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.Tongnao Decoction Promotes Angiogenesis and Alleviates Cerebral Ischemic Injury via PI3K/Akt/GSK-3β Signaling Pathway
Yan LIU ; Yang WU ; Wanhui PENG ; Jingyi CHEN ; Jiale GAN ; Li LI ; Yangjingyi XIA ; Yunze LI ; Zhaoyao CHEN ; Wenlei LI ; Minghua WU
Chinese Journal of Experimental Traditional Medical Formulae 2026;32(9):100-110
ObjectiveTo investigate the mechanisms of Tongnao decoction (TND) in mice with acute ischemic stroke (AIS). MethodsFifty male C57BL/6J mice were randomly divided into a sham operation group, model group, TND low-dose group (1.86 g·kg-1), TND high-dose group (3.72 g·kg-1), and butylphthalide (NBP) group (10 mg·kg-1), with 10 mice in each group. A mouse model of cerebral ischemic injury was established using photochemical thrombosis (PT). The sham operation group and model group were administered an equal volume of normal saline by gavage. All five groups were treated once daily for 14 consecutive days. Behavioral tests were performed before modeling and at the end of administration. T2-weighted imaging (T2WI) was performed 3 days after modeling to evaluate the extent of injury. Hematoxylin-eosin (HE) staining was used to observe histological changes in the cerebral cortex, and Nissl staining was used to observe neuronal morphology. Cerebral blood flow in mice was detected using a laser speckle contrast imaging (LSCI) system. Immunofluorescence staining was used to detect the cell proliferation marker bromodeoxyuridine (BrdU) and the highly glycosylated type I transmembrane glycoprotein CD34. Western blot analysis was used to detect the expression levels of phosphatidylinositol 3-kinase (PI3K), protein kinase B (Akt), glycogen synthase kinase-3β (GSK-3β), and their phosphorylation levels, as well as tight junction-related proteins zonula occludens-1 (ZO-1), Occludin, and Claudin-5 in the peri-infarct tissue. Thirty-five zebrafish were randomly divided into normal control group, model group, TND low and high dose groups (0.16, 0.32 g·L-1) and NBP group (10 μmol·L-1), with 7 in each group. A stereoscopic fluorescence microscope was used to observe vascular growth in zebrafish. ResultsImaging showed that PT caused ischemia in the right cortical region. Behavioral tests indicated that, compared with the model group, the drug-treated groups reduced the error rate of irregular balance ladder climbing on the affected side and shortened the tape removal time (P<0.05). HE staining and Nissl staining showed that, compared with the model group, the drug-treated groups exhibited reduced brain tissue damage, fewer scars, and improved neuronal morphology. LSCI results showed that the drug-treated groups partially restored cerebral blood perfusion and promoted the establishment of collateral circulation compared with the model group. Immunofluorescence staining indicated that the drug-treated groups increased the positive rates of BrdU and CD34 compared with the model group (P<0.01), promoting angiogenesis. Meanwhile, compared with the model group, the drug-treated groups upregulated the expression levels of p-PI3K, p-Akt, p-GSK-3β, and tight junction proteins ZO-1, Occludin, and Claudin-5 (P<0.05,P<0.01), and increased the number of intersegmental vessels in zebrafish (P<0.05,P<0.01). ConclusionTND can promote angiogenesis around the infarct in PT model mice by regulating the PI3K/Akt/GSK-3β signaling pathway, thereby improving cerebral ischemic injury.
4.Investigation of somatization symptoms and related factors in adolescents during frequent earthquakes in Hefei
Yu ZHUANG ; Pei TANG ; Yinghan TIAN ; Peng YAO ; Lei XIA ; Huanzhong LIU
Acta Universitatis Medicinalis Anhui 2026;61(1):141-145
ObjectiveTo investigate somatization symptoms in adolescents during frequent earthquakes in Hefei, and to explore their correlation with earthquake experiences. MethodsA cross-sectional survey was used to select 324 adolescents in Hefei as the survey objects. The self-rating scale of somatization symptoms (SSS) and the fatigue intensity scale (FIS) were used to evaluate the somatization symptoms and fatigue degree of middle school students, and multivariate Logistic regression analysis was used to explore the related factors of somatization symptoms and fatigue among middle school students. ResultsA total of 324 adolescents were included, and the overall detection rate of somatization symptoms was 6.5%, and the detection rate of moderate or above fatigue was 20.1%. The results of regression analysis showed that adolescents who were concerned about the earthquake for a longer time (≥1 h) had a higher risk of somatization symptoms (OR=5.430, 95%CI: 1.547-19.058), and adolescents who received pre-earthquake training had a lower degree of fatigue (OR=0.535, 95%CI: 0.292-0.981) (P<0.05). ConclusionDuring the frequent earthquakes, adolescents have more somatization symptoms and fatigue. Therefore, it is crucial to enhance health education, reduce the emphasis on event-related reports, and implement earthquake prevention and disaster reduction training to improve the physical and mental health of adolescents.
