Wilson's disease （WD） is a copper metabolism disorder caused by mutations in the ATP7B gene， with diverse phenotypes and complex pathogenesis. It is one of the few rare diseases that can achieve good clinical efficacy through standardized treatment. Since there are few systematic reviews of this disease， we summarize the pathogenesis and treatment methods of WD from traditional Chinese and western medicine by reviewing the literature related to WD. In western medicine， ATP7B gene mutation is considered as the root cause of WD， which affects copper transport and causes copper metabolism disorders. The excessive copper deposited in the body will result in oxidative stress， defects in mitochondrial function， and cell death. Western medicine treatment of WD relies mainly on drugs， and copper antagonists are the first choice in clinical practice， which are often combined with hepatoprotective and antioxidant therapy. Surgery is a common therapy for the patients with end-stage WD， and gene therapy provides an option for WD patients. According to the traditional Chinese medicine （TCM） theory， WD is rooted in constitutional deficiency and copper accumulation and triggered by dampness-heat accumulation or phlegm combined with stasis. The patient syndrome varies in different stages of the disease， and thus the treatment should be based on syndrome differentiation. The TCM treatment method of nourishing the liver and kidneys and warming the spleen and kidneys can address the root cause. The methods of clearing heat and drying dampness， resolving phlegm and dispelling stasis， and soothing liver and regulating qi movement can be adopted to treat symptoms. On the basis of syndrome differentiation， special prescriptions for the treatment of WD have been formulated， such as Gandou decoction， Gandouling， and Gandou Fumu decoction， which have been widely used in clinical practice. TCM and western medicine have their own advantages and shortcomings. The integrated Chinese and western medicine complementing with each other demonstrates great therapeutic potential. This paper summarizes the pathogenesis and treatment of WD with integrated Chinese and western medicine， aiming to provide a reference for the clinical diagnosis and treatment of this disease.

Objective:To investigate the clinical effects of adjustable external fixation traction combined with arthroscopic microfracture in the treatment of osteochondral lesions of the talus (OLT).Methods:A retrospective study was conducted to analyze the data of 27 OLT patients who had been treated at Department of Orthopedics, Beijing Rehabilitation Hospital from May 2017 to March 2022. There were 16 males and 11 females, aged (32.4±7.2) years. Lesion site: 23 medial and 4 lateral cases; Hepple staging: 7 cases at stage Ⅰ, 15 cases at stage Ⅱ, and 5 cases at stage Ⅲ; disease duration: (10.6±3.3) months. All the patients were treated by adjustable external fixation traction combined with arthroscopic microfracture. Recorded were the patients' visual analogue scale (VAS) pain scores and American Orthopedic Foot and Ankle Society (AOFAS) ankle-hindfoot scores at 6 months and 12 months after surgery, levels of interleukin-1 (IL-1), interleukin-6 (IL-6) and tumour necrosis factor- α (TNF- α) at 1 month after surgery, lesion area at 12 months after surgery, and incidence of complications. Results:The follow-up time for this cohort was (16.2±6.7) months. The AOFAS score was (61.52±6.75) points before surgery, (84.15±5.56) points at 6 months after surgery and (95.67±4.30) points at 12 months after surgery. The VAS score was (5.88±1.02) points before surgery, (2.12±0.48) points at 6 months after surgery and (0.66±0.36) points at 12 months after surgery. The two-by-two comparisons between the 3 time points for the above items were statistically significant ( P<0.05). IL-1 was (32.37±6.64) pg/mL, IL-6 (34.04±7.12) pg/mL, and TNF- α (17.89±4.96) ng/L at 1 month after surgery in the 27 patients, all of which were significantly lower than their preoperative levels [(96.63±14.80) pg/mL, (102.33±20.42) pg/mL, and (54.48±9.33) ng/L] ( P<0.05). The lesion area was (28.66±6.52) mm 2 at 12 months after surgery, significantly smaller than the value before surgery [(128.52±11.32) mm 2] ( P<0.05). Infection at the adjustable external fixation needle track occurred in 1 patient and lower limb thrombosis in 2 patients. Conclusion:In the treatment of OLT, adjustable external fixation and traction combined with arthroscopic microfracture can achieve satisfactory results and improve symptoms for the patients.

