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.Randomized Controlled Trial of Traditional Chinese Medicine Sequential Syndrome Differentiation Protocol for Reducing Relapse Rate of Frequently Relapsing/Steroid-dependent Nephrotic Syndrome in Children
Zhuiyue WANG ; Xianqing REN ; Wenqing PAN ; Tingting XU ; Xia ZHANG
Chinese Journal of Experimental Traditional Medical Formulae 2025;31(18):126-134
ObjectiveTo assess the relapse rate, clinical efficacy, and safety of a traditional Chinese medicine (TCM) sequential syndrome differentiation protocol for frequently relapsing/steroid-dependent nephrotic syndrome (FRNS/SDNS) in children. MethodsA total of 151 children with FRNS/SDNS treated in the First Affiliated Hospital of Henan University of Chinese Medicine from December 2020 to June 2024 were randomized into an observation group (77 cases) and a control group (74 cases). Both groups received Western medicine (prednisone tablets and tacrolimus capsules). In addition, the observation group additionally underwent TCM sequential syndrome differentiation and the control group received 1/10 of the TCM dose. The 6-month intervention was followed by a 12-month follow-up, totaling 18 months of observation across seven time points (before treatment and after 1, 2, 4, 24, 52, 76 weeks of treatment). The evaluation was carried out based on the following indicators. ① The relapse rates were mainly recorded after 24, 52, 76 weeks of treatment. ② The efficacy was evaluated based on the clinical remission rates after 1, 2, 4 weeks of treatment, the time to proteinuria clearance, the levels of 24-hour urine total protein (24-h UTP), serum total protein (TP), serum albumin (ALB), cholesterol (CHO), and triglycerides (TG) and the TCM symptom scores before treatment and after 24 weeks of treatment. ③ The treatment safety was evaluated based on blood routine and levels of liver enzymes, renal function indicators and blood glucose (Glu) before treatment and after 24 weeks of treatment. Results① Relapse rate: After 24 weeks of treatment, no significant difference in relapse rate was found between the two groups. The observation group showed lower relapse rates than the control group after 52 weeks of treatment [24.2% (16/66) vs. 52.5% (31/59), χ2=10.634, P<0.01] and 76 weeks of treatment [42.4% (28/66) vs. 74.6% (44/59), χ2=13.186, P<0.01] than the control group. ② Efficacy indicators: The two groups showed no significant difference in remission rate after 1 week of treatment. The observation group demonstrated higher remission rates after 2 weeks of treatment [88.2% (67/76) vs. 74.0% (54/73), Z=-1.999, P<0.05] and 4 weeks of treatment [94.7% (72/76) vs. 82.2% (60/73), Z=-2.3589, P<0.05). In addition, the observation group had shorter time to proteinuria clearance (P<0.01). After treatment, both groups showed declined 24 h-UTP, CHO, TG, and TCM symptom scores and elevated TP and ALB levels (P<0.01), and the observation group had lower CHO, TG, and TCM symptom scores and higher TP and ALB than the control group (P<0.05). ③ Safety indicators: After treatment, both groups showed declined white blood cell count (WBC), red blood cell count (RBC), hemoglobin (HB), and alanine aminotransferase (ALT) (P<0.05, P<0.01) and elevated Glu (P<0.01) and blood urea nitrogen (BUN) (P<0.05). After 24 weeks of treatment, none of WBC, RBC, HB, PLT, ALT, AST, BUN, Cr or Glu had significant differences between groups. Moreover, the incidence of adverse reactions showed no significant difference between the two groups. ConclusionThe TCM sequential syndrome differentiation protocol effectively reduces the relapse rate, improves the remission rate, shortens the time to proteinuria clearance, raised serum protein levels, lowers blood lipid levels, and alleviates symptoms, demonstrating good clinical safety in children with FRNS/SDNS.
4.Equivalence of SYN008 versus omalizumab in patients with refractory chronic spontaneous urticaria: A multicenter, randomized, double-blind, parallel-group, active-controlled phase III study.
