1.Clinical Efficacy of Gandou Fumu Decoction in Treating Hepatolenticular Degeneration with Liver Fibrosis of Liver-kidney Deficiency and Phlegm-blood Stasis Syndrome
Pingping YANG ; Meixia WANG ; Changchang CAO ; Zhuang TAO ; Jiang DU ; Yun XU ; Wenming YANG
Chinese Journal of Experimental Traditional Medical Formulae 2026;32(10):182-191
ObjectiveThis paper aims to evaluate the intervention effect of Gandou Fumu Decoction (GDFMD) in treating hepatolenticular degeneration with liver fibrosis of liver-kidney deficiency and phlegm-blood stasis syndrome, thereby providing evidence-based medical evidence for the treatment of Wilson's disease (WD)-related liver fibrosis with traditional Chinese medicine through clinical efficacy analysis. MethodsA total of 70 patients with WD-related liver fibrosis of liver-kidney deficiency and phlegm-blood stasis syndrome meeting the inclusion criteria were enrolled from Anhui Provincial Hospital of TCM from October 1, 2023, to October 1, 2024. Participants were divided into a control group and an observation group, with 35 cases in each group. The control group received conventional copper chelation therapy with sodium dimercaptopropanesulfonate (DMPS). On this basis, the observation group was additionally administered GDFMD orally. Each treatment course lasted eight days, for a total of four treatment courses. Efficacy evaluations were performed before treatment and after the second and fourth treatment courses, respectively. The clinical efficacy and safety of GDFMD in the treatment of WD-related liver fibrosis were assessed by comparing the changes in liver stiffness measurement (LSM), liver serological markers [alanine aminotransferase (ALT), aspartate aminotransferase (AST), type Ⅳ collagen (C-Ⅳ), laminin (LN), N-terminal propeptide of type Ⅲ procollagen (PⅢNP), and hyaluronic acid (HA)], fibrosis index based on 4 factors (FIB-4), AST to platelet ratio index (APRI), unified Wilson's disease rating scale part Ⅱ (UWDRS-Ⅱ), traditional Chinese medicine (TCM) syndrome score, 24-hour urinary copper, and safety indicators between the two groups before and after treatment. ResultsCompared with those before treatment, LSM levels decreased in both groups after two and four treatment courses (P<0.05). Compared with those after treatment, there was no statistically significant difference in the improvement of LSM levels in the observation group after two treatment courses, and the improvement of LSM levels in the observation group was more obvious after four treatment courses (P<0.05). Compared with those before treatment, the levels of HA, LN, PⅢNP, and C-Ⅳ decreased in both groups after two and four treatment courses (P<0.05). Compared with those after treatment, there was no statistically significant difference in the improvement of the C-Ⅳ levels in the observation group after two treatment courses, and the levels of HA, LN, and PⅢNP were more obvious (P<0.05). After four treatment courses in the observation group, the levels of HA, LN, PⅢNP, and C-Ⅳ were improved more significantly (P<0.05). Compared with those before treatment, ALT and AST levels decreased in both groups after two and four treatment courses (P<0.05). Compared with the control group after treatment, there was no statistically significant difference in the improvement of ALT and AST levels in the observation group after two treatment courses, and the improvement of ALT and AST levels in the observation group was more obvious after four treatment courses (P<0.05). Compared with those before treatment, APRI score and FIB-4 index level decreased in both groups after two and four treatment courses (P<0.05). Compared with those in control group after treatment, there was no statistically significant difference in the improvement of APRI score and FIB-4 index level in the observation group after two treatment courses, and the APRI score in the observation group was more obvious after four treatment courses (P<0.05), with no statistically significant improvement in the FIB-4 index difference. Compared with those before treatment, the levels of TCM syndrome scores decreased in both groups after two and four treatment courses (P<0.05). Compared with that of the control group after treatment, there was no statistically significant difference in the