5.Key scientific issues and breakthrough paths to eliminate the harm of hepatitis B virus infection
Yixue WANG ; Bo PENG ; Lei WEI ; Quanxin LONG ; Yuchen XIA ; Yinyan SUN ; Wenhui LI
Journal of Clinical Hepatology 2026;42(1):2-6
Hepatitis B virus (HBV) exclusively infects liver parenchymal cells and forms covalently closed circular DNA (cccDNA) within their nuclei. HBV cccDNA serves as the essential template for viral gene transcription, the sole source of progeny virus production, and the key driver of viral antigen expression, and it is the molecular basis for the persistence of HBV infection. Therefore, elimination and/or functional silencing of cccDNA is the key to eradicate chronic HBV infection. This article discusses the critical scientific issues that need to be solved during elimination of the harm of HBV infection from the perspectives of the synthesis, transcription, and clearance of cccDNA, as well as the impact of nonparenchymal cells on cccDNA, in order to provide a reference for eradicating HBV infection in the future.
6.Optimizing the whole-process quality control system of intravenous drug distribution center based on failure mode and effect analysis
Wei WEI ; Mingxia ZHANG ; Yanping ZHOU ; Lan YAN ; Peng TIAN ; Xia FENG
Journal of Pharmaceutical Practice and Service 2026;44(6):322-328
Objective To explore the application effect of a standardized management method based on failure mode and effect analysis (FMEA) in optimizing the whole-process quality control system of the intravenous admixture service (PIVAS). Methods The quality control management system of the PIVAS was optimized by establishing six quality control groups led by the head nurse, with full participation of pharmacy, nursing, and logistical staff, ensuring comprehensive coverage and traceability of all quality control links. Each group conducted risk priority number (RPN) scoring for potential failure modes in their respective quality control processes, and targeted improvement measures were formulated based on the scoring results. The RPN values of failure modes and quality control-related evaluation indicators before and after implementation were compared to achieve closed-loop management. Results After one year of management, the RPN values of the six major failure modes significantly decreased compared to those before implementation (P<0.05). The compounding error rate dropped to 0.13%, the dispensing error rate decreased to 0.95%, the compounding efficiency increased to 98%, the delivery time was shortened by 0.45 h per batch, the intervention rate for irrational prescriptions rose to 94.87%, satisfaction improved to 96.78%, and the participation rate of quality control personnel reached 95.36% (P<0.05). Conclusion FMEA-based identification of potential failure modes in the whole-process quality control system of the IVAS, combined with risk quantification and targeted interventions, significantly reduced high-risk failure modes, improved compounding accuracy and efficiency, and ensured the safety of clinical intravenous medication and the effectiveness of healthcare quality management.
