2).Compared to patients with normal or mildly impaired baseline eGFR [≥ 60 mL/(min·1.73 m2)], those with significantly decreased baseline eGFR [< 60 mL/(min·1.73 m2)] were older (46 years This study reports the clinical characteristics of the largest single-center cohort of pSS-FS patients internationally, characterized by varying degrees of proximal renal tubular dysfunction and renal impairment. Timely initiation of immunosuppressive therapy, including glucocorticoids, is crucial, particularly for patients with significantly reduced eGFR, who may experience more substantial renal function improvement.
3.Guidelines for establishing animal models of rheumatoid arthritis with cold dampness obstruction pattern and damp heat obstruction pattern (2024 Version)
Na LIN ; Yanqiong ZHANG ; Changhong XIAO ; Shenghao TU ; Jianning SUN ; Shijun XU ; Xia MAO
Science of Traditional Chinese Medicine 2026;4(2):174-180
Rheumatoid arthritis belongs to arthralgia in the theory of traditional Chinese medicine, with cold dampness obstruction pattern (CDO) and damp heat obstruction pattern (DHO) as the main pattern types. Fine therapeutic effects have been obtained in clinical practice following the differentiation of CDO and DHO. However, suitable animal models are not available currently. For adapting to the clinical diagnosis, as well as carrying out basic research of integrated Chinese and Western medicine and preclinical study on new Chinese medicine in a better way, the “Guidelines for Establishing Animal Models of Rheumatoid Arthritis with Cold Dampness Obstruction Pattern and Damp Heat Obstruction Pattern” (referred to as “Guidelines”) were compiled by our research group in cooperation with renowned experts in clinical, pharmaceutical, zoological, and methodological research fields. According to the theory of disease and syndrome integration, experts standardized the establishment methods for animal models of rheumatoid arthritis with CDO and DHO. The Guidelines were compiled via the nominal group method according to the principle of evidence coming in main place, consensus as the auxiliary, and experience as the references. Contents such as animal species, arthritis induction methods, external stimulation conditions, and assessment indicators were specified in the Guidelines based on the comprehensive evaluation of pathogenesis homology, behavioral phenotypic consistency, and drug therapy predictability between animal models and human diseases. The Guidelines will be applicable to the research on syndromes in rheumatoid arthritis, elucidation of acting mechanism, and development of new medicine, and provide reference for standardizing other types of animal models with the integration of disease and syndrome, to promote the modernization of Chinese medicine research.
4.Serological and molecular genetic study of the Ael/AelB phenotype induced by the c. 575T>C variant in the ABO gene
Yanying DONG ; Qinqin ZUO ; Peixing XU ; Minggang ZHANG ; Xia JIANG ; Zixuan WANG ; Miao WANG ; Gengyuan LIU ; Yi DING ; Tianju WANG ; Junhui QUAN
Chinese Journal of Blood Transfusion 2026;39(7):922-927
Objective: To analyze the serological phenotypes and molecular genetic characteristics of a proband with ABO forward and reverse typing discrepancy and their family members, and to investigate the molecular genetic mechanism underlying the Ael/AelB phenotype caused by the c. 575T>C variant on an ABO
A1.02 background. Methods: Serological testing and adsorption-elution assays were performed for blood group identification of the proband and family members. PCR-SBT, cloning sequencing, and Nanopore third-generation sequencing were used to analyze ABO gene variants and haplotypes. Phyre2 and PyMOL were used to predict the effect of the p. Ile192Thr variant on the structure of glycosyltransferase A (GTA), and plasma GTA activity was evaluated using an in vitro transferase assay. Results: Among thirteen family members, four members showed a serological phenotype consistent with the Ael subtype and one with the AelB subtype. Cloning sequencing and third-generation sequencing confirmed that five family members carried the ABO
A1.02 (c. 575T>C) variant, which co-segregated with the weak A phenotype in the family. Structural modeling suggested that p. Ile192Thr may alter the local spatial conformation and hydrogen-bond interactions of GTA. Plasma GTA activity testing showed markedly reduced GTA activity in the tested individuals. Conclusion: The ABO
A1.02 (c. 575T>C) variant is associated with the Ael/AelB phenotype. The p. Ile192Thr substitution may weaken A antigen expression by altering GTA conformation and reducing enzymatic activity. This study provides additional evidence for the role of this variant in the Ael/AelB phenotype from the perspectives of familial inheritance, full-length haplotype analysis, and functional assessment, which is important for accurate ABO subtype identification and individualized transfusion strategies.
