1.Exploration of Training System for Visiting Physicians in Department of Rare Diseases
Jiayuan DAI ; Jing XIE ; Jingjing CHAI ; Yueying MAO ; Chunlei LI ; Yaping LIU ; Jin XU ; Min SHEN ; Shuyang ZHANG
JOURNAL OF RARE DISEASES 2026;5(1):112-116
The construction of a training system for visiting physicians in the department of rare diseases in China is an important measure to improve the overall diagnosis and treatment capacity for rare diseases and address the critical challenge of insufficient knowledge and skills among clinicians in practice. This article systematically describes the visiting physician training system established by the Department of Rare Diseases at Peking Union Medical College Hospital. It summarizes the training objectives and positioning, design logic, and learning modules of the system, aiming to provide a reference for the construction of the specialized talent team for rare diseases in China.
2.A Case of Multidisciplinary Treatment for a Patient with Gorham-Stout Disease
Jing HU ; Ying JIN ; Yan ZHANG ; Ji LI ; Wenhui WANG ; Yue CHI ; Chunxu LI ; Zhenjie ZHANG ; Yaping LIU ; Xiaotian CHU ; Jin XU ; Min SHEN
JOURNAL OF RARE DISEASES 2026;5(1):52-59
Gorham-Stout disease(GSD) is a rare osteolytic disorder characterized by spontaneous and progressive osteolysis, along with abnormal angiogenesis and lymphangiogenesis, with no new bone formation. We present a case of a 15-year-old female admitted due to " recurrent right leg pain for 5 years, 11 months after undergoing right femoral fracture surgery". Through comprehensive integration of the patient's clinical phenotype, laboratory tests, imaging findings, pathological examinations, and molecular biological test results, GSD was considered highly likely. A multidisciplinary treatment approach was conducted, including a combination of zoledronic acid and sirolimus to inhibit osteolysis, along with rehabilitation training and orthopedic intervention, providing a personalized and comprehensive treatment strategy.
3.Guizhi Shaoyao Zhimutang and Active Components of Its Single Herbs in Treatment of Rheumatoid Arthritis: A Review
Jian LIU ; SHIPEIRU ; Shuang LI ; Jinzhao ZHAO ; Naijun CAO ; Mingxiu JIN ; Jing YU
Chinese Journal of Experimental Traditional Medical Formulae 2026;32(10):346-354
Rheumatoid arthritis (RA) is a common autoimmune disease characterised clinically by symmetrical joint pain, swelling, and stiffness. Long-term chronic synovial inflammation can lead to severe joint damage and even disability, thereby affecting quality of life for patients. Current clinical treatment of RA emphasises an integrated approach combining traditional Chinese and Western medicine, with traditional Chinese medicine offering certain advantages in reducing disease activity of RA, preventing relapses, and other aspects. Modern clinical evidence confirms that Guizhi Shaoyao Zhimutang (GSZT) is effective in improving symptoms such as immune metabolism, joint stiffness, and joint pain in RA patients. Pharmacological studies have revealed that GSZT primarily contains components such as cinnamaldehyde, total glucosides of paeony, total alkaloids of Aconiti Lateralis Radix Praeparata, glycyrrhetinic acid, zingiberone, isoimperatorin, ephedra polysaccharides, and cedrol. It improves RA symptoms via multiple mechanisms and targets, including enhancing immune responses, exerting anti-inflammatory and analgesic effects, regulating relevant signalling pathways, inhibiting cell apoptosis, and suppressing bone destruction. This paper reviewed the syndrome patterns and pharmacological basis of GSZT in the treatment of RA, as well as its clinical applications and related mechanisms, thereby providing a theoretical basis and reference for the further development and utilisation of GSZT in the treatment of RA.