improvement of the level of TCM syndrome scores in the observation group after two treatment courses, and the improvement of the level of TCM syndrome scores in the observation group was more obvious after four treatment courses (P<0.05). Compared with those before treatment, the UWDRS-Ⅱ scores in both groups after two treatment courses were not improved obviously, and the UWDRS-Ⅱ scores in both groups decreased after four treatment courses (P<0.05). Compared with those of the control group after treatment, there was no statistically significant difference in the improvement of the UWDRS-Ⅱ scores in the observation group after two treatment courses, and the improvement of the UWDRS-Ⅱ scores in the observation group after four treatment courses was more obvious (P<0.05). Compared with those before treatment, the 24-h urine copper levels were significantly higher in both groups after two and four treatment courses (P<0.05). Compared with those in the control group after treatment, the 24-h urine copper levels in the observation group were significantly higher after two and four treatment courses (P<0.01). After two treatment courses, the 24-h urine copper level in the observation group showed a gradual decreasing trend, although it was higher than that before treatment. After four treatment courses, the control group had an improvement rate of 91.43%, an effective rate of 34.29%, and an apparent rate of 2.86%. The observation group had an improvement rate of 94.29%, an effective rate of 71.43%, and an apparent rate of 8.57%. The efficacy of the observation group was better than that of the control group (P<0.05). Conclusion① The efficacy of GDFMD combined with DMPS therapy in patients with WD-related liver fibrosis of liver-kidney deficiency and phlegm-blood stasis syndrome is significantly better than that of single DMPS therapy, and the advantages of the combined therapy are more obvious with the prolongation of the treatment cycle. ② GDFMD combined with the DMPS therapy program in the long-term application exhibits no obvious adverse reactions with good safety, which is worthy of clinical popularization and application.
2.Clinical Efficacy of Gandou Fumu Decoction in Treating Hepatolenticular Degeneration with Liver Fibrosis of Liver-kidney Deficiency and Phlegm-blood Stasis Syndrome
Pingping YANG ; Meixia WANG ; Changchang CAO ; Zhuang TAO ; Jiang DU ; Yun XU ; Wenming YANG
Chinese Journal of Experimental Traditional Medical Formulae 2026;32(10):182-191
ObjectiveThis paper aims to evaluate the intervention effect of Gandou Fumu Decoction (GDFMD) in treating hepatolenticular degeneration with liver fibrosis of liver-kidney deficiency and phlegm-blood stasis syndrome, thereby providing evidence-based medical evidence for the treatment of Wilson's disease (WD)-related liver fibrosis with traditional Chinese medicine through clinical efficacy analysis. MethodsA total of 70 patients with WD-related liver fibrosis of liver-kidney deficiency and phlegm-blood stasis syndrome meeting the inclusion criteria were enrolled from Anhui Provincial Hospital of TCM from October 1, 2023, to October 1, 2024. Participants were divided into a control group and an observation group, with 35 cases in each group. The control group received conventional copper chelation therapy with sodium dimercaptopropanesulfonate (DMPS). On this basis, the observation group was additionally administered GDFMD orally. Each treatment course lasted eight days, for a total of four treatment courses. Efficacy evaluations were performed before treatment and after the second and fourth treatment courses, respectively. The clinical efficacy and safety of GDFMD in the treatment of WD-related liver fibrosis were assessed by comparing the changes in liver stiffness measurement (LSM), liver serological markers [alanine aminotransferase (ALT), aspartate aminotransferase (AST), type Ⅳ collagen (C-Ⅳ), laminin (LN), N-terminal propeptide of type Ⅲ procollagen (PⅢNP), and hyaluronic acid (HA)], fibrosis index based on 4 factors (FIB-4), AST to platelet ratio index (APRI), unified Wilson's disease rating scale part Ⅱ (UWDRS-Ⅱ), traditional Chinese medicine (TCM) syndrome score, 24-hour urinary copper, and safety indicators between the two groups before and after treatment. ResultsCompared