5.Clinical Efficacy and Mechanism of Anmei Dan in Treating Insomnia with Amnesia Based on Principle of Treating Different Diseases with Same Method: A Review
Qiqi FENG ; Bo XU ; Kang SUN ; Miao ZHU ; Zhi ZHANG ; Jing XIA ; Guangjing XIE ; Yuan LIU
Chinese Journal of Experimental Traditional Medical Formulae 2026;32(17):107-116
In modern society, the comorbidity of insomnia with amnesia has shown a significantly increasing incidence and become increasingly severe, emerging as a public health issue that endangers people's physical and mental health and quality of life. Anmei Dan (AMD), originally recorded in the Shishi Milu by CHEN Shiduo in the Qing Dynasty, possesses profound theoretical foundations and distinct clinical advantages in ameliorating insomnia with amnesia. Basic research has unequivocally confirmed its definite therapeutic effects on insomnia with amnesia and revealed partial mechanisms. AMD is therefore a veritable classic prescription embodying the principle of treating different diseases with the same method for insomnia and amnesia. However, few researchers have reviewed the related research progress. This article systematically summarized the theoretical connotations of traditional Chinese medicine in treating insomnia with amnesia, delineated the multi-component material basis of AMD in ameliorating insomnia with amnesia (including various chemical components such as ginsenosides, jujubosides, and salvianolic acids), and evaluate its multi-dimensional clinical efficacy (e.g., prolonging total sleep time, improving sleep efficiency, and enhancing memory). Moreover, it systematically summarizes the multi-pathway mechanisms of action. The results indicate that AMD has a clear material basis, definite clinical efficacy, and a favorable safety profile in ameliorating insomnia and amnesia. AMD may exert the therapeutic effect by regulating sleep-wake rhythms, enhancing synaptic plasticity, inhibiting neuroimmune inflammation, and modulating autophagy, oxidative stress, and energy metabolism. In conclusion, AMD exerts synergistic effects through multi-component, multi-dimensional, and multi-pathway approaches, serving as an important prescription for enriching and developing the theory of treating different diseases with the same method for insomnia with amnesia.
6.Genomic characterization of Legionella pneumophila isolated from industrial circulating cooling water
Man YU ; Ying TANG ; Xiangjun ZHANG ; Chenyi TAO ; Yong NING ; Yi XU ; Xiaodong JIA ; Xia ZHANG
China Occupational Medicine 2026;53(1):50-57
Objective To analyze the genomic characteristics of Legionella pneumophila (L. pneumophila) isolated from industrial circulating cooling water in workplace. Methods A total of 20 L. pneumophila strains isolated from industrial circulating cooling water collected from eight chemical enterprises were selected as the research subjects. Whole-genome sequencing was performed on the strains, followed by multilocus sequence typing (MLST), whole-genome single nucleotide polymorphism analysis, virulence factor profiling, and antibiotic resistance gene detection. Results The dominant MLST typing among the 20 L. pneumophila strains was ST2. Serogroup 1 (Lp1) strains exhibited high genetic diversity. Strains from different enterprises showed substantial genomic diversity, suggesting possible inter-enterprise dissemination. Each isolate harbored 175-295 virulence genes; among them, virulence genes were identified, and 139 genes were detected in all the 20 strains. These genes primarily included those related to the Dot/Icm type Ⅳ bacterial secretion system, flagellar synthesis and motility, and type Ⅳ pili. The number of virulence genes carried by L. pneumophila strains of different ST types differed (P<0.01), with unidentified sequence types harboring more virulence genes than other STs (P<0.01). A total of four resistance genes were predicted, including aminoglycoside phosphotransferase-encoding gene APH(9)-Ia, carbapenemase-encoding gene OXA-29, and efflux pump genes-encoding genes LpeA and LpeB, with detection rates of 100.00%, 100.00%, 10.00%, and 10.00%, respectively. These genes may confer resistance to cephalosporins, penicillins, and aminoglycosides, and all strains were multidrug resistance. Conclusion L. pneumophila isolated from industrial recirculating cooling water exhibits high genetic diversity, strong pathogenicity, and significant multidrug resistance potential.
