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.

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）. Conlusion： The digital muscle function assessment system Myoton PRO can objectively evaluate the muscle tension level of patients with WD accompanied by abnormal muscle tension. This level has significant differences among different TCM syndromes and can evaluate the patient's condition to some extent.

ObjectiveTo observe the clinical efficacy of Gandouling decoction combined with neuromuscular electrical stimulation （NMES） in the treatment of dysphagia in Wilson disease （WD） with combined phlegm and stasis. MethodsA total of 80 WD patients with dysphagia due to combined phlegm and stasis treated in the Department of Encephalopathy， the First Affiliated Hospital of Anhui University of Chinese Medicine were randomized into a control group and an observation group， with 40 patients in each group. In addition， 40 healthy volunteers were recruited as the normal group. The control group was treated with basic copper drainage combined with NMES. The observation group was treated with Gandouling Decoction on the basis of the therapy in the control group. Each course of treatment lasted for 8 days， and the patients were treated for a total of 4 courses. All subjects underwent video fluoroscopic swallowing study （VFSS） before and after treatment. During the examination， contrast agents with 4 different characters were used for the swallowing action， and the passing time was recorded. The TCM syndrome score， water swallow test score， standard swallowing assessment （SSA） score， and 24-h urinary copper level before and after treatment were analyzed. ResultsWhen performing VFSS， the passing time of contrast agents of different characters in the oral stage was longer in the WD group than in the normal group （P<0.01）， while it had no significant difference in the pharyngeal stage. After treatment， the passing time in the oral stage shortened in the control and observation groups （P<0.01）， and the observation group outperformed the control group （P<0.01）. After treatment， both the control and observation groups showed declines in TCM syndrome score and SSA score （P<0.01） and an increase in water swallow test score （P<0.01）， and the changes were more obvious in the observation group than in the control group （P<0.01）. In addition， the treatment in the control and observation groups elevated the 24-h urinary copper level （P<0.01）， and the elevation in the observation group was more obvious than that in the control group （P<0.01）. Neither group showed obvious adverse reaction. ConclusionGandouling decoction combined with NMES can significantly ameliorate dysphagia in WD patients with the syndrome of combined phlegm and stasis regarding the TCM syndrome score， water swallow test score， and SSA score， demonstrating definite clinical efficacy and high safety.

ObjectiveTo investigate the correlation with cognitive function based on a new Kayser-Fleischer ring （K-F ring） grading method in Wilson’s disease （WD）. MethodsA total of 136 WD patients who were hospitalized in Encephalopathy Center， The First Affiliated Hospital of Anhui University of Chinese Medicine， from April 2022 to October 2023 were enrolled. All subjects underwent slit lamp examination， and the grade of K-F ring was determined according to the shape and extent of copper deposition in the cornea， whether it formed a ring or not， and whether there was a sunflower-like cloudy change in the lens. The patients were instructed to complete UWDRS， MoCA， and MMSE scale assessments， and these indicators were compared between patients with different K-F ring grades. An analysis of variance was used for comparison of normally distributed continuous data between multiple groups， and the least significant difference t-test （homogeneity of variance） or the Dunnett’s T3 test （heterogeneity of variance） was used for further multiple comparisons； the Kruskal-Wallis H test was used for comparison of non-normally distributed continuous data between multiple groups； the chi-square test was used for comparison of categorical data between groups. The Spearman correlation analysis was used to investigate the correlation of K-F ring grade with UWDRS， MoCA， and MMSE scores. ResultsAmong the 136 patients with WD， there were 40 patients with grade 4 K-F ring， accounting for the highest proportion of 29.4%， and 14 patients with grade 0 K-F ring， accounting for the lowest proportion of 10.3%， and there were 22 patients with grade 1 K-F ring （16.2%）， 19 with grade 2 K-F ring （14%）， 25 with grade 3 K-F ring （18.4%）， and 16 with grade 5 K-F ring （11.7%）. According to the different grades of K-F ring， there was a significant increase in UWDRS score （F=22.61， P<0.001） and significant reductions in MoCA and MMSE scores （F=16.40 and 13.80， both P<0.001）. The Spearman correlation analysis showed that K-F ring grade was positively correlated with UWDRS score （r=0.67， P<0.01） and was negatively correlated with MoCA and MMSE scores in WD patients （r=-0.59 and -0.57， both P<0.01）. ConclusionThe new K-F ring grading method can determine disease severity in WD patients to a certain degree and partially reflect cognitive function and activities of daily living in such patients.