with those before treatment, LSM levels decreased in both groups after two and four treatment courses (P<0.05). Compared with those after treatment, there was no statistically significant difference in the improvement of LSM levels in the observation group after two treatment courses, and the improvement of LSM levels in the observation group was more obvious after four treatment courses (P<0.05). Compared with those before treatment, the levels of HA, LN, PⅢNP, and C-Ⅳ decreased in both groups after two and four treatment courses (P<0.05). Compared with those after treatment, there was no statistically significant difference in the improvement of the C-Ⅳ levels in the observation group after two treatment courses, and the levels of HA, LN, and PⅢNP were more obvious (P<0.05). After four treatment courses in the observation group, the levels of HA, LN, PⅢNP, and C-Ⅳ were improved more significantly (P<0.05). Compared with those before treatment, ALT and AST levels decreased in both groups after two and four treatment courses (P<0.05). Compared with the control group after treatment, there was no statistically significant difference in the improvement of ALT and AST levels in the observation group after two treatment courses, and the improvement of ALT and AST levels in the observation group was more obvious after four treatment courses (P<0.05). Compared with those before treatment, APRI score and FIB-4 index level decreased in both groups after two and four treatment courses (P<0.05). Compared with those in control group after treatment, there was no statistically significant difference in the improvement of APRI score and FIB-4 index level in the observation group after two treatment courses, and the APRI score in the observation group was more obvious after four treatment courses (P<0.05), with no statistically significant improvement in the FIB-4 index difference. Compared with those before treatment, the levels of TCM syndrome scores decreased in both groups after two and four treatment courses (P<0.05). Compared with that of the control group after treatment, there was no statistically significant difference in the improvement of the level of TCM syndrome scores in the observation group after two treatment courses, and the improvement of the level of TCM syndrome scores in the observation group was more obvious after four treatment courses (P<0.05). Compared with those before treatment, the UWDRS-Ⅱ scores in both groups after two treatment courses were not improved obviously, and the UWDRS-Ⅱ scores in both groups decreased after four treatment courses (P<0.05). Compared with those of the control group after treatment, there was no statistically significant difference in the improvement of the UWDRS-Ⅱ scores in the observation group after two treatment courses, and the improvement of the UWDRS-Ⅱ scores in the observation group after four treatment courses was more obvious (P<0.05). Compared with those before treatment, the 24-h urine copper levels were significantly higher in both groups after two and four treatment courses (P<0.05). Compared with those in the control group after treatment, the 24-h urine copper levels in the observation group were significantly higher after two and four treatment courses (P<0.01). After two treatment courses, the 24-h urine copper level in the observation group showed a gradual decreasing trend, although it was higher than that before treatment. After four treatment courses, the control group had an improvement rate of 91.43%, an effective rate of 34.29%, and an apparent rate of 2.86%. The observation group had an improvement rate of 94.29%, an effective rate of 71.43%, and an apparent rate of 8.57%. The efficacy of the observation group was better than that of the control group (P<0.05). Conclusion① The efficacy of GDFMD combined with DMPS therapy in patients with WD-related liver fibrosis of liver-kidney deficiency and phlegm-blood stasis syndrome is significantly better than that of single DMPS therapy, and the advantages of the combined therapy are more obvious with the prolongation of the treatment cycle. ② GDFMD combined with the DMPS therapy program in the long-term application exhibits no obvious adverse reactions with good safety, which is worthy of clinical popularization and application.
