Objective: Amyotrophic lateral sclerosis(ALS)is a chronic fatal disease.At present,there is no single diagnostic experiment or biomarker.Different levels of creatine kinase were observed in some patients with amyotrophic lateral sclerosis.Objective to investigate the relationship between serum creatine kinase(CK)level and clinical features and survival prognosis in patients with amyotrophic lateral sclerosis(ALS).Methods: this study selected 1206 ALS patients from PRO-ACT database,including complete creatine kinase level and survival time data,creatine kinase normal range male(38-174u/L),female(96-140u/L).The relationship between CK level and clinical features(sex,age,course of disease,history of Riluzole administration,site of onset,BMI,FVC,ALSFRS score)was analyzed by rank correlation and linear correlation.Binary logistic regression was used to study the independent influencing factors of CK elevation.Ordered logistic regression was used to study the relationship between CK level and ALSFRS progression rate.Kaplan-Meier and Cox proportional hazard models were used to investigate whether CK level was independently related to the survival and prognosis of ALS.Results: The baseline CK level of 656 subjects was higher than the normal upper limit,and the serum CK level of male patients was significantly higher than that of female patients(p < 0.001).The level of CK in patients with non-medulla oblongata onset was also significantly higher than that in patients with medulla oblongata onset(p < 0.001).The level of CK in patients with upper limb onset was higher than that in patients with medulla oblongata onset(p < 0.001).The level of CK in patients with onset of lower extremities.There was no significant difference in CK between patients with medulla oblongata onset and patients with medulla oblongata onset(p < 0.180).The increase of CK level was related to sex [OR=4.125(2.633-6.461,p< 0.001),riluzole administration history [OR=0.629(0.430-0.916),p=0.011],serum albumin [OR=1.191(1.102-1.287),p <0.001],ALSFRS upper limb score decreased rate [OR=0.210(0.078 ±0.569),p < 0.002],lower limb score decreased rate [OR=0.340(0.119 ±0.968),p<0.043] independently correlated.There was a correlation between Log CK and ALSFRS progress rate [OR=0.212(0.123 ±0.368),p< 0.001],Log CK,the higher the ALSFRS progress rate was,the smaller the ALSFRS progress rate was.The survival curve of Kaplan-Meier showed that the survival time of the elevated CK group was higher than that of the normal CK group(p < 0.008),and the survival time of the elevated CK group was also higher than that of the normal CK group(p=0.008).After adjusting for other variables,Log CK was significantly correlated with overall survival,and the risk of death decreased by 29.6% for each additional 1U [adjusted HR=0.704(0.530 to 0.935),p< 0.015].Conclusion:(1)the serum CK concentration in ALS patients can be increased,but it is less than 1000 U/L.the level of CK is relatively high in limb onset and male patients.obese and overweight ALS patients may have higher CK levels.However,the difference was not obvious in multivariate regression.(2)the level of CK in ALS patients was independently correlated with serum albumin,sex,Riluzole administration and the decrease rate of upper and lower limb score of ALSFRS.The protective mechanism supporting the increase of CK level is hypothetical,but there may be many mechanisms for the increase of CK level.(3)Log CK is an independent predictor of survival in ALS patients,and may have protective effect on upper and lower limb function in ALS patients. |