Mild cognitive impairment due to Alzheimer′s disease is an inevitable pathological stage in the early development of Alzheimer′s disease, which can be classified as "microlumps in the brain collaterals" in traditional Chinese medicine. Based on the theory of latent toxin blocking collaterals, this article discusses the etiology and pathogenesis, clinical sequelae, and traditional Chinese medicine intervention strategies for mild cognitive impairment due to Alzheimer′s disease. The onset of mild cognitive impairment due to Alzheimer′s disease is very similar to the latent pathogen theory, which states that "the latent pathogen is latent and then develops, the poison is deep and difficult to cure, and the development can be recognized but the latent pathogen cannot be detected." Combining clinical experience, our team believes that the basic nature of the disease is a slight deficiency and a slight excess of symptoms. A slight deficiency of the five zang viscera and six fu viscera as root and a latent toxin colling collaterals of qi, fire, phlegm, and blood stasis as manifestaion. These usually start from the qi depression and develop into phlegm coagulation and blood stasis, then end up in latent toxin and gradually become the healthy qi deficiency. Therefore, the deficiency of vital qi and incubation of evil, latent toxin blocking collaterals the pathogenesis of early intervention of this disease should be carried out, upholding the idea that "the upper workman treats the disease before it is diagnosed." The principle of strengthening vital qi to eliminate pathogenic factors, slowing down and promoting pathogenic factors elimination, establishing the method of supporting correctness and wisdom, simultaneously detoxifying and clearing the blood stasis, pattern differentiation as the main and the disease differentiation as the first, combining the disease and pattern, and adjusting the macroscopic and microscopic, focusing simultaneously on eliminating and replenishing, dispel phlegm and remove blood stasis, achieve a strong vital qi and the elimination of evil, and enhance intelligence, delay or even block the progression of mild cognitive impairment due to Alzheimer′s disease, improve patients′ quality of life, and provide a theoretical basis for the early clinical prevention and treatment of Alzheimer′s disease.

Objective To study the impact of a Western diet-type feed on biological indicators and histopathology in APOE-/-mice.Methods Forty-eight female and 48 male APOE--mice,and 48 female and 48 male C57BL/6J mice were divided into eight breeding groups:APOE-/-breeding feed group,APOE-/-Western dietary feed group,C57BL/6J breeding feed group,and C57BL/6J Western dietary feed group(24 male and 24 female mice per group).Mice were fed the respective diets from 3 weeks until the end of the experiment at 20 weeks.After the experiment,serum was collected for measurement of biochemical indicators.Aortas were removed for oil red O staining and gross examination and the aorta root was paraffin sectioned and stained with hematoxylin and eosin.Results A Western diet did not significantly increase body weight in APOE-/-mice,but did significantly improve the blood lipid index and total cholesterol,low-density lipoprotein,and high-density lipoprotein levels,and promoted the formation of atherosclerotic plaques.Male mice were suitable for modeling gross aortic plaques while female mice were suitable for modeling aortic arch root plaques.Conclusions A Western diet can promote atherosclerosis in APOE-/-mice,increase the aortic plaque area ratio,shorten modeling time,and improve modeling uniformity.