3.Differential Analysis of Clinical Features and Outcomes Between Syndrome of Combined Phlegm and Stasis and Syndrome of Dampness-heat Internal Accumulation in Hepatic Wilson's Disease
Lulu TANG ; Fengying WANG ; Wenming YANG
Chinese Journal of Experimental Traditional Medical Formulae 2026;32(9):189-195
ObjectiveTo investigate the differences in clinical features and outcomes between patients with hepatic Wilson's disease (WD) presenting with the syndrome of combined phlegm and stasis and the syndrome of dampness-heat internal accumulation. MethodsA retrospective cohort study was conducted by consecutively recruiting patients with hepatic WD from the Encephalopathy Center of the First Affiliated Hospital of Anhui University of Chinese Medicine between January 2022 and August 2025. According to traditional Chinese medicine (TCM) syndrome differentiation, the patients were assigned into a combined phlegm and stasis group and a dampness-heat internal accumulation group. All the patients received standard treatment. Baseline data, laboratory indicators, complications, Model for End-Stage Liver Disease (MELD) score, Child-Turcotte-Pugh (CTP) score, and Chronic Liver Failure-Sequential Organ Failure Assessment (CLIF-SOFA) score were recorded. The clinical features and outcomes of the two groups of patients were compared by t-test, U-test and multivariate logistic regression. ResultsA total of 141 patients with hepatic WD were included. The combined phlegm and stasis group comprised 68 patients with an average age of (28.22±10.47) years, including 43 males and 25 females. The dampness-heat internal accumulation group comprised 73 patients with an average age of (30.22±8.79) years, including 44 males and 29 females. Univariate analysis showed no statistically significant differences in age or gender between the two groups. The combined phlegm and stasis group had lower platelet (PLT), alanine aminotransferase (ALT), aspartate aminotransferase (AST), creatine (CRE), total cholesterol (TC), and triglycerides (TG) levels (P<0.05 or P<0.01) and higher total bilirubin (TBIL) and prothrombin time (PT) (P<0.05) than the dampness-heat internal accumulation group. There were no statistically significant differences in the incidence of hepatic encephalopathy, infection, spontaneous bacterial peritonitis, ascites, or gastrointestinal bleeding between the two groups. The incidence of splenomegaly and the MELD score were higher in the combined phlegm and stasis group (P<0.05). The CTP and CLIF-SOFA scores were also higher in the combined phlegm and stasis group, while these differences were not statistically significant. Eleven patients in the combined phlegm and stasis group and 9 patients in the dampness-heat internal accumulation group developed liver failure. Multivariate logistic regression analysis showed that PT (OR=1.794, 95%CI 1.249-2.576), TBIL (OR=1.111, 95%CI 1.026-1.203), ALT (OR=1.053, 95%CI 1.004-1.105), and TCM syndrome (OR=5.420, 95%CI 1.384-21.227) were independent risk factors for the development of liver failure in hepatic WD. ConclusionCompared with the hepatic WD patients with the syndrome of dampness-heat internal accumulation, those with the syndrome of combined phlegm and stasis exhibit severe liver function impairment and disease conditions. Furthermore, TCM syndrome serves as an independent predictive factor for the occurrence of liver failure in patients with hepatic WD.
4.Traditional Chinese medicine syndrome and syndrome differentiation-based treatment of Wilson disease
Wenjie HAO ; Wenming YANG ; Ting CHENG ; Hailin JIANG ; Han WANG ; Meixia WANG
Journal of Clinical Hepatology 2026;42(3):522-528
Wilson disease (WD) is an autosomal recessive disorder of copper metabolism, and decoppering therapy and symptomatic treatment are the main Western medicine therapies for WD. This article systematically reviews the understanding of the etiology and pathogenesis of WD in traditional Chinese medicine (TCM) and points out that abnormal natural endowment is the core etiology and pathogenesis of WD, with internal accumulation of copper toxicity as the manifestation, liver/spleen/kidney dysfunction as the root cause, and intermingled “toxin, stasis, phlegm, and deficiency” as the key pathogenesis. Literature research and clinical observation are conducted to summarize the common TCM syndromes of WD, including stagnation of liver Qi, internal retention of damp-heat, phlegm-stasis-heat accumulation syndrome, liver-kidney Yin deficiency syndrome, spleen-kidney Yang deficiency, and syndrome of deficiency damage and phlegm stasis. This article proposes the corresponding therapies and representative prescriptions for each syndrome and discusses the advantages of treatment by stage and integrated traditional Chinese and Western medicine therapy. This article aims to provide a systematic reference for the syndrome differentiation-based treatment of WD in clinical practice of TCM, thereby giving full play to the advantages of TCM in the treatment of this disease.