Objective:To explore the pathogenic genes, clinical characteristics and treatment follow-up of children with congenital long QT syndrome (LQTS).Methods:Clinical data of 20 cases diagnosed with congenital LQTS and underwent gene testing from April 15, 2011 to April 15, 2021 in Department of Pediatric Cardiology, Shandong Provincial Hospital Affiliated to Shandong University were retrospectively collected and analyzed using independent sample t-test and Fisher′ s exact probability method. Results:LQTS-related gene mutations were detected in all the 20 cases, and pathogenic or suspected pathogenic mutations were identified in 18 cases (90.0%). Five LQTS mutation genes were discovered, including KCNQ1, KCNH2, SCN5A, CACNA1C and AKAP9.Eighteen cases (90.0%) had positive symptoms, and 13 cases (65.0%) had definite inducements.The inducement of symptoms in children with LQTS type 1(LQT1) was related to exercise, the causes of syncope in LQT1 and Jervell-Lange-Nielsen syndrome type 1 (JLNS1) with complex heterozygous mutations were exercise or emotional agitation; the causes of syncope in LQTS type 2 (LQT2) were unrelated to exercise; severe exercise in LQTS type 3 (LQT3) resulted in symptoms; and seizure in LQTS type 8 (LQT8) was non-induced.The corrected QT(QTc) interval of 20 cases was (553.1±66.6) ms, with a range of 460-707 ms, among which 17 cases showed QTc≥480 ms.The electrocardiogram(ECG) manifestations of children with various types of LQTS were different.There was no significant difference in QTc between different genders, or between children with syncope and those without syncope (all P>0.05). The follow-up time was (3.4±2.3) years, ranging from 0 to 8.3 years.Seventeen children received treatment[beta blockers and implantable cardiovertor-defibrillator(ICD)] and 3 cases did not.By the end of the follow-up, 1 child died, 19 cases survived, and 2 cases of the surviving children lost consciousness. Conclusions:There is a high consistency between genetic diagnosis and clinical diagnosis of congenital LQTS.The positive rate of gene detection is 90.0%.The clinical manifestations and ECG characteristics vary with genotypes.Beta blockers are protective.ICD therapy can prevent sudden cardiac death when oral medication does not respond.

Objective : To investigate the willingness to receive HIV testing in primary health service institutions (PHSIs) among young students in Wuhan City, so as to provide the evidence for improving the detection of HIV testing among young students. Methods: Fifteen PHSIs were sampled using a stratified random sampling method in 14 districts of Wuhan City, and school students at ages of 15 to 24 years were sampled from each district using a convenience sampling method. Participants' demographics, awareness of AIDS-related knowledge, HIV testing and willingness to receive HIV testing were collected using questionnaires, and factors affecting the willingness to receive HIV testing in PHSIs were identified among school students using a multivariable logistic regression model. Results : A total of 301 questionnaires were allocated, and 299 valid questionnaires were recovered, with an effective recovery rate of 99.34%. The respondents included 143 men (47.83%) and 156 women (52.17%), and had a mean age of (19.36±2.40) years; there were 223 respondents with an educational level of diploma and above (74.58%). The awareness of AIDS-related knowledge was 71.57% among the respondents, and 144 respondents had received AIDS-related health education in PHSIs (48.16%). There were 34 respondents that had received HIV testing (11.37%) and 203 respondents that were willing to receive HIV testing in PHSIs (67.89%). The respondents that were unwilling to receive HIV testing in PHSIs were mainly attributed to considering to be unlikely to get HIV infections (82.29%). Multivariable logistic regression analysis showed that school students who knew AIDS-related knowledge (OR=2.797, 95%CI: 1.583-4.941), knew free HIV counseling and testing services in PHSIs (OR=2.070, 95%CI: 1.123-3.814), and had received AIDS-related health education in PHSIs (OR=2.814, 95%CI: 1.573-5.032) were more willing to receive HIV testing in PHSIs. Conclusions There were 67.89% of school students that were willing to receive HIV testing in PHSIs in Wuhan City, and the willingness to receive HIV testing was correlated with the awareness of risk of HIV infections, and awareness and experience of AIDS control services in PHSIs.

In recent years, foreign countries are gradually implementing broad consent to improve the utilization of medical data and biological samples, but broad consent may face ethical issues such as imperfect notification and affecting the rights of subjects. There are already relevant regulations and practices on broad consent in foreign countries. The concept of broad consent is not clearly defined in China′s laws. At present, the treatment of biological samples can be roughly divided into four categories in practice, and there is potential application space for broad consent. The specific scope of broad consent should be clarified, distinguished from donation behavior, and the implementation of broad consent should be explored on the basis of protecting the rights of subjects.

