| Objective: The aim of the study was to investigate the age of onset,clinical classification,clinical manifestation,major related inspections and treatment of Wilson's disease(WD).Methods: Collecting clinical data of 57 cases of WD in hospitalized patients in the First Hospital of Lanzhou University from January 2008 to December 2017.we collected the general information(age,gender,family history),Clinical features,laboratory tests,imaging examination,liver pathology examination and treatment outcome.statistical methods to analyze the age of onset,clinical features,the significance of the test results and treatment of WD patients.results:1.General Information: 57 cases of WD patients aged ranged from 0.4 to 68 years,the patients accounted for 50.48 percent from 5 to 35 years,average age of 26.76 ±19.54,the male has a higher incidence,38(66.67%)males,19(33.33%)females.Divided into two groups according to age: group I: 24(42.11%)patients was less than 18 years,Group II: 33 patients(57.89%)was more than 18 years old.The positive rate of family history was 5.2%.2.Clinical grouping: in the 57 cases of WD,the hepatic type were 39 cases(68.42 %),brain type were 8 cases(14.04%),mixed type were 8 cases(14.04%)and other types were 2 cases(3.50 %).Among them,the first symptom of liver disease was 42 cases(73.68),neuropathy was 11 cases(19.30),and other was 4 cases(7.02),respectively,showing joint pain,urine foam more,edema.All age groups were mainly liver type,with no statistical significance in different age groups(P> 0.05).3.Clinical manifestations: Clinical manifestations of WD patients had not specific.The study of more common symptoms are abdominal pain(28.1%),jaundice(40.4%),extrapyramidal symptoms(21.1%),and systemic symptoms of fatigue(31.6%),anorexia(19.3%),other rare Such as asymptomatic transaminases(19.3%),gastrointestinal bleeding(7%),psychosis(8.8%)and other joint pain,edema,increased urine foam(14%).no significant difference was noted in clinical manifestations between different the age groups.4.Laboratory tests: 57 patients of the study performed ceruloplasmin detection,54 cases below the normal lower limit,the positive rate was 94.7%;28 cases underwent serum copper detection,25 cases with different degree of reduction(89.29%);13 cases underwent 24 hours urinary copper detection,10 cases with varying degrees elevated(76.92%),by comparing the various clinical types of patients with no statistically significant differences in serum copper and ceruloplasmin(P > 0.05).there is statistically significant(P <0.05)between difference clinical types in terms of NEUT,AST,ALT,TBIL,DBIL,GGT,TG.There was no significant difference in immunological examination between different clinical types,but the patients in this study had different levels increased of IgG and IgM,decreased of C3 and C4;Calculate the Child-pugh score of liver function of the patients of liver type: grade A level 20 cases(51.3%),grade B level 13 cases(33.3%),grade C level 6 cases(15.4%).with the deterioration of liver function,King score higher,significant difference was noted in King score between grade A and C.With the deterioration of liver function,C3 decreased,this was statistically significant between different Child-pugh score of liver function.5.Imaging examination: 42 cases of the study performed K-F ring examination,of which 26 cases were positive,the positive rate was 61.9%,no significant difference between the various clinical types.40 cases of the study performed abdominal ultrasound examination,of which 36 patients(90%)patients with varying degrees of diffuse liver disease,hepatosplenomegaly,ascites,intrahepatic solid nodules.11 patients underwent abdominal CT examination,of which 10 cases(91%)of patients with cirrhosis,ascites,hepatosplenomegaly,liver nodules,esophageal varices and so on.7 patients underwent Abdominal MRI.,Six patients(85.7%)had diffuse liver anomalies,ascites,hepatosplenomegaly,and diffuse decrease of hepatic parenchymal T2 signals.9 patients underwent head CT examination,abnormal in 3 cases(33.3%).7 patients performed head MRI examination,6 cases were abnormal(85.7%).8 patients in this study underwent pathological examination,with varying degrees of hepatocyte edema,balloon-like changes,steatosis,portal lymphocytic infiltration,fibrous tissue hyperplasia,false lobules,consistent with the performance of WD lesions.6.Treatment and prognosis: All patients were given a low-copper diet after diagnosis.Among them,51 patients(89.5%)were improved after treated with penicillamine,zinc and liver-preserving drugs.The treatment effect was poor in 4 patients,order to Gastrointestinal bleeding,hepatic encephalopathy and other give up treatment,2 patients give up treatment due to economic problems,be discharged automatically.conclusion:1.The age of onset of Wilson's disease is all,but mainly is adolescents,the male has a higher incidence.2.the main clinical types and first symptoms were liver type,followed by brain type.so adolescents with unknown causes of liver dysfunction or nervous system symptoms should be highly alert to WD.3.Ceruloplasmin and serum copper had high positive rate can be used as the preferred laboratory indicators,Cranial CT and MRI had specific.4.Wilson's disease can invade the liver,nervous system,blood system,kidney,cornea,skeletal system and other systems.5.WD had good prognosis if early diagnosis,early treatment. |
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