5.Gandouling Regulates PI3K/Akt/mTOR Autophagy Signaling Pathway via LncRNA H19 for Treatment of Wilson Disease Liver Fibrosis
Xin YIN ; Han WANG ; Daiping HUA ; Lanting SUN ; Yunyun XU ; Wenming YANG
Chinese Journal of Experimental Traditional Medical Formulae 2025;31(1):131-138
ObjectiveTo investigate the potential mechanisms and pathways through which Gandouling (GDL) exerts its effects in the treatment of liver fibrosis in Wilson disease. MethodsSixty male SD rats were randomly divided into six groups: the normal group, the model group, the GDL low-, medium-, and high-dose groups (0.24, 0.48, 0.96 g·kg-1), and the penicillamine group (90 mg·kg-1), with 10 rats in each group. A copper-loaded Wilson disease rat model was established by gavage administration of 300 mg·kg-1 copper sulfate pentahydrate to all groups except the normal group. Hematoxylin-eosin (HE) staining and Masson staining were used to observe the pathomorphological changes in the liver. Enzyme-linked immunosorbent assay (ELISA) was employed to measure the levels of hyaluronic acid (HA), laminin (LN), procollagen type-Ⅲ peptide (PC-Ⅲ), and collagen type-Ⅳ (C-Ⅳ). Transmission electron microscopy was used to examine the ultrastructure of liver tissues. Real-time quantitative polymerase chain reaction (Real-time PCR) was used to detect the expression levels of liver tissues and serum exosomal long noncoding RNA H19 (LncRNA H19), phosphatidylinositol 3-kinase (PI3K), protein kinase B (Akt), and mammalian target of rapamycin (mTOR). Western blot analysis was performed to assess the expression levels of PI3K, Akt, mTOR, and their phosphorylated forms, as well as autophagy-related proteins Beclin1 and microtubule-associated protein 1 light chain 3B (LC3-Ⅱ/LC3-Ⅰ) in liver tissues. Beclin1 and LC3-Ⅱ fluorescence signal intensity was observed by immunofluorescence. ResultsCompared with the normal group, the model group exhibited inflammatory cell infiltration in hepatocytes, unclear nuclear boundaries with cell cleavage and necrosis, and collagen fiber deposition around confluent areas. The levels of HA, LN, PC-Ⅲ, and C-Ⅳ were significantly elevated (P<0.01). Transmission electron microscopy revealed an increased number of autophagic vesicles, with autophagic lysosomes exhibiting a single-layer membrane structure following degradation of most envelopes. Expression levels of Beclin1 and LC3-Ⅱ/LC3-Ⅰ were significantly increased (P<0.01), and fluorescence signals of Beclin1 and LC3-Ⅱ were markedly enhanced. The protein expression levels of PI3K, Akt, mTOR, p-PI3K, p-Akt, and p-mTOR were reduced (P<0.01), while LncRNA H19 expression was increased (P<0.01), and mRNA expression levels of PI3K, Akt, and mTOR were decreased (P<0.01). After treatment with GDL, the degree of liver fibrosis was significantly improved, with decreased levels of HA, LN, PC-Ⅲ, and C-Ⅳ. The number of autophagic vesicles was significantly reduced, and expression levels of Beclin1 and LC3-Ⅱ/LC3-Ⅰ proteins were lower (P<0.01). The fluorescence signals of Beclin1 and LC3-Ⅱ weakened dose-dependently. The protein levels of PI3K, Akt, mTOR, p-PI3K, p-Akt, and p-mTOR were elevated (P<0.01), while the expression level of LncRNA H19 was reduced (P<0.01). Furthermore, the mRNA expression levels of PI3K, Akt, and mTOR increased (P<0.05, P<0.01). ConclusionGDL may alleviate liver fibrosis and reduce liver injury by regulating the PI3K/Akt/mTOR autophagy signaling pathway via LncRNA H19.