4.Guizhi Shaoyao Zhimutang and Active Components of Its Single Herbs in Treatment of Rheumatoid Arthritis: A Review
Jian LIU ; SHIPEIRU ; Shuang LI ; Jinzhao ZHAO ; Naijun CAO ; Mingxiu JIN ; Jing YU
Chinese Journal of Experimental Traditional Medical Formulae 2026;32(10):346-354
Rheumatoid arthritis (RA) is a common autoimmune disease characterised clinically by symmetrical joint pain, swelling, and stiffness. Long-term chronic synovial inflammation can lead to severe joint damage and even disability, thereby affecting quality of life for patients. Current clinical treatment of RA emphasises an integrated approach combining traditional Chinese and Western medicine, with traditional Chinese medicine offering certain advantages in reducing disease activity of RA, preventing relapses, and other aspects. Modern clinical evidence confirms that Guizhi Shaoyao Zhimutang (GSZT) is effective in improving symptoms such as immune metabolism, joint stiffness, and joint pain in RA patients. Pharmacological studies have revealed that GSZT primarily contains components such as cinnamaldehyde, total glucosides of paeony, total alkaloids of Aconiti Lateralis Radix Praeparata, glycyrrhetinic acid, zingiberone, isoimperatorin, ephedra polysaccharides, and cedrol. It improves RA symptoms via multiple mechanisms and targets, including enhancing immune responses, exerting anti-inflammatory and analgesic effects, regulating relevant signalling pathways, inhibiting cell apoptosis, and suppressing bone destruction. This paper reviewed the syndrome patterns and pharmacological basis of GSZT in the treatment of RA, as well as its clinical applications and related mechanisms, thereby providing a theoretical basis and reference for the further development and utilisation of GSZT in the treatment of RA.
5.Herbal Textual Research on Inulae Flos in Famous Classical Formulas
Caixia LIU ; Yue HAN ; Yanzhu MA ; Lei GAO ; Sheng WANG ; Yan YANG ; Wenchuan LUO ; Ling JIN ; Jing SHAO ; Zhijia CUI ; Zhilai ZHAN
Chinese Journal of Experimental Traditional Medical Formulae 2026;32(3):210-221
In this paper, by referring to ancient and modern literature, the textual research of Inulae Flos has been conducted to clarify the name, origin, production area, quality evaluation, harvesting, processing and others, so as to provide reference and basis for the development and utilization of famous classical formulas containing this herb. After textual research, it could be verified that the medicinal use of Inulae Flos was first recorded in Shennong Bencaojing of the Han dynasty. In successive dynasties, Xuanfuhua has been taken as the official name, and it also has other alternative names such as Jinfeicao, Daogeng and Jinqianhua. The period before the Song and Yuan dynasties, the main origin of Inulae Flos was the Asteraceae plant Inula japonica, and from the Ming and Qing dynasties to the present, I. japonica and I. britannica are the primary source. In addition to the dominant basal species, there are also regional species such as I. linariifolia, I. helianthus-aquatili, and I. hupehensis. The earliest recorded production areas in ancient times were Henan, Hubei and other places, and the literature records that it has been distributed throughout the country since modern times. The medicinal part is its flower, the harvesting and processing method recorded in the past dynasties is mainly harvested in the fifth and ninth lunar months, and dried in the sun, and the modern harvesting is mostly harvested in summer and autumn when the flowers bloom, in order to remove impurities, dry in the shade or dry in the sun. In addition, the roots, whole herbs and aerial parts are used as medicinal materials. In ancient times, there were no records about the quality of Inulae Flos, and in modern times, it is generally believed that the quality of complete flower structure, small receptacles, large blooms, yellow petals, long filaments, many fluffs, no fragments, and no branches is better. Ancient processing methods primarily involved cleaning, steaming, and sun-drying, supplemented by techniques such as boiling, roasting, burning, simmering, stir-frying, and honey-processing. Modern processing focuses mainly on cleaning the stems and leaves before use. Regarding the medicinal properties, ancient texts describe it as salty and sweet in taste, slightly warm in nature, and mildly toxic. Modern studies characterize it as bitter, pungent, and salty in taste, with a slightly warm nature. Its therapeutic effects remain consistent across eras, including descending Qi, resolving phlegm, promoting diuresis, and stopping vomiting. Based on the research results, it is recommended that when developing famous classical formulas containing Inulae Flos, either I. japonica or I. britannica should be used as the medicinal source. Processing methods should follow formula requirements, where no processing instructions are specified, the raw products may be used after cleaning.
