| Hepatolenticular degeneration,also known as Wilson’s disease(WD),is an autosomal recessive inherited disorder of copper metabolism that requires lifelong medication.Its main clinical manifestations include acute and chronic liver damage,extrapyramidal symptoms,chronic kidney damage,and other systemic multi-system injuries.These symptoms have a severe impact on the patient’s quality of life,work ability,and lifespan,imposing a heavy burden on both the patient and their family.In clinical practice,many WD patients suffer from malnutrition and movement disorders,issues that not only affect the treatment outcome but may also lead to a worsening of the condition and poor prognosis.Currently,both domestic and international guidelines for the prevention and treatment of WD lack research,theoretical methods,and management models concerning the coordinated intervention of exercise and nutrition in WD.This makes the implementation and supervision of related intervention plans difficult.The problem mainly stems from three issues:first,the lack of systematic nutritional and physical ability assessment in WD patients;second,insufficient understanding of the therapeutic mechanisms of exercise and nutritional intervention in WD;and third,the absence of precedents for exercise and nutritional intervention in WD patients.To address these issues,this study assessed the nutritional status of WD patients.and explored the impact and mechanisms of coordinated exercise and nutritional intervention on the motor function of WD model TX mice.Additionally,the study investigated the effects of lifestyle and simple exercise and nutritional intervention education on WD patients.The main content includes:1.Evaluation of nutritional status and exercise capacity of Chinese WD patients based on a large sampleAfter assessing the body composition,nutritional scoring,anthropometric data,physical ability,and serum biochemical indicators of 128 WD patients,we found that 43.41%of WD patients(56/129)have abnormal nutritional status.Among them,the incidence of high nutritional risk and overweight patients was 17.83%(23/129)and 25.58%(33/129),respectively.Compared to the healthy population,WD patients have lower total muscle and segmented muscle indicators,and higher overall fat and segmented body fat.The waist-hip ratio of cerebral-type WD patients(0.96±0.83)is higher than that of hepatic-type WD patients(0.86±0.08),and exceeds the recommended value for the same ethnicity(less than 0.85).Compared to WD patients with normal nutrition and overweight,those with malnutrition show significant characteristics in body composition and blood indicators.Among males,6 have a grip strength of less than 28kg,20 have a walking speed of less than lm/s,and 27 have an appendicular skeletal muscle mass index(ASM)of less than 7.0kg/m^2;among females,4 have a grip strength of less than 18kg,12 have a walking speed of less than lm/s,and 19 have an ASM of less than 5.7kg/m^2.The grip strength and walking speed of cerebral-type WD patients are significantly lower than those of hepatic-type WD patients.In the WD patient population,the incidence rates of reduced skeletal muscle content,reduced muscle strength,decreased physical mobility,and sarcopenia are 35.66%,7.75%,24.81%,and 13.18%,respectively.Therefore,this study revealed that 17.83%of WD patients have nutritional disorders,and 24.81%have movement disorders.These nutritional disorders are reflected in abnormalities in body fat percentage,muscle mass,large-to-small platelet ratio,monoamne oxidase ratio,creatinine,amylase ratio,waist-to-hip ratio,and basal metabolic rate.Movement disorders are mainly manifested as slower walking speed and weaker grip strength.High nutritional risk is more common among hepatic and cerebral-type WD patients.Compared to the healthy population,WD patients generally have higher body fat percentage and waist-to-hip ratio,suggesting that cerebral-type WD patients may be at higher risk for cardiovascular diseases.Due to the reduced muscle mass compared to the normal population,close attention should be paid to signs of sarcopenia in WD patients.2.Effect and mechanism of exercise&nutrition intervention on motor function of WD model TX miceWD model toxic milk(TX)mice were used as the object and divided them into a model group(no special intervention),an exercise group(8 weeks of treadmill training),and an exercise+nutrition group(8 weeks of treadmill training+albumin gavage).We studied the exercise intervention pattern of TX mice,the effects of exercise and nutrition intervention on their exercise function,brain tissue oxidative stress levels,and the expression changes of clusterin(CLU),Tensin-4(Tns4),and complement C3related proteins.The results showed that compared with the start of the exercise plan,the exercise group mice lost more weight