6.Gandou Fumu Decoction improves liver steatosis by inhibiting hepatocyte ferroptosis in mice with Wilson's disease through the GPX4/ACSL4/ALOX15 signaling pathway
Mengying ZHANG ; Chenling ZHAO ; Liwei TIAN ; Guofang YU ; Wenming YANG ; Ting DONG
Journal of Southern Medical University 2025;45(7):1471-1478
Objective To explore the mechanism of Gandou Fumu Decoction(GDFMD)for improving Wilson's disease(WD)in tx-J mice.Methods With 6 syngeneic wild-type mice as the control group,30 tx-J mice were randomized into WD model group,low-,medium-and high-dose GDFMD treatment groups,and Fer-1 treatment group.Saline(in control and model groups)and GDFMD(3.48,6.96 or 13.92 g/kg)were administered by gavage,and Fer-1 was injected intraperitoneally once daily for 14 days.Oil red and HE staining were used to observe lipid deposition and pathological conditions in the liver tissue;ALT,AST,albumin,AKP levels were determined to assess liver function of the mice.Western blotting and RT-qPCR were used to detect hepatic protein and mRNA expressions of GPX4,ACSL4,ALOX15,FTH1,FLT,TFR1,FAS,SCD1,and ACOX1,and Fe2+,MDA,ROS,SOD,GSH and 4-HNE levels were analyzed to assess oxidative stress.Results The mouse models of WD showed obvious fatty degeneration in the liver tissue significantly increased serum levels of ALT,AST and AKP,decreased albumin level,increased Fe2+,MDA,ROS,4-HNE levels,decreased SOD and GSH levels(P<0.05),lowered protein expressions of ACOX1,GPX4,FTH1,FLT,FAS,and SCD1,and increased protein contents of TFR1,ACSL4 and ALOX15 in the liver.Treatment with GDFMD and Fer-1 improved liver histopathology and liver function of the mouse models,decreased the levels of Fe2+,MDA and ROS,increased SOD and GSH levels,and reversed the changes in hepatic protein expressions.Conclusion GDFMD improves liver steatosis in mouse models of WD possibly by inhibiting hepatocyte ferroptosis through the GPX4/ACSL4/ALOX15 signaling pathway.
7.Bioinformatics Analysis and Validation of Cuproptosis-related Genes in Wilson Disease
Zhuang TAO ; Meixia WANG ; Shuai KANG ; Jipeng LIU ; Rui WANG ; Jiafeng ZHOU ; Wenming YANG
Chinese Journal of Experimental Traditional Medical Formulae 2025;31(15):124-131
ObjectiveTo explore the role of cuproptosis and identify cuproptosis-related genes in Wilson disease (WD) through bioinformatics analysis and clinical validation,providing implications and directions for the diagnosis and treatment of WD. Methods(1) Screening of target genes: The differentially expressed genes (DEGs) between WD and healthy control were obtained from GeneCards,and the cuproptosis-related genes were obtained from Gene Expression Omnibus (GEO) and published literature.The cuproptosis-related genes in WD were obtained by intersection.Through gene ontology (GO) and Kyoto Encyclopedia of Genes and Genomes (KEGG) enrichment analyses,the specific biological process,functions or metabolic pathways of cuproptosis-related genes in WD were predicted.Molecular docking and PyMOL visualization were then performed to analyze and verify the potential regulatory mechanism of Gandou Fumu Decoction for cuproptosis.(2)Validation of target genes: The blood samples of 15 WD patients treated in the department of encephalopathy and 15 healthy volunteers undergoing physical examinations in the health management center were randomly collected from the First Affiliated Hospital of Anhui University of Chinese Medicine.The expression levels of target genes were determined by Western blot and real-time PCR. Results(1) A total of 3 607 DEGs in WD were obtained from GSE107323 in GEO,and 68 cuproptosis-related genes were obtained from GeneCards and published literature.Twelve common target genes were obtained by intersection,including three up-related genes(SQSTM1,MIF1,and TAX1BP1) and nine down-regulated genes(CP,SERPINE1,AOC3,GPX4,SLC27A5,VEGF-A,PDHB,PDK1,and ATP7B).The common target genes were mainly enriched in monocarboxylic acid metabolism,oxidoreductase activity,negative regulation of molecular functions,which mainly involved HIF-1,ferroptosis and other signaling pathways.Molecular docking and PyMOL visualization results showed Gandou Fumu Decoction had good binding ability with the cuproptosis-related genes PDK1,SERPINE1,VEGFA,and AOC3 in WD.(2)A total of 30 blood samples were collected,including 15 WD patients and 15 health volunteers.Western blot results showed that expression levels of target genes were consistent with the results obtained by bioinformatics analysis.RT-qPCR results showed that compared with healthy volunteers,WD patients had down-regulated mRNA levels of SERPINE1,GPX4,SLC27A5,and VEGF-A and up-regulated mRNA levels of SQSTM1 and MIF1(P<0.05). ConclusionThe expression levels of cuproptosis-related genes in WD patients are consistent with the results predicted by bioinformatics analysis.The characteristic preparation Gandou Fumu Decoction of Xin'an Medicine showed good binding abilities with the cuproptosis-related genes in WD.Cuproptosis may play a key role in the pathophysiological mechanism of WD,which can provide a new target for the diagnosis and treatment of WD.