6.Herbal Textual Research on Inulae Flos in Famous Classical Formulas
Caixia LIU ; Yue HAN ; Yanzhu MA ; Lei GAO ; Sheng WANG ; Yan YANG ; Wenchuan LUO ; Ling JIN ; Jing SHAO ; Zhijia CUI ; Zhilai ZHAN
Chinese Journal of Experimental Traditional Medical Formulae 2026;32(3):210-221
In this paper, by referring to ancient and modern literature, the textual research of Inulae Flos has been conducted to clarify the name, origin, production area, quality evaluation, harvesting, processing and others, so as to provide reference and basis for the development and utilization of famous classical formulas containing this herb. After textual research, it could be verified that the medicinal use of Inulae Flos was first recorded in Shennong Bencaojing of the Han dynasty. In successive dynasties, Xuanfuhua has been taken as the official name, and it also has other alternative names such as Jinfeicao, Daogeng and Jinqianhua. The period before the Song and Yuan dynasties, the main origin of Inulae Flos was the Asteraceae plant Inula japonica, and from the Ming and Qing dynasties to the present, I. japonica and I. britannica are the primary source. In addition to the dominant basal species, there are also regional species such as I. linariifolia, I. helianthus-aquatili, and I. hupehensis. The earliest recorded production areas in ancient times were Henan, Hubei and other places, and the literature records that it has been distributed throughout the country since modern times. The medicinal part is its flower, the harvesting and processing method recorded in the past dynasties is mainly harvested in the fifth and ninth lunar months, and dried in the sun, and the modern harvesting is mostly harvested in summer and autumn when the flowers bloom, in order to remove impurities, dry in the shade or dry in the sun. In addition, the roots, whole herbs and aerial parts are used as medicinal materials. In ancient times, there were no records about the quality of Inulae Flos, and in modern times, it is generally believed that the quality of complete flower structure, small receptacles, large blooms, yellow petals, long filaments, many fluffs, no fragments, and no branches is better. Ancient processing methods primarily involved cleaning, steaming, and sun-drying, supplemented by techniques such as boiling, roasting, burning, simmering, stir-frying, and honey-processing. Modern processing focuses mainly on cleaning the stems and leaves before use. Regarding the medicinal properties, ancient texts describe it as salty and sweet in taste, slightly warm in nature, and mildly toxic. Modern studies characterize it as bitter, pungent, and salty in taste, with a slightly warm nature. Its therapeutic effects remain consistent across eras, including descending Qi, resolving phlegm, promoting diuresis, and stopping vomiting. Based on the research results, it is recommended that when developing famous classical formulas containing Inulae Flos, either I. japonica or I. britannica should be used as the medicinal source. Processing methods should follow formula requirements, where no processing instructions are specified, the raw products may be used after cleaning.
7.Wall-broken ganoderma lucidum spore powder protects against DEN/CCl4-induced hepatocellular carcinoma in mice by modulating the Nrf2 pathway
Hai JIN ; Shuo WAN ; Qian LIU ; Siyu ZHAO ; Jing LI ; Jiaxing ZHU ; Jiaxing AN ; Feng JIN
Acta Universitatis Medicinalis Anhui 2026;61(7):1176-1182
ObjectiveTo investigate the therapeutic effect of wall-broken ganoderma lucidum spore powder (GLS) on a mouse model of hepatocellular carcinoma and to explore its underlying mechanism. MethodsFifty 2-week-old C57BL/6J mice were divided into a control group, a model group and low, medium and high-dose GLS groups(0.25, 0.5, 1.0 g/kg ), with 10 mice in each group. A DEN/CCL4 mouse hepatocellular carcinoma model was established, and GLS was administered by gavage starting from the 8th week, once a day for 14 weeks. At the end of the final administration, the number of liver nodules was observed , and the serum alpha-fetoprotein (AFP) was detected using enzyme-linked immunosorbent assay. The content of catalase (CAT) in serum and liver