Jingyi LI ; Yunsheng LIANG ; Wenli FENG ; Liehua DENG ; Hong FANG ; Chao JI ; Youkun LIN ; Furen ZHANG ; Rushan XIA ; Chunlei ZHANG ; Shuping GUO ; Mao LIN ; Yanling LI ; Shoumin ZHANG ; Xiaojing KANG ; Liuqing CHEN ; Zhiqiang SONG ; Xu YAO ; Chengxin LI ; Xiuping HAN ; Guoxiang GUO ; Qing GUO ; Xinsuo DUAN ; Jie LI ; Juan SU ; Shanshan LI ; Qing SUN ; Juan TAO ; Yangfeng DING ; Danqi DENG ; Fuqiu LI ; Haiyun SUO ; Shunquan WU ; Jingbo QIU ; Hongmei LUO ; Linfeng LI ; Ruoyu LI
Chinese Medical Journal 2025;138(16):2040-2042
5.Guidelines for the diagnosis and treatment of prurigo nodularis.
Li ZHANG ; Qingchun DIAO ; Xia DOU ; Hong FANG ; Songmei GENG ; Hao GUO ; Yaolong CHEN ; Chao JI ; Chengxin LI ; Linfeng LI ; Jie LI ; Jingyi LI ; Wei LI ; Zhiming LI ; Yunsheng LIANG ; Jianjun QIAO ; Zhiqiang SONG ; Qing SUN ; Juan TAO ; Fang WANG ; Zhiqiang XIE ; Jinhua XU ; Suling XU ; Hongwei YAN ; Xu YAO ; Jianzhong ZHANG ; Litao ZHANG ; Gang ZHU ; Fei HAO ; Xinghua GAO
Chinese Medical Journal 2025;138(22):2859-2861
6.Randomized, double-blind, parallel-controlled, multicenter, equivalence clinical trial of Jiuwei Xifeng Granules(Os Draconis replaced by Ostreae Concha) for treating tic disorder in children.
Qiu-Han CAI ; Cheng-Liang ZHONG ; Si-Yuan HU ; Xin-Min LI ; Zhi-Chun XU ; Hui CHEN ; Ying HUA ; Jun-Hong WANG ; Ji-Hong TANG ; Bing-Xiang MA ; Xiu-Xia WANG ; Ai-Zhen WANG ; Meng-Qing WANG ; Wei ZHANG ; Chun WANG ; Yi-Qun TENG ; Yi-Hui SHAN ; Sheng-Xuan GUO
China Journal of Chinese Materia Medica 2025;50(6):1699-1705
Jiuwei Xifeng Granules have become a Chinese patent medicine in the market. Because the formula contains Os Draconis, a top-level protected fossil of ancient organisms, the formula was to be improved by replacing Os Draconis with Ostreae Concha. To evaluate whether the improved formula has the same effectiveness and safety as the original formula, a randomized, double-blind, parallel-controlled, equivalence clinical trial was conducted. This study enrolled 288 tic disorder(TD) of children and assigned them into two groups in 1∶1. The treatment group and control group took the modified formula and original formula, respectively. The treatment lasted for 6 weeks, and follow-up visits were conducted at weeks 2, 4, and 6. The primary efficacy endpoint was the difference in Yale global tic severity scale(YGTSS)-total tic severity(TTS) score from baseline after 6 weeks of treatment. The results showed that after 6 weeks of treatment, the declines in YGTSS-TSS score showed no statistically significant difference between the two groups. The difference in YGTSS-TSS score(treatment group-control group) and the 95%CI of the full analysis set(FAS) were-0.17[-1.42, 1.08] and those of per-protocol set(PPS) were 0.29[-0.97, 1.56], which were within the equivalence boundary [-3, 3]. The equivalence test was therefore concluded. The two groups showed no significant differences in the secondary efficacy endpoints of effective rate for TD, total score and factor scores of YGTSS, clinical global impressions-severity(CGI-S) score, traditional Chinese medicine(TCM) response rate, or symptom disappearance rate, and thus a complete evidence chain with the primary outcome was formed. A total of 6 adverse reactions were reported, including 4(2.82%) cases in the treatment group and 2(1.41%) cases in the control group, which showed no statistically significant difference between the two groups. No serious suspected unexpected adverse reactions were reported, and no laboratory test results indicated serious clinically significant abnormalities. The results support the replacement of Os Draconis by Ostreae Concha in the original formula, and the efficacy and safety of the modified formula are consistent with those of the original formula.