8.Treatment of Renal Injury in Wilson Disease Based on Pathogenesis of Latent Toxin in Kidney Collaterals
Ke DIAO ; Wenming YANG ; Xiang LI ; Yue YANG ; Yulong YANG ; Zhihong RAO ; Shuzhen FANG ; Yufeng DING
Chinese Journal of Experimental Traditional Medical Formulae 2025;31(15):132-139
Hepatolenticular degeneration, also known as Wilson disease (WD), is a hereditary disease caused by mutations in the ATP7B gene, leading to copper metabolism disorders. Gene mutations result in impaired synthesis of copper-binding protein, and abnormal excretion of copper through bile leads to pathological deposition of copper in various organs, ultimately causing multi-organ damage. The insidious onset and low specificity of symptoms make it difficult to diagnose this disease. On the basis of existing studies and the theory of latent toxin, this paper proposes that latent toxin in kidney collaterals is the main pathogenesis of renal injury in WD. It is pointed out that health Qi deficiency and latent pathogen are the premises for the occurrence of this disease, and the transformation of latent pathogen into toxin is the ley pathological process. Toxin damaging kidney collaterals is the ultimate result. According to the pathogenesis, this paper proposes the treatment principle of reinforcing healthy Qi and resolving toxin and treatment based on syndrome differentiation. This review provides new ideas for the diagnosis and treatment of renal injury in WD with traditional Chinese medicine.
9.Correlations Between Traditional Chinese Medicine Syndromes and Lipid Metabolism in 341 Children with Wilson Disease
Han WANG ; Wenming YANG ; Daiping HUA ; Lanting SUN ; Qiaoyu XUAN ; Wei DONG ; Xin YIN
Chinese Journal of Experimental Traditional Medical Formulae 2025;31(15):140-146
ObjectiveTo study the correlations between traditional Chinese medicine (TCM) syndromes and lipid metabolism in children with Wilson disease (WD). MethodsClinical data and lipid metabolism indicators [total cholesterol (TC), triglycerides (TG), low-density lipoprotein cholesterol (LDL-C), high-density lipoprotein cholesterol (HDL-C), apolipoprotein A1 (ApoA1), apolipoprotein B (ApoB), and lipoprotein a (Lpa)] were retrospectively collected from 341 children with WD. The clinical data were compared among WD children with different syndromes, and the correlations between TCM syndromes and lipid metabolism in children with WD were analyzed. Least absolute shrinkage and selection operator (LASSO) regression was used for variable screening, and unordered multinomial Logistic regression was employed to analyze the effects of lipid metabolism indicators on TCM syndromes. ResultsThe 341 children with WD included 121 (35.5%) children with the dampness-heat accumulation syndrome, 103 (30.2%) children with the liver-kidney Yin deficiency syndrome, 68 children with the combined phlegm and stasis syndrome, 29 children with the spleen-kidney Yang deficiency syndrome, and 20 children with the liver qi stagnation syndrome. The liver-kidney Yin deficiency syndrome, combined phlegm and stasis syndrome, and spleen-kidney Yang deficiency syndrome had correlations with the levels of lipid metabolism indicators (P<0.05). Lipid metabolism abnormalities occurred in 232 (68.0%) children, including hypertriglyceridemia (108), hypercholesterolemia (23), mixed hyperlipidemia (67), lipoprotein a-hyperlipoproteinemia (12), and hypo-HDL-cholesterolemia (22). The percentages of hypertriglyceridemia and hypo-HDL-cholesterolemia varied among children with different TCM syndromes (P<0.05). Correlations existed for the liver-kidney Yin deficiency syndrome with TG, TC, and HDL-C, the combined phlegm and stasis syndrome with TG, the spleen-kidney Yang deficiency syndrome with TG, TC, and LDL-C, and the liver Qi stagnation syndrome with TC and LDL-C (P<0.05, P<0.01). ConclusionThe TCM syndromes of children with WD are dominated by the dampness-heat accumulation syndrome and the liver-kidney Yin deficiency syndrome, and dyslipidemia in the children with WD is dominated by hypertriglyceridemia and mixed hyperlipidemia. There are different correlations between TCM syndromes and lipid metabolism indicators, among which TG, TC, LDL-C, and HDL-C could assist in identifying TCM syndromes in children with WD.