Objective:To investigate the prognosis of newly diagnosed multiple myeloma (MM) patients with t (14;16) abnormality.Methods:Clinical data from 564 patients diagnosed with initial MM from January 2018 to November 2020 at Beijing Chaoyang Hospital affiliated with Capital Medical University and the First Affiliated Hospital of Sun Yat-sen University were collected and retrospectively analyzed. The prognoses of patients with t (14;16) were analyzed and compared with the prognoses of patients with normal FISH, and those with t (4;14) and del (17p) .Results:Among 564 newly diagnosed MM patients, 19 (3.4%) exhibited t (14;16) abnormalities, with 14 cases diagnosed with 1q21+ and three cases with del (17p). Progression-free survival (PFS) and overall survival (OS) of patients with t (14;16) were significantly shorter compared with patients with normal FISH (the median PFS: 14 months vs not reached, P<0.001; the median OS: 42 months vs not reached, P=0.002). No statistically significant difference was detected in PFS and OS between the 15 patients with t (14;16) and the 15 with t (4;14) after propensity score matching (the median PFS: 13.0 months vs not reached, P=0.247; the median OS: 42 months vs not reached, P=0.609). Similarly, no statistically significant difference was observed in PFS and OS between 15 patients with t (14;16) and 15 with del (17p) (the median PFS: 13 months vs 31 months, P=0.939; the median OS: 42 months vs 37.3 months, P=0.557). Propensity score matching indicated that when combined with 1q21+, no statistically significant differences were present in PFS and OS between patients with t (14;16) and patients with t (4;14) or patients with del (17p) (all P>0.05). Whether or not the patients with t (14;16) had undergone auto-HSCT did not significantly impact the PFS and OS (all P>0.05) . Conclusion:t (14;16) is often associated with high-risk cytogenetic abnormalities in newly diagnosed MM patients, and its adverse prognostic value is similar to that of t (4;14) and del (17p) .

Objective:To investigate the necessity of internal fixation of the fibular fracture in the treatment of degree Ⅱ ankle supination-external rotation injury by the Dias-Tachdjian classification in children.Methods:A retrospective study was conducted to analyze the data of 69 children with ankle fracture (degree Ⅱ ankle supination-external rotation injury by the Dias-Tachdjian classification) who had been treated at Department of Orthopedic Trauma, The People's Hospital of Weifang and Department of Pediatric Orthopedics, Tianjin Hospital from January 2015 to October 2021. There were 41 males and 28 females with an age of (10.1±1.4) years, and 37 left and 32 right sides affected. The patients were divided into 2 groups according to whether internal fixation of the fibular fracture was performed. Group A consisted of 21 cases with fibular internal fixation and group B of 48 cases without fibular internal fixation. The preoperative data, operation time and operation expenses were recorded and compared between the 2 groups. At the last follow-up, the anteroposterior and lateral radiographs of bilateral full length lower limbs and ankle joints were taken; the lateral distal tibial angle (LDTA) and the anterior distal tibial angle (ADTA) on the affected side, and the disparity between bilateral ankle tibiotalar angles were measured; ankle function was assessed according to the ankle-hindfoot score of American Association of Foot and Ankle Surgery (AOFAS); the occurrence of premature physeal closure (PPC) was recorded.Results:There was no significant difference in the preoperative general data between the 2 groups, indicating comparability ( P>0.05). All patients were followed up for (19.5±4.1) months. At the last follow-up, the LDTA on the affected side was 89.6° (87.9°, 90.5°) in group A and 88.6°±1.9° in group B; the ADTA on the affected side was 80.9° (79.0°, 81.4°) in group A and 80.0° (78.6°, 81.2°) in group B; the disparity between bilateral ankle tibiotalar angles was 1.1°±0.5° in group A and 1.2°±0.5° in group B; the AOFAS ankle-hindfoot score was (89.5±5.2) points in group A and 89.0 (87.0, 92.0) points in group B. There was no statistically significant difference between the 2 groups in the above items ( P>0.05). The incidence of PPC was, respectively, 14.3% (3/21) and 14.6% (7/48) in groups A and B, showing no statistically significant difference ( P>0.05). Conclusions:In the treatment of degree Ⅱ ankle supination-external rotation injury in children, internal fixation of the fibular fracture has no significant effect on the imaging angles or the function of the ankle joint. After anatomic reduction of the distal tibia, the fibular fracture can be treated without internal fixation to reduce operational trauma, shorten operation time and reduce operation expenses.