7.Clinical Significance of Decreased Serum IgA Levels in IgG4-Related Disease
Xia ZHANG ; Yuetong XU ; Difei LIAN ; Zhihui WANG ; Hang ZHOU ; Fan YANG ; Yanying LIU
JOURNAL OF RARE DISEASES 2026;5(3):292-302
To explore the clinical significance of decreased serum immunoglobulin (Ig) A levels in patients with IgG4-related disease (IgG4-RD). A retrospective analysis was performed on patients with IgG4-RD hospitalized at Beijing Friendship Hospital, Capital Medical University from January 2012 to December 2024. Patients were divided into three groups according to serum IgA levels: decreased IgA group (< 70 mg/dL), normal group (70-400 mg/dL), and elevated group (> 400 mg/dL), with the decreased IgA group set as the main research subject. Clinical data were collected to compare clinical characteristics and organ involvement among the three groups, and the correlations between IgA levels and various clinical indicators were analyzed. To further verify the robustness of the results, propensity score matching(PSM) with a 1∶2 matching ratio was performed between the decreased IgA group and the normal IgA group for sensitivity analysis. Meanwhile, principal component analysis (PCA) was used to evaluate the overall distribution differences in clinical characteristics among the three groups with different IgA levels. A total of 330 patients with IgG4-RD were included, including 14 in the decreased IgA group, 297 in the normal IgA group, and 19 in the elevated IgA group. The decreased IgA group had a significantly longer median disease course than the normal IgA group and the elevated IgA group (all Decreased IgA level represents a distinct immune subtype of IgG4-RD, with distinct immunological features (elevated IgG/IgG4 levels, reduced C4 level, and increased EOS count), independent of age, gender and disease course. These findings suggest that serum IgA level has potential value for immune typing of IgG4-RD.
8.Correlation between fundus blood flow parameters and carotid artery ultrasound blood flow parameters in patients with hypertensive retinopathy
Fang YUAN ; Wenxiu XIA ; Peiqiu XU ; Yawei LI ; Junchen CHEN ; Xiaoling ZHANG
International Eye Science 2025;25(2):306-310
AIM: To investigate the correlation between fundus blood flow parameters and carotid artery ultrasound blood flow parameters in patients with hypertensive retinopathy(HRP).METHODS: A total of 50 patients(22 left eyes and 28 right eyes)with HRP admitted to our hospital from June 2021 to June 2023 were retrospectively included as the experimental group, and 50 healthy physical examination subjects(22 left eyes and 28 right eyes)during the same period were included as the healthy group. Pearson correlation was used to analyze the correlation between fundus blood flow parameters and carotid artery ultrasound blood flow parameters.RESULTS: The AUC values of fundus blood flow parameters and carotid artery ultrasound blood flow parameters and their combined diagnosis of HRP were 0.853, 0.844 and 0.935, respectively. Pearson correlation analysis showed that carotid systolic peak blood flow velocity was negatively correlated with foveal avascular zone(FAZ)area, FAZ circumference and non-circularity index, and positively correlated with macular vascular density(all P<0.05). The end-diastolic blood flow velocity was positively correlated with FAZ area and macular vascular density(all P<0.05). The internal carotid artery resistance index was positively correlated with FAZ area(P<0.05).CONCLUSION: The combination of fundus blood flow parameters and carotid artery ultrasound blood flow parameters in the diagnosis of HRP has good application value in the diagnosis of HRP.