7.Optimizing the whole-process quality control system of intravenous drug distribution center based on failure mode and effect analysis
Wei WEI ; Mingxia ZHANG ; Yanping ZHOU ; Lan YAN ; Peng TIAN ; Xia FENG
Journal of Pharmaceutical Practice and Service 2026;44(6):322-328
Objective To explore the application effect of a standardized management method based on failure mode and effect analysis (FMEA) in optimizing the whole-process quality control system of the intravenous admixture service (PIVAS). Methods The quality control management system of the PIVAS was optimized by establishing six quality control groups led by the head nurse, with full participation of pharmacy, nursing, and logistical staff, ensuring comprehensive coverage and traceability of all quality control links. Each group conducted risk priority number (RPN) scoring for potential failure modes in their respective quality control processes, and targeted improvement measures were formulated based on the scoring results. The RPN values of failure modes and quality control-related evaluation indicators before and after implementation were compared to achieve closed-loop management. Results After one year of management, the RPN values of the six major failure modes significantly decreased compared to those before implementation (P<0.05). The compounding error rate dropped to 0.13%, the dispensing error rate decreased to 0.95%, the compounding efficiency increased to 98%, the delivery time was shortened by 0.45 h per batch, the intervention rate for irrational prescriptions rose to 94.87%, satisfaction improved to 96.78%, and the participation rate of quality control personnel reached 95.36% (P<0.05). Conclusion FMEA-based identification of potential failure modes in the whole-process quality control system of the IVAS, combined with risk quantification and targeted interventions, significantly reduced high-risk failure modes, improved compounding accuracy and efficiency, and ensured the safety of clinical intravenous medication and the effectiveness of healthcare quality management.
8.A preliminary investigation on the carriage of Bartonella by rodents at key ports in western Inner Mongolia
Ruo-wen GUO ; Huai-bo WEI ; Peng LUO ; Xia LIU ; Zong-di LIU ; Jing WU ; Jia XU
Acta Parasitologica et Medica Entomologica Sinica 2026;33(2):134-140
Objective This study investigated the diversity of rodent populations and the infection status of Bartonella at three ports along the China-Mongolia border in 2022. Methods Rodents at three Belt and Road ports along the China-Mongolia border, namely Ganqimaodu, Erenhot, and Zhuengadabuqi, were morphologically identified. The Bartonella citrate synthase(gltA)gene was amplified by nested polymerase chain reaction(PCR), and PCR-positive products were sequenced. The resulting sequences were then analyzed for genetic characteristics. Phylogenetic analysis was performed using MEGA 11.0 software using Neighbor-Joining and Maximum-Likelihood method. Results A total of 94 rodents were captured, representing seven species from five families and six genera: Mus musculus, Meriones unguiculatus, Meriones meridianus, Spermophilus dauricus, Allactaga sibirica, Dipus sagitta, and Phodopus roborovskii. Among them, M. unguiculatus was the most abundant species, with 58 rodents, accounting for 61.70% of the total. Overall,11 positive Bartonella pathogen sequences were obtained from the three ports, with a positive detection rate of 11.70%(11/94). The infected rodent species included M. unguiculatus, M. meridianus, and A. sibirica, and two species of Bartonella were detected. Conclusions Rodents at Ganqimaodu, Erenhot, and Zhuengadabuqi ports along the China-Mongolia border were naturally infected with Bartonella. Rodent monitoring and pathogen prevention and control in this area should be strengthened.
9.Left sided sternocleidomastoid interosseous intravascular papillary endothelial hyperplasia: A case report.
Xiaodi XIAO ; Youchen XIA ; Jianying LIU ; Peng FU
Journal of Peking University(Health Sciences) 2025;57(5):1002-1004
Intravascular papillary endothelial hyperplasia (IPEH), also known as Masson tumor, is a rare vascular benign tumor of blood vessels. It may occur in any part of the body, especially the deep dermis and subcutaneous tissue of the head, neck, fingers and trunk. The imaging and histopathology of IPEH are similar to hemangiosarcoma, especially in the case of active vascular endothelial hyperplasia. IPEH is a reactive proliferative lesion of vascular intima. The etiology is still unclear. After some studies showed that IPEH was a benign lesion, few reports on the etiology of it were reported. IPEH is usually limited to the thrombotic vessels or lumens of vascular malformations, usually accompanied by a clear history of trauma. IPEH usually does not cause any symptoms. It looks like a slow-growing lump. Some cases have been reported with pain and swelling. Although IPEH is relatively rare, its accurate diagnosis is crucial because it may be similar to malignant angiogenic lesions in clinical practice. There were few reports of cases related to intravascular papillary endothelial hyperplasia located in the sternocleidomastoid muscle after reviewing the domestic and foreign literature in recent 10 years. This case reports that a young male, who was admitted to the hospital one month after finding a subcutaneous tumor in the left neck. After admittance, relevant preoperative examinations were completed. After multi-disciplinary discussion and elimination of surgical contraindications, a specific surgical plan was formulated. The tumor was removed under local anesthesia on the second day after admission. During the operation, it was found that the tumor was located between the sternocleidomastoid muscle bundles, and it was sent for pathologic examination. Paraffin section pathology was reported after operation. Histological examination showed that the morphology was consistent with vascular endothelial papillary hyperplasia. There were no related surgical complications and recurrence in the 3-month follow-up. The purpose of this paper is to provide clinicians with a certain understanding of this rare disease through the report of this case of IPEH, and to identify it in later clinical work, and at the same time, to avoid confusion with malignant diseases, such as hemangiosarcoma, leading to unnecessary treatment and increase the cost of treatment.