Objective:To study the application effect of modified butterfly needle fixation method for implantable venous access port in breast cancer patients, in order to povide reference for the clinical applicaiton.Methods:A total of 300 patients with breast cancer from Janurary to December 2019 in Sun Yat-Sen University Cancer Center were divided into two groups by random digits table method: experimental group and control group, 150 cases in each group. The experimental group was treated with modified implantable intravenous port butterfly needle fixation method, while the control group was treated with conventional fixation method. The Visual Analogue Scale of pain during needle extraction, the incidence of needle stick injury and the time of needle extraction were measured between the two groups.Results:The Visual Analogue Scale of pain, the time of needle pulling were (4.01±0.89) points, (2.71±0.52) min in the control group, and (1.84±0.84) points, (1.86±0.39) min in the experimental group, the differences were statistically significant ( t values were -21.70, -16.02, P<0.01). The incidence of needle stick injury was 4.00% (6/150) in the control group and no needlestick injury event occurred in the experimental group, the difference was statistically significant (Fisher exact probability, P<0.05). Conclusions:The modified method of dressing fixation can effectively reduce the pain during needle pulling, reduce the incidence of needle stick injury, and effectively shorten the time of needle pulling.

Objective:To investigate the etiology of necrotizing pneumonia (NP) in children and the clinical characteristics of NP caused by different pathogens in China.Methods:A retrospective, case-control study was performed in children with NP who were admitted to 13 hospitals in China from January 2008 to December 2019. The demographic and clinical information, laboratory data, etiological and radiological findings were analyzed. The data were divided into three groups based on the following years: 2008-2011, 2012-2015 and 2016-2019, and the distribution characteristics of the pathogens in different period were compared. Meanwhile, the pathogens of pediatric NP in the southern and northern China were compared. And the clinical characteristics of the Mycoplasma pneumoniae (MP) NP and the bacterial NP were also compared. T-test or Mann-Whitney nonparametric test was used for comparison of numerical variables, and χ 2 test was used for categorical variables. Results:A total of 494 children with NP were enrolled, the median ages were 4.7 (0.1-15.3) years, including 272 boys and 222 girls. Among these patients, pathogens were identified in 347 cases and the pathogen was unclear in the remaining 147 cases. The main pathogens were MP (238 cases), Streptococcus pneumoniae (SP) (61 cases), Staphylococcus aureus (SA) (51 cases), Pseudomonas aeruginosa (13 cases), Haemophilus influenzae (10 cases), adenovirus (10 cases), and influenza virus A (7 cases), respectively. MP was the most common pathogen in all three periods and the proportion increased yearly. The proportion of MP in 2016-2019 was significantly higher than that in 2012-2015 (52.1% (197/378) vs. 36.8% (32/87), χ 2= 6.654, P=0.010), while there was no significant difference in the proportion of MP in 2012-2015 and that in 2008-2011 (36.8% (32/87) vs. 31.0% (9/29), χ2=0.314, P=0.575).Regarding the regional distribution, 342 cases were in the southern China and 152 in the northern China. Also, MP was the most common pathogen in both regions, but the proportion of MP was higher and the proportion of SP was lower in the north than those in the south (60.5% (92/152) vs. 42.7% (146/342), χ 2=13.409, P<0.010; 7.9% (12/152) vs. 14.3% (49/342), χ 2= 4.023, P=0.045). Comparing the clinical characteristics of different pathogens, we found that fever and cough were the common symptoms in both single MP and single bacterial groups, but chest pain was more common (17.0% (34/200) vs. 6.1% (6/98), χ 2=6.697, P=0.010) while shortness of breath and wheezing were less common in MP group (16.0% (32/200) vs. 60.2% (59/98), χ 2=60.688, P<0.01; 4.5% (9/200) vs. 21.4% (21/98), χ 2=20.819, P<0.01, respectively). The white blood cell count, C-reactive protein and procalcitonin in the bacterial group were significantly higher than those in the MP group (14.7 (1.0-67.1)×10 9/L vs. 10.5 (2.5-32.2)×10 9/L, 122.5 (0.5-277.3) mg/L vs. 51.4 (0.5-200.0) g/L, 2.13 (0.05-100.00) μg/L vs. 0.24 (0.01-18.85) μg/L, Z=-3.719, -5.901 and -7.765, all P<0.01). Conclusions:The prevalence of pediatric NP in China shows an increasing trend during the past years. MP, SP and SA are the main pathogens of NP, and the most common clinical symptoms are fever and cough. The WBC count, C-reactive protein and procalcitonin in bacterial NP are significantly higher than those caused by MP.