9.Effects of Yunpi Tongchang Formula on intestinal mucosal barrier damage via TLR4/MyD88/NF-κB signaling pathway in rats with opioid-induced constipation of Spleen-Kidney Yang Deficiency Syndrome
Lu-mei ZHANG ; Zhi-ming ZHANG ; Zhong-yang SONG ; Xin WANG ; Qian XU ; Xia YANG ; Xin-yu LI ; Yan-yun SHEN ; Hai-hong ZHAO ; Zhi-gang WANG
Chinese Traditional Patent Medicine 2025;47(7):2205-2212
AIM To investigate the effects of Yunpi Tongchang Formula on intestinal mucosal barrier damage in rats with opioid-induced constipation(OIC)of Spleen-Kidney Yang Deficiency Syndrome.METHODS In contrast to the 10 rats of the blank group,the 50 rats of the modeling group were induced into models of OIC of Spleen-Kidney Yang Deficiency Pattern by 7 days consecutive administration of both subcutaneous loperamide injection and alternating gavage of activated carbon ice water and vinegar.Following successful modeling,rats were randomly allocated into the model group,the mosapride citrate tablet group(1.35 mg/kg),and the high-dose,medium-dose,and low-dose Yunpi Tongchang Formula groups(15.12,7.56,3.78 g/kg),with 8 mice in each group.Upon the completion of the 14 days treatment,the rats had their TCM Syndrome scores assessed;their fecal water content,initial black stool excretion time,and small intestine propulsion rate measured;their colon tissue morphology observed by HE staining;their serum levels of IL-6,TNF-α,and IL-1β detected by ELISA;their expressions of occludin and zonula occludens-1(ZO-1)in colon tissues detected by immunohistochemistry;their mRNA expressions of MyD88,TLR4 and NF-κB p65 in the colon tissues detected by RT-qPCR;and their protein expressions of MyD88,TLR4 and NF-κB p65 in the colon tissues detected by Western blot.RESULTS Compared to the blank group,the model group had higher TCM Syndrome scores(P<0.01);lower fecal water content and small intestine propulsion rate(P<0.05,P<0.01);longer initial black stool excretion time(P<0.01);more mucosal edema in colon tissue,obvious inflammatory infiltration,and glandular disorder;increased serum levels of IL-6,TNF-α and IL-1 β(P<0.05);decreased colon expressions of ZO-1 and occludin(P<0.01);and increased mRNA and protein expressions of TLR4,MyD88 and NF-κB p65(P<0.01).Compared to the model group,both the medium-dose Yunpi Tongchang Formula group and the mosapride citrate tablet group demonstrated effectively reduced TCM syndrome scores(P<0.01);increased fecal water content and small intestine propulsion rate(P<0.05,P<0.01);and shorter initial black stool excretion time(P<0.01);improved colon mucosal edema and inflammatory infiltration;decreased serum levels of IL-6,TNF-α and IL-1β(P<0.01);upregulated protein expressions of ZO-1 and occludin(P<0.01);and downregulated mRNA and protein expressions of TLR4,MyD88 and NF-κB p65(P<0.05,P<0.01).CONCLUSION Yunpi Tongchang Formula significantly ameliorates constipation symptoms in OIC rat models of Spleen-Kidney Yang Deficiency Syndrome because of its efficacy in attenuating intestinal inflammation and preserving the integrity of intestinal epithelial barrier structure,with its mechanistic action in downregulating TLR4/MyD88/NF-κB signaling pathway activation.
10.Expert Consensus on Combined Screening for Common Cancers(2025 Edition)
Kexin CHEN ; Wanqing CHEN ; Yubei HUANG ; Zhangyan LYU ; Fangfang SONG ; Changfa XIA ; Yongjie XU ; Lei YANG ; Chao SHENG ; Yacong ZHANG ; Peng WANG ; Yunmeng ZHANG ; Yuting JI ; Jingjing LI ; Wenxuan LI ; Jie WU ; Qianyun JIN ; Fengju SONG
China Cancer 2025;34(8):583-610
Malignant tumors(commonly referred to as cancer)represent a major global public health challenge and contribute significantly to the worldwide disease burden.Early screening plays a critical role in improving detection rates,enabling timely intervention,and enhancing pa-tient survival rates.However,current cancer screening guidelines primarily focus on site-specific screening,which may not fully address the need for comprehensive early detection.A scientifical-ly rational,multi-cancer screening approach offers several advantages:it optimizes the use of bio-logical samples,reduces time costs for participants,enhances the efficiency and comprehensive-ness of screening,and minimizes overall expenses.Such an approach also facilitates the rational allocation of healthcare resources,ultimately helping to reduce the societal burden of cancer.To address this need,the Cancer Epidemiology Committee of the Chinese Anti-Cancer Association has developed the Expert Consensus on Combined Screening for Common Cancers in China.This consensus integrates multidisciplinary expertise and synthesizes the latest domestic and interna-tional researches on cancer screening,early detection,and treatment for prevalent malignancies.Drawing upon China's unique demographic and healthcare context,as well as practical screening experiences,the consensus provides evidence-based recommendations on target populations,screening technologies,and procedural workflows for multi-cancer screening.These guidelines align with the principles and methodologies established by the World Health Organization(WHO),aiming to:enhance the effectiveness of combined cancer screening in China,improve early detec-tion rates,and provide a scientific foundation for national cancer prevention and control strategies.

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