Adolescent
;
Child
;
Child, Preschool
;
Female
;
Humans
;
Male
;
Double-Blind Method
;
Drugs, Chinese Herbal/therapeutic use*
;
Tic Disorders/drug therapy*
;
Treatment Outcome
7.Three new chalcone C-glycosides from Carthami Flos.
Jia-Xu BAO ; Yong-Xiang WANG ; Xian ZHANG ; Ya-Zhu YANG ; Yue LIN ; Jiao-Jiao YIN ; Yun-Fang ZHAO ; Hui-Xia HUO ; Peng-Fei TU ; Jun LI
China Journal of Chinese Materia Medica 2025;50(13):3715-3745
The chemical components of Carthami Flos were investigated by using macroporous resin, silica gel column chromatography, reversed-phase octadecylsilane(ODS) column chromatography, Sephadex LH-20, and semi-preparative high-performance liquid chromatography(HPLC). The planar structures of the compounds were established based on their physicochemical properties and ultraviolet-visible(UV-Vis), infrared(IR), high-resolution electrospray ionization mass spectrometry(HR-ESI-MS), and nuclear magnetic resonance(NMR) spectroscopic technology. The absolute configurations were determined by comparing the calculated and experimental electronic circular dichroism(ECD). Six flavonoid C-glycosides were isolated from the 30% ethanol elution fraction of macroporous resin obtained from the 95% ethanol extract of Carthami Flos, and identified as saffloquinoside F(1), 5-hydroxysaffloneoside(2), iso-5-hydroxysaffloneoside(3), isosafflomin C(4), safflomin C(5), and vicenin 2(6). Among these, the compounds 1 to 3 were new chalcone C-glycosides. The compounds 1, 2, 4, and 5 could significantly increase the viability of H9c2 cardiomyocytes damaged by oxygen-glucose deprivation/reoxygenation(OGD/R) at a concentration of 50 μmol·L~(-1), showing their good cardioprotective activity.
Glycosides/pharmacology*
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Flowers/chemistry*
;
Drugs, Chinese Herbal/pharmacology*
;
Carthamus tinctorius/chemistry*
;
Chalcones/pharmacology*
;
Animals
8.Theoretical discussion and research progress on treatment of glucocorticoid- induced osteoporosis with traditional Chinese medicine.
Ting-Ting XU ; Ying DING ; Xia ZHANG ; Long WANG ; Shan-Shan XU ; Chun-Dong SONG ; Wen-Sheng ZHAI ; Xian-Qing REN
China Journal of Chinese Materia Medica 2025;50(16):4437-4450
Glucocorticoid-induced osteoporosis(GIOP) is a serious metabolic bone disease caused by long-term application of glucocorticoids(GCs). Traditional Chinese medicine(TCM) has unique advantages in improving bone microstructure and antagonizing hormone toxicity. This paper systematically reviews the theoretical research, clinical application, and basic research progress of TCM intervention in GIOP. In terms of theoretical research, the theory of "kidney governing bone and generating marrow" indicates that the kidney is closely related to bone development, revealing that core pathogenesis of GIOP is Yin-Yang disharmony, which can be discussed using the theories of "Yin fire", "ministerial fire", and "Yang pathogen damaging Yin". Thus, regulating Yin and Yang is the basic principle to treat GIOP. In terms of clinical application, effective empirical prescriptions(such as Bushen Zhuanggu Decoction, Bushen Jiangu Decoction, and Zibu Ganshen Formula) and Chinese patent medicines(Gushukang Capsules, Hugu Capsules, Xianling Gubao Capsules, etc.) can effectively increase bone mineral density(BMD) and improve calcium and phosphorus metabolism. The combination of traditional Chinese and western medicine can reduce the risk of fracture and play an anti-GIOP role. In terms of basic research, it has been clarified that active ingredients of TCM(such as fraxetin, ginsenoside Rg_1, and salidroside) reduce bone loss and promote bone formation by inhibiting oxidative stress, ferroptosis, and other pathways, effectively improving bone homeostasis. Additionally, classical prescriptions(Modified Yiguan Decoction, Modified Qing'e Pills, Zuogui Pills, etc.) and Chinese patent medicines(Gushukang Granules, Lurong Jiangu Dropping Pills, Gubao Capsules, etc.) can improve bone marrow microcirculation, promote osteoblast differentiation, and inhibit bone cell apoptosis through multiple pathways, multiple targets, and multiple mechanisms. Through the above three aspects, the TCM research status on GIOP is elucidated in the expectation of providing reference for its diagnosis and treatment using traditional Chinese and western medicine treatment programs.