Objective:To explore the neutral mechanism of influence of long-term exposure to high altitude hypoxia environment on the processing stage of conflict inhibition.Methods:Differences in the electroencephalogram during a 2-1 mapping Stroop task were compared between a high-altitude (HA) group ( n=20) who lived in a high-altitude area (3 650 m) for two years, and a low-altitude (LA) group ( n=20) who had never visited high-altitude areas. Results:The HA group had a higher pulse rate ((82.0±9.7)/min vs (74.0±11.3)/min) and lower oxygen saturation level ((90.7±2.8)% vs (97.4±1.1)%) compared with LA group.Behavior data showed that the reaction time (RT) in congruent (CO) condition was shorter than that in stimulus incogruent (SI) condition, and the RT in SI condition was shorter than that in RI condition ((707.91±70.33)ms vs (753.76±72.96)ms vs (803.88±73.21)ms); accuracy(ACC) rate in SI condition was higher than CO condition, and the ACC in CO condition was higher than RI condition((96.35±2.91)% vs (95.90±2.83)% vs (91.55±5.22)%). And the response conflict effect was larger than the stimulus conflict effect((49.76±33.90) vs (23.60±30.20)). The differences were all statistically significant (all P<0.01). In SI condition, the RT of HA group was longer than LA group((730.43±60.50)ms vs (777.09±78.23)ms) ( P<0.05). As for the EEG, the amplitude of P2((6.18±0.62)μV vs (3.21±0.62)μV) and N450((1.39±1.48)μV vs (5.91±1.48)μV) component of the HA group were higher those that of the LA group.The amplitude of N450 at P4 electrode site was larger than that at P3 electrode site in HA group((2.26±1.34)μV vs (0.62±1.43)μV). The differences were all statistically significant (all P<0.05). Conclusion:Long-term exposure to high altitude hypoxia environment affects the early stage of stimulus perception and task retrieval, as well as the conflict monitoring in later stage of stimulus and response conflict.The HA group need compensation of right parietal to complete the conflict monitoring of stimulus and response conflict.

Objective: To understand the molecular evolution characteristics of the nucleoprotein (N) genes and epidemiological feature of 118 rabies virus (RABV) strains isolated in Yunnan province, China from 2006 to 2015. Methods: The brain tissue samples from mad dogs, suspicious sick dogs, sick cow, and human brain tissue, saliva and CSF samples from rabies patients were collected in Yunnan province to detect the viral antigen by direct immunofluorescence assay (DFA). The viral RNA from positive samples was extracted. Coding region of N gene was amplified by RT-PCR and sequenced. The phylogenetic tree was constructed by Neighbor-Joining method of MEGA5.0 software. Results: The sequences of N genes of 91 RABV strains in Yunnan from 2012 to 2015 were obtained. With the sequences of N genes of 27 RABV strains in Yunnan from 2006 to 2011 and 29 RABV strains from Southeast Asian Countries, the phylogenetic analysis was performed. RABV strains in Yunnan were divided into clades YN-A (105 strains), YN-B (6 strains), YN-C (7 strains), which belonged to clades China-I, China-VI, China-II respectively. Clade YN-A was epidemic every year from 2006 to 2015, of them, 14 strains from 2006 to 2011 and 91 strains from 2012 to 2015 were distributed in 13 prefectures (cities) of Yunnan. Clades YN-B and YN-C were epidemic only from 2006 to 2010 and from 2008 to 2011 respectively. The regional distribution of clades YN-B and YN-C was limited. The strains of YN-A and YN-C were closely related to the strains of clades China-I and China-II from neighboring Sichuan, Guizhou, Guangxi and Hunan provinces. The strains of YN-B were closely related to the strains from Myanmar, Laos, Vietnam and Cambodia. Conclusions Three RABV clades with multiple transmission sources were identified in Yunnan. Clade YN-A was widely distributed in rabies endemic area in Yunnan from 2006 to 2015, and it has strong ability to spread as principal clade in Yunnan. Since 2012, clades YN-B and YN-C were not found again in Yunnan.

Objective To analyze the mechanism of interaction between cardiac and renal failure and observe the protective effects of Zhenwu decoction on cardio-renal syndrome rats. Methods The rats were divided into five groups: Sham group, MI (myocardial infarction) group, MI merger STNx group, MI intervention group, MI merger STNx intervention group. The first three groups were fed with saline (NS) for two weeks after surgery. The latter two groups of rats were fed with an equal volume of Zhenwu decoction. The cardiac function of rats in each group were evaluated , including the mass of heart , left ventricle and left kidney weights as a ratio of body weight, fractional shortening and left ventricular ejection fraction. And serum levels of indoxyl sulfate and serum creatinine were also achieved. Results MI merger STNx could further aggravate the heart function, kidney function and left ventricular remodeling in rats. Zhenwu decoction could obviously improve the heart function, kidney function and left ventricular remodeling in rats. Conclusions Based on the MI models, STNx can worsen heart function and kidney function. Zhenwu decoction can significantly improve heart function and kidney function.