Objective:To investigate the efficacy of staged treatment of infectious femoral defects of Cierny-Mader type Ⅳ using bone transport combined with locking plating after En-Bloc resection debridement.Methods:A retrospective analysis was conducted of the 10 patients with distal femoral traumatic bone infection who had been treated at Department of Orthopedics, The People's Hospital of Weifang from January 2020 to January 2023. There were 8 males and 2 females with an age of (48.5±11.4) years. All cases were classified as Cierny-Mader type Ⅳ. At the first stage, En-Bloc resection debridement was performed in all cases to remove previous internal fixation devices and fill the defects with antibiotic bone cement. After infection control, the second stage involved removal of bone cement, re-fixation with internal devices, and external fixation support for bone transport. After the bone segments met, freshening of the bone ends, minor bone grafting, and screw locking of the transported bone segments were performed. Outcomes observed included bone defect length, frame carrying time and index, bone healing time, limb function, and complications.Results:After the first stage of debridement, a bone defect of (9.1±2.1) cm was created in 10 patients. All patients were followed up for (19.8±6.6) months. The duration for carrying external fixation frame was (107.2±25.1) days, and the frame index (11.8±0.5) d/cm. No recurrence was observed postoperatively. Bone union was achieved in 9 patients within 8 months, but in 1 patient only after secondary bone grafting due to poor healing at the meeting ends. All patients returned to their previous life or physical labor with no complications like pain or re-fracture. Three patients experienced varying degrees of knee joint stiffness, but were able to meet needs of daily life; one requested joint release surgery which resulted in satisfactory therapeutic efficacy.Conclusion:Staged treatment of infectious femoral defects of Cierny-Mader type Ⅳ using bone transport combined with locking plating after En-Bloc resection debridement is simple and effective.

ObjectiveTo evaluate the effectiveness of Gandou decoction （GDD） by analyzing theclinical efficacy of GDD combined with speech training on the treatment of dysarthria with endoretention of damp-heat in Wilson's disease （WD）， so as to provide more clinical data and theoretical support for the selection of appropriate treatment schemes for WD patients with dysarthria with endoretention of damp-heat. MethodA total of 60 eligible WD patients with dysarthria with endoretention of damp-heat were selected and divided into a control group and a treatment group according to the random grouping method， with 30 cases in each group. The control group was treated with speech training + sodium dimercaptopropanesulfonate （DMPS）， and the treatment group was combined with GDD on the basis of the control group， with eight days as a course of treatment for 32 days. The total clinical effectiveness rate （Goldstein clinical classification）， dysarthria grading assessment from China Rehabilitation Research Center， TCM syndrome scores， 24-hour urine copper content， and modified Frenchay dysarthria rating scale scores of the two groups were compared before and after treatment. ResultAfter treatment， the total effective rate of the observation group was 90.0% （27/30）， and that of the control group was 70.0% （21/30）. The total effective rate of the observation group was significantly higher than that of the control group （Z=-1.986，P<0.05）. After treatment， the modified Frenchay dysarthria score， dysarthria grading assessment from China Rehabilitation Research Center， and 24-h urine copper in the two groups were significantly increased （P<0.05， P<0.01）， and the TCM syndrome score was significantly decreased （P<0.01）. Compared with the control group after treatment， except for the respiratory and jaw score， the modified Frenchay dysarthria score of the observation group was significantly increased （P<0.05， P<0.01）. The dysarthria grading from China Rehabilitation Research Center and 24-h urine copper content were significantly increased （P<0.01）， and the observation group had better efficacy. During the study period， there were no serious adverse reactions such as fever， rash， oral and eyelid mucosal swelling， exfoliative dermatitis， vomiting， diarrhea， or allergic shock during copper excretion treatment of DPMS and oral administration of GDD. ConclusionGDD combined with speech training can improve the symptoms and efficacy of WD patients with dysarthria with endoretention of damp-heat and enhance the patients' living standard to a certain extent， which can be widely used in clinics.