Clinical Features And Relevant Factors Of Cardiac And Renal Damage In Chinese Fabry Disease Patients

Aim:Fabry disease is a X-linked genetic lysosomal storage disease,which its manifestations are involved into many systems,which is early accumulation of skin,nervous and digestive systems,and late accumulation of large organs such as heart and kidney.It is related to the genders and ages.Nowadays,a lot of surveys are aimed to European and American populations,there is no large-scale study aimed for Chinese populations.The purpose of the article is to discuss the manifestations in different genders and ages,as well as the clinical characteristics and influence factors of heart and kidney damage.Methods:Collecting the clinical data of the patients,the patients are divided into several groups,women and men;juveniles,young people and mid-aged people.The manifestations were statistically analyzed.Groups were divided according to the presence of renal system damage,the presence of cardiovascular damage and the presence of cardiac and renal damage.Comparative analysis of male and female,different age Fabry disease clinical characteristics and heart and kidney damage when the clinical characteristics and influence factors.Results:The study has collected108 patients,including 82 males and 26 females,the youngest is 11 years old and the oldest is 68.The average age of the patients is 31.85±11.43.The age of diagnosis of Fabry disease is 37.48±12.09 in females,25.74±9.34 in males.The influence of neuropsychiatric pain,decreased attention,skin and ear manifestations,diarrhea,gastrointestinal dysfunction,hypertension,proteinuria and chronic kidney disease were significantly higher in men than in women,with statistically significant difference.The MSSI severity score is higher for men than for women[(20.00(13.38,28.63)vs 10.50(4.56,15.63)],which the difference was statistically significant.When grouped according to different ages,the incidence of no sweat/lean sweat in the juvenile group was higher,the tinnitus and proteinuria,chronic renal failure in the young people were more obvious;the incidence of cardiac hypertrophy and conduction block increase in mid-aged people,there are statistical difference.Compared with no renal damage group,renal damage group of depression,anxiety,decreased vision,angiokeratoma,hearing loss,tinnitus,nausea,vomiting,bowel dysfunction,myocardial hypertrophy,high blood pressure,breathing difficulties,the higher the proportion of the chronic bronchitis(P<0.05),and the severity of disease more severe(P<0.05).Multivariate logistic regression analysis showed that the independent risk factors for renal damage were hearing loss(OR=13.424,95%CI:4.115-43.799,P<0.001),tinnitus(OR=5.001,95%CI:1.672-14.957,P<0.0.001),hypertension(OR=8.736,95%CI:1.533-49.797,P<0.05).In terms of cardiovascular system,the age and diagnosis age of the group with cardiovascular damage were older and the course of disease was longer(P<0.05).There was no significant difference in age of onset between groups.Decreased attention,depression and anxiety,corneal vortex opacity,retinal vascular tortuosity,conjunctival vascular tortuosity,posterior capsular opacity,tinnitus,hearing loss,nausea and vomiting,chronic renal insufficiency,and chronic bronchitis had a higher proportion in the group with cardiovascular damage,with statistical difference(P<0.05).Multivariate logistic regression analysis showed that the independent risk factors for cardiovascular damage were depression and anxiety(OR=8.720,95%CI:2.063-36.857,P<0.05),and the age of diagnosis(OR=1.116,95%CI:1.034-1.205,P<0.05).In terms of cardiorenal damage,compared with the group with no cardiorenal damage and the group with only cardiovascular system or kidney damage,the age and diagnosis age of the group with cardiorenal damage were older and the course of disease was longer(P<0.05).There was no significant difference in age of onset among the three groups.At the same time,there was a higher proportion of depression and anxiety,tinnitus,hearing loss,sensorineural hearing loss,nausea and vomiting,dyspnea and chronic bronchitis in the group with heart and kidney damage(P<0.05),and the disease severity was more serious(P<0.05).Independent risk factors for simultaneous cardiac and renal impairment included the age of diagnosis(OR=1.108,95%CI:1.037-1.185,P<0.05),and tinnitus(OR=6.617,95%CI:1.584-27.632,P<0.05).Conclusion:The age of diagnosis and course of the disease is younger in males than females.Many clinical manifestations of Fabry disease are more obvious and more serious in males.The incidence of Fabry disease symptoms is different in different age groups.Non-sweat/sweat are more common in juveniles,symptoms of ear and kidney are more common in young people and heart damage is common in middle age.Independent influence factors for renal impairment include tinnitus,hypertension,and hearing loss.The independent influence factors of cardiovascular damage were depression and anxiety,and the age of diagnosis.At the same time,the independent relevant factors of heart and kidney damage were tinnitus and the age of diagnosis.Incorporating appropriate relevant factors into the prediction model can improve the prediction efficiency of Fabry disease.The results of this study provide important scientific basis for the clinical diagnosis,follow-up observation and disease management of Fabry's disease.