Humans
;
Male
;
Endothelium, Vascular/pathology*
;
Hemangioendothelioma/surgery*
;
Hyperplasia/pathology*
;
Neck Muscles/surgery*
;
Vascular Neoplasms/pathology*
10.Glucocorticoid Discontinuation in Patients with Rheumatoid Arthritis under Background of Chinese Medicine: Challenges and Potentials Coexist.
Chuan-Hui YAO ; Chi ZHANG ; Meng-Ge SONG ; Cong-Min XIA ; Tian CHANG ; Xie-Li MA ; Wei-Xiang LIU ; Zi-Xia LIU ; Jia-Meng LIU ; Xiao-Po TANG ; Ying LIU ; Jian LIU ; Jiang-Yun PENG ; Dong-Yi HE ; Qing-Chun HUANG ; Ming-Li GAO ; Jian-Ping YU ; Wei LIU ; Jian-Yong ZHANG ; Yue-Lan ZHU ; Xiu-Juan HOU ; Hai-Dong WANG ; Yong-Fei FANG ; Yue WANG ; Yin SU ; Xin-Ping TIAN ; Ai-Ping LYU ; Xun GONG ; Quan JIANG
Chinese journal of integrative medicine 2025;31(7):581-589
OBJECTIVE:
To evaluate the dynamic changes of glucocorticoid (GC) dose and the feasibility of GC discontinuation in rheumatoid arthritis (RA) patients under the background of Chinese medicine (CM).
METHODS:
This multicenter retrospective cohort study included 1,196 RA patients enrolled in the China Rheumatoid Arthritis Registry of Patients with Chinese Medicine (CERTAIN) from September 1, 2019 to December 4, 2023, who initiated GC therapy. Participants were divided into the Western medicine (WM) and integrative medicine (IM, combination of CM and WM) groups based on medication regimen. Follow-up was performed at least every 3 months to assess dynamic changes in GC dose. Changes in GC dose were analyzed by generalized estimator equation, the probability of GC discontinuation was assessed using Kaplan-Meier curve, and predictors of GC discontinuation were analyzed by Cox regression. Patients with <12 months of follow-up were excluded for the sensitivity analysis.
RESULTS:
Among 1,196 patients (85.4% female; median age 56.4 years), 880 (73.6%) received IM. Over a median 12-month follow-up, 34.3% (410 cases) discontinued GC, with significantly higher rates in the IM group (40.8% vs. 16.1% in WM; P<0.05). GC dose declined progressively, with IM patients demonstrating faster reductions (median 3.75 mg vs. 5.00 mg in WM at 12 months; P<0.05). Multivariate Cox analysis identified age <60 years [P<0.001, hazard ratios (HR)=2.142, 95% confidence interval (CI): 1.523-3.012], IM therapy (P=0.001, HR=2.175, 95% CI: 1.369-3.456), baseline GC dose ⩽7.5 mg (P=0.003, HR=1.637, 95% CI: 1.177-2.275), and absence of non-steroidal anti-inflammatory drugs use (P=0.001, HR=2.546, 95% CI: 1.432-4.527) as significant predictors of GC discontinuation. Sensitivity analysis (545 cases) confirmed these findings.
CONCLUSIONS
RA patients receiving CM face difficulties in following guideline-recommended GC discontinuation protocols. IM can promote GC discontinuation and is a promising strategy to reduce GC dependency in RA management. (Trial registration: ClinicalTrials.gov, No. NCT05219214).
Adult
;
Aged
;
Female
;
Humans
;
Male
;
Middle Aged
;
Arthritis, Rheumatoid/drug therapy*
;
Glucocorticoids/therapeutic use*
;
Medicine, Chinese Traditional
;
Retrospective Studies


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