10.Correlation between Muscle Tension,Clinical Characteristics, and Traditional Chinese Medicine Syndromes in Patients with Wilson Disease Based on Digital Muscle Function Assessment System Myoton PRO
Yulong YANG ; Wenming YANG ; Han WANG ; Xiang LI ; Taohua WEI ; Wenjie HAO ; Yue YANG ; Yufeng DING ; Yuqi SONG ; Wei HE
Chinese Journal of Experimental Traditional Medical Formulae 2025;31(15):147-154
ObjectiveThis paper aims to use the digital muscle function assessment system Myoton PRO to assess the correlation between muscle tension,clinical characteristics, and traditional Chinese medicine(TCM) syndromes in patients with hepatolenticular degeneration [also known as Wilson disease(WD)]. MethodsA total of 104 patients with WD accompanied by abnormal muscle tension(increased or decreased,hereinafter the same) who were hospitalized in the Brain Disease Centre of the First Affiliated Hospital of Anhui University of Chinese Medicine from April 2021 to November 2023 were selected,all of whom were subjected to TCM syndrome diagnosis and Myoton PRO for the measurement of F value of muscle tension,Goldstein, and UWDRS-N scales. The age of onset of the disease and disease duration were analyzed,and the differences and correlations of the above indexes in different TCM syndromes of WD were analyzed ResultsAmong the 104 patients with WD ,the phlegm and stasis syndrome was the most common(60 patients),followed by the damp-heat syndrome(33 patients),and the least common was the liver-kidney Yin deficiency syndrome(11 patients). The F value of the phlegm and stasis syndrome group was higher than that of the liver-kidney Yin deficiency syndrome group and the damp-heat syndrome group(P<0.01). The F value of the damp-heat syndrome group was higher than that of the liver-kidney Yin deficiency syndrome group(P<0.05),and the F value of the lower limbs of each group was higher than that of the upper limbs(P<0.01). Goldstein and UWDRS-N scores of the patients in the phlegm and stasis syndrome group were higher than those in the damp-heat syndrome group and the liver-kidney Yin deficiency syndrome group(P<0.05). There was no significant difference between the Goldstein and UWDRS-N scores of patients in the liver-kidney Yin deficiency syndrome group and the damp-heat syndrome group. Correlation analysis revealed that the age of onset and duration of the disease were positively correlated with the F values of the lower limbs(r=0.20,P<0.05,r=0.38,P<0.01)and had no significant correlation with those of the upper limbs. The F value levels of muscle tension of all limbs in the three groups of patients were positively correlated with the Goldstein and UWDRS-N scores(muscle tension of the upper limbs in the phlegm and stasis syndrome group,r=0.36,P<0.01,r=0.42,P<0.01. muscle tension of the lower limbs in the phlegm and stasis syndrome group,r=0.70,P<0.01,r=0.60,P<0.01. muscle tension of the upper limbs in the damp-heat syndrome group,r=0.64,P<0.01,r=0.53,P<0.01. muscle tension of the lower limbs in the damp-heat syndrome group,r=0.59,P<0.01,r=0.70,P<0.01. muscle tension of the upper limbs in the liver-kidney Yin deficiency syndrome group,r=0.70,P<0.01,r=0.74,P<0.01. muscle tension of the lower limbs in the liver-kidney Yin deficiency syndrome group,r=0.85,P<0.01,r=0.62,P<0.01).

Result Analysis
Print
Save
E-mail