tissue was determined by ammonium molybdate method. The content of reduced glutathione (GSH) in serum and liver tissue was measured by microplate assay. The content of glutathione peroxidase (GSH-PX) in serum and liver tissue was detected by colorimetric method. Immunohistochemistry (IHC-P) was employed to detect the protein expression of proliferating cell nuclear antigen (PCNA). R eal-time fluorescence quantitative polymerase chain reaction was utilized to detect the relative expression of nuclear factor E2-related factor 2 (Nrf2), Kelch-like epichlorohydrin-associated protein 1 (Keap1), NAD(P)H quinone oxidoreductase 1 (Nqo1), heme oxygenase-1 (Ho-1) and glutamate cysteine ligase catalytic subunit (Gclc) mRNA. ResultsCompared with the control group, the content of serum AFP were significantly higher in the model group (P<0.01), while the contents of CAT, GSH and GSH-PX in liver and serum were significantly lower in the model group (P<0.01). The relative expression of Keap1 and Nqo1 mRNA was significantly higher (P<0.01), the relative expression of Nrf2, Ho-1, Gclc mRNA relative expression was significantly lower (P<0.01), and the positive expression rate of PCNA was significantly higher (P<0.01). Compared with the model group, the total number of liver nodules and content of serum AFP in the administered group were significantly lower (P<0.01), and the contents of CAT, GSH, and GSH-PX in the liver and serum of mice significantly increased (P<0.05 or P<0.01). The expression of Keap1 and Nqo1 mRNA significantly decreased (P<0.05). The expression of Nrf2, Ho-1 and Gclc mRNA significantly increased (P<0.05), and positive expression rate of PCNA significantly decreased (P<0.01). ConclusionGLS has a significant protective effect on DEN/CCL4 -induced mouse hepatocellular carcinoma model, and the mechanism of action is related to the regulation of oxidative stress in mouse liver.
8.Exploration of Traditional Chinese and Western Medicine in Prevention and Treatment of DKD Based on Mitochondrial Autophagy Mediated by PINK1/Parkin Signaling Pathway: A Review
Runsheng LIU ; Xiaodong ZHANG ; Zhaoqing LI ; Jing WANG ; Jinglu ZHANG ; Lixia JIN
Chinese Journal of Experimental Traditional Medical Formulae 2025;31(7):302-313
Diabetic kidney disease (DKD) is one of the more common chronic kidney diseases,and its causes are complex. DKD is very easy to progress to end-stage renal disease,and the current therapeutic effect still needs to be improved. As an important excretive organ of the human body, the kidney has physiological functions such as discharging metabolic waste, regulating fluid balance, and maintaining the stability of the body's internal environment. These highly complex biochemical processes all depend on the energy support provided by mitochondria. Mitochondrial dysfunction is a key factor causing kidney injury, and the imbalance of mitochondrial homeostasis is an important link leading to mitochondrial dysfunction. The occurrence and development of DKD are often accompanied by the imbalance of mitochondrial homeostasis in renal cells. Mitochondrial autophagy, as a means of regulating mitochondrial homeostasis, is very important for the prevention and treatment of DKD. The PTEN-induced putative kinase 1 (PINK1)/Parkin pathway is one of the most classical pathways to regulate mitochondrial autophagy. Recent studies have found that some drugs can regulate the PINK1/Parkin signaling pathway to target mitochondrial homeostasis and exert renoprotective effects. In particular, traditional Chinese medicine has a significant effect on early and middle stage DKD by regulating PINK1/Parkin pathway-mediated mitochondrial autophagy. This article discussed the mechanism of PINK1/Parkin pathway in mitochondrial autophagy and DKD and reviewed the effect of PINK1/Parkin pathway-mediated mitochondrial autophagy on DKD. At the same time, it explored the therapeutic effect of traditional Chinese and western medicine on DKD mediated by PINK1/Parkin-mediated mitochondrial autophagy, aiming to broaden the ideas of traditional Chinese and western medicine for the prevention and treatment of DKD from the perspective of PINK1/Parkin regulating mitochondrial autophagy.