than the exercise+nutrition group mice.The exercise&nutrition group mice had fewer exercise refusal times in the later stage of the exercise plan than the exercise group mice.The Rotarod experiment showed that compared with the TX group mice,the average rotation time and distance of the exercise group mice were significantly increased,but there was no statistical significance.The average rotation time and distance of the exercise+nutrition group mice were significantly increased.Compared with the exercise group mice,the average rotation time and distance of the exercise+nutrition group mice were slightly increased,but there was no statistical significance.The enzyme-linked immunosorbent assay(Elisa)results showed that compared with the TX group mice,the expression levels of brain tissue Nitrotyrosine and 8-hydroxyguanosine proteins were significantly downregulated in the exercise group mice and exercise+nutrition group mice.The Western blot(WB)experiment results showed that compared with the TX group,the expression levels of CLU and Tns4 in the exercise group and exercise+ nutrition group were significantly higher,and the expression level of C3 was significantly lower.Therefore,exercise and nutritional intervention can enhance the physical ability of WD model TX mice.The possible mechanism is through enhancing the expression of CLU and Tns4 proteins,exerting anti-neuroinflammatory and neuronal repair effects.Nutritional intervention can also assist exercise intervention,improving the mice’s compliance with the training plan,and mitigating the impact of weight loss caused by the exercise regimen.3.Lifestyle survey and exercise&nutrition intervention study among WD patientsNutritional&sports intervention may be an important aspect of long-term management for patients with WD.Therefore,based on lifestyle education,we conducted a nutritional intervention for the first group of WD patients who underwent testing.Prior to this,we conducted a web-based survey on the lifestyle and medical burden of WD patients.The results showed that 406 online questionnaires were collected from 26 provinces,autonomous regions,and municipalities across the country.Among these patients,18.97%of them had a smoking habit,while 9.36%had an alcohol consumption habit.Besides,80.3%of the respondents had a sedentary lifestyle,and more than half of the patients(52.96%)reported that they had regular exercise habits.However,most of them(77.1%)exercised no more than three times a week.Additionally,13.79%of the respondents believed that they had malnutrition,while 32.76%were uncertain about their nutritional status.More than half of WD patients spent more than 50%of their total household income on the treatment of the disease every year.In the intervention experiment,one patient out of 129 died unexpectedly,interrupting the intervention experiment.Among the remaining 128 patients,46 expressed a willingness to accept sports nutrition intervention,accounting for 36%of the total.After a one-year follow-up,we found that 15 of them(32%of those willing to undergo intervention)were able to follow our sports nutrition intervention education and make appropriate adjustments to their exercise and diet.Compared with patients who did not follow intervention education or refused to participate in follow-up,these 15 patients had relief in liver function indicators,such as transaminase and bile acid,upon returning to the hospital for treatment.We believe that there are still unhealthy lifestyles,such as smoking and drinking,among WD patients in China.Although most WD patients have exercise habits,most of them require professional guidance.Thirty percent of WD patients are unaware of their own nutritional status.Exercise&nutrition intervention can provide protective effects on the liver function of WD patients in the short term.Therefore,regular assessment,education,and supervision of sports nutrition should be carried out for WD patients in chronic disease management.In summary,this study has initially established a comprehensive system for sports nutrition assessment,centered around body composition analysis,scoring scales,anthropometry,serum biochemistry,and exercise function evaluation.The study has identified general patterns of sports nutrition disorders in patients with WD(Wilson’s Disease),preliminarily clarified the therapeutic.effects of sports nutrition on WD,and consequently developed a methodological system for assessment,intervention,and reassessment.Furthermore,the study has conducted initial research on the mechanisms by which sports nutrition interventions affect CLU-related proteins.This provides methodological and theoretical support for scientific research and clinical practice in the prevention and treatment of chronic diseases and WD. |
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