Osteoporosis/physiopathology*
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Humans
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Glucocorticoids/adverse effects*
;
Drugs, Chinese Herbal/administration & dosage*
;
Animals
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Medicine, Chinese Traditional
;
Bone Density/drug effects*
9.Family socioeconomic status and children's reading fluency: the chain mediating role of family reading environment and children's living and learning styles.
Wen-Xin HU ; Lei ZHANG ; Cai WANG ; Zi-Yue WANG ; Jia-Min XU ; Jing-Yu WANG ; Jia ZHOU ; Wen-Min WANG ; Meng-Meng YAO ; Xia CHI
Chinese Journal of Contemporary Pediatrics 2025;27(4):451-457
OBJECTIVES:
To study the impact of family socioeconomic status on children's reading fluency and the chain mediation effect of family reading environment and children's living and learning styles in this relationship.
METHODS:
A total of 473 children from grades 2 to 6 in two primary schools in Nanjing were selected through stratified random sampling. The children's reading fluency was assessed, and a questionnaire was used to collect information on family socioeconomic status, family reading environment, and children's living and learning styles. The mediation model was established using the Process macro in SPSS, and the Bootstrap method was employed to test the significance of the mediation effects.
RESULTS:
Family socioeconomic status, family reading environment, and children's living and learning styles were significantly positively correlated with reading fluency (P<0.001). The family reading environment and children's living and learning styles mediated the relationship between family socioeconomic status and children's reading fluency. Specifically, the independent mediation effect of family reading environment accounted for 11.02% of the total effect, while the independent mediation effect of children's living and learning styles accounted for 10.79%. The chain mediation effect of family reading environment and children's living and learning styles accounted for 7.41% of the total effect.
CONCLUSIONS
Family socioeconomic status can affect children's reading fluency through three pathways: family reading environment, children's living and learning styles, and the chain mediation effect of family reading environment and children's living and learning styles.
Humans
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Child
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Male
;
Female
;
Reading
;
Learning
;
Social Class
;
Family
10.Colonoscopy in infants: procedure and disease spectrum analysis of 184 cases.
Xiao-Li FU ; Xu-Xia WEI ; Jun-Jie XU ; Ning XUE ; Hong-Ling CHEN ; Le ZHANG
Chinese Journal of Contemporary Pediatrics 2025;27(8):917-922
OBJECTIVES:
To investigate the underlying causes and clinical manifestations in infants undergoing colonoscopy, and to analyze changes in disease spectrum.
METHODS:
Clinical data from 180 infants who underwent a total of 184 colonoscopies at the Department of Gastroenterology, Children's Hospital Affiliated to Shandong University from January 2015 to December 2024 were retrospectively analyzed. Patients were grouped by age: ≤6 months (n=41) and >6-12 months (n=139); and by examination period: 2015-2019 (n=83) and 2020-2024 (n=97). Primary causes for performing colonoscopy, final diagnoses, and disease spectrum evolution were assessed.
RESULTS:
Among 184 colonoscopies, the leading causes prompting examination were hematochezia (37.8%, 68/180), diarrhea (36.7%, 66/180), and co-occurring hematochezia and diarrhea (21.1%, 38/180). Causes for performing colonoscopy differed significantly by age group (P<0.05). Colonic polyps were only detected in the >6-12 months group (P<0.05). Compared to the 2015-2019 group, the 2020-2024 group had fewer food allergy-related gastrointestinal diseases (P<0.05) but more colitis (P<0.05).
CONCLUSIONS
Colonoscopy is essential for diagnosing infantile digestive disorders, with disease spectra varying by age and time period.
Humans
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Infant
;
Colonoscopy
;
Male
;
Female
;
Retrospective Studies
;
Infant, Newborn
;
Diarrhea/etiology*
;
Gastrointestinal Hemorrhage/etiology*

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