9.Exploration of Traditional Chinese and Western Medicine in Prevention and Treatment of DKD Based on Mitochondrial Autophagy Mediated by PINK1/Parkin Signaling Pathway: A Review
Runsheng LIU ; Xiaodong ZHANG ; Zhaoqing LI ; Jing WANG ; Jinglu ZHANG ; Lixia JIN
Chinese Journal of Experimental Traditional Medical Formulae 2025;31(7):302-313
Diabetic kidney disease (DKD) is one of the more common chronic kidney diseases,and its causes are complex. DKD is very easy to progress to end-stage renal disease,and the current therapeutic effect still needs to be improved. As an important excretive organ of the human body, the kidney has physiological functions such as discharging metabolic waste, regulating fluid balance, and maintaining the stability of the body's internal environment. These highly complex biochemical processes all depend on the energy support provided by mitochondria. Mitochondrial dysfunction is a key factor causing kidney injury, and the imbalance of mitochondrial homeostasis is an important link leading to mitochondrial dysfunction. The occurrence and development of DKD are often accompanied by the imbalance of mitochondrial homeostasis in renal cells. Mitochondrial autophagy, as a means of regulating mitochondrial homeostasis, is very important for the prevention and treatment of DKD. The PTEN-induced putative kinase 1 (PINK1)/Parkin pathway is one of the most classical pathways to regulate mitochondrial autophagy. Recent studies have found that some drugs can regulate the PINK1/Parkin signaling pathway to target mitochondrial homeostasis and exert renoprotective effects. In particular, traditional Chinese medicine has a significant effect on early and middle stage DKD by regulating PINK1/Parkin pathway-mediated mitochondrial autophagy. This article discussed the mechanism of PINK1/Parkin pathway in mitochondrial autophagy and DKD and reviewed the effect of PINK1/Parkin pathway-mediated mitochondrial autophagy on DKD. At the same time, it explored the therapeutic effect of traditional Chinese and western medicine on DKD mediated by PINK1/Parkin-mediated mitochondrial autophagy, aiming to broaden the ideas of traditional Chinese and western medicine for the prevention and treatment of DKD from the perspective of PINK1/Parkin regulating mitochondrial autophagy.
10.Effect of erioside E on myocardial injury after myocardial infarction by regulating pyroptosis through HIF-2α
Li YU ; Jing JIN ; Zhijing LIU ; Manyu ZENG ; Youyou CHEN ; Qi XU
Journal of Xi'an Jiaotong University(Medical Sciences) 2025;46(4):599-605
Objective To investigate the effect of erioside E on myocardial injury after myocardial infarction(MI)by regulating hypoxia-inducible factor-2α(HIF-2α)-mediated pyroptosis of cardiomyocytes and its possible mechanism.Methods Thirty rats were randomly divided into sham-operation group,MI group,and erioside E group,with 10 rats in each group.Cardiomyocytes H9C2 were cultured and divided into control group,hypoxia group,erioside E group and HIF-2α overexpression group.Masson staining was used to observe myocardial fibrosis and infarct size.The protein expressions of HIF-2α,NLRP3,Caspase-1 and GSDMD were detected by Western blotting.RT-qPCR was used to detect the mRNA expressions of HIF-2α,NLRP3,Caspase-1 and GSDMD.EdU kit was used to detect the proliferation of cardiomyocytes.The expressions of NLRP3,Caspase-1 and GSDMD proteins in cardiomyocytes were detected by immunofluorescence staining.Results Compared with the sham-operation group,the myocardial fibrosis level and infarct size were significantly increased in the MI group(P<0.05),and the protein expressions of HIF-2α,pyroptosis-related proteins of NLRP3,Caspase-1 and GSDMD in myocardial tissue were increased(P<0.05).Compared with the MI group,the level of myocardial fibrosis and infarct size were significantly decreased(P<0.05),the protein expression of HIF-2α in myocardial tissue was increased,and the protein expressions of NLRP3,Caspase-1 and GSDMD were decreased in the erioside E group(P<0.05).Compared with the control group,the hypoxia group had a significant reduction in the proliferation of cardiomyocytes and significant increase in the mRNA and protein expressions of HIF-2α,NLRP3,Caspase-1 and GSDMD(P<0.05).Compared with the hypoxia group,the proliferation ability of cardiomyocytes was significantly increased,and the expressions of HIF-2α,NLRP3,Caspase-1 and GSDMD mRNA in cardiomyocytes were decreased in the erioside E group(P<0.05),while the protein expression of HIF-2α in cardiomyocytes was increased in the HIF-2αoverexpression group.The protein expressions of NLRP3,Caspase-1 and GSDMD were significantly decreased(P<0.05).Conclusion Erioside E can improve myocardial injury by upregulating HIF-2α to inhibit myocardial pyroptosis.

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