CAJ | 학술논문

探讨不同亚型Wilson’s病（WD）的临床特点及肝型患者预后转归。方法收集256例WD患者的临床资料并随访，根据临床表现将患者分为不同的临床亚型，进一步分析比较肝型、脑型和混合型WD患者的临床特点差异和随访追踪肝型WD患者的预后。结果在256例WD患者中，以混合型（152例，59.4%）和肝型（74例，28.9%）患者常见，而脑型（27例，10.5%）和其他亚型（3例，1.2%）较少；肝型WD患者失代偿期肝硬化比例（78.4%）高于混合型患者（22.0%，P<0.001）；肝型WD患者肝脏血清生化学指标（转氨酶、ALP、GGT、胆红素以及球蛋白水平）高于混合型WD患者（P<0.05）；肝型WD患者血清铜[（1.04±1.50） mg/L]水平明显低于脑型WD患者[（2.96±2.88） mg/L]和混合型WD患者[（2.34±2.68） mg/L，P<0.001]，但两者铜蓝蛋白和尿铜水平无统计学差异（P>0.05）；肝型WD患者K-F环检出率（64.9%）低于脑型WD患者（92.6%）和混合型WD患者（90.1%，P<0.05）；经Logistic回归分析显示角膜K-F的有无与年龄（OR=0.922，P=0.014）、血清铜蓝蛋白（OR=35902.1，P=0.015）相关；平均随访31例肝型WD患者（8.3±5.8）年，3例（9.7%）进展为混合型WD患者。结论 WD以混合型和肝型最多见，肝型患者肝脏损害比混合型更为严重，提示肝脏是WD最主要的靶器官。
탐토불동아형Wilson’s병（WD）적림상특점급간형환자예후전귀。방법수집256례WD환자적림상자료병수방，근거림상표현장환자분위불동적림상아형，진일보분석비교간형、뇌형화혼합형WD환자적림상특점차이화수방추종간형WD환자적예후。결과재256례WD환자중，이혼합형（152례，59.4%）화간형（74례，28.9%）환자상견，이뇌형（27례，10.5%）화기타아형（3례，1.2%）교소；간형WD환자실대상기간경화비례（78.4%）고우혼합형환자（22.0%，P<0.001）；간형WD환자간장혈청생화학지표（전안매、ALP、GGT、담홍소이급구단백수평）고우혼합형WD환자（P<0.05）；간형WD환자혈청동[（1.04±1.50） mg/L]수평명현저우뇌형WD환자[（2.96±2.88） mg/L]화혼합형WD환자[（2.34±2.68） mg/L，P<0.001]，단량자동람단백화뇨동수평무통계학차이（P>0.05）；간형WD환자K-F배검출솔（64.9%）저우뇌형WD환자（92.6%）화혼합형WD환자（90.1%，P<0.05）；경Logistic회귀분석현시각막K-F적유무여년령（OR=0.922，P=0.014）、혈청동람단백（OR=35902.1，P=0.015）상관；평균수방31례간형WD환자（8.3±5.8）년，3례（9.7%）진전위혼합형WD환자。결론 WD이혼합형화간형최다견，간형환자간장손해비혼합형경위엄중，제시간장시WD최주요적파기관。
To investigate the clinical features of patients with Wilson’s disease (WD) in differ-ent clinical classification. Methods Clinical data of 256 inpatients with WD were collected retrospectively. The patients were then divided into liver type,cerebral type and mixed type according to their major clinical presenta-tions and the differences in clinical features of patients in each group were further analyzed. Results Among 256 patients with WD,patients with mixed type (152 cases,59.4%) and liver type(74cases,28.9%)were the com-mon type as compared to cerebral type (27cases,10.5%) or other subtypes(3 cases,1.2%). The ratio of patients with decompensated cirrhosis and the levels of various liver serum biochemical parameters (ALT,AST,ALP,GGT, BIL and GLB) in patients with liver type (78.4%) were higher than in patient with mixed type (22.0%,P﹤0.05). Serum copper in patients with liver type [(1.04±1.50)mg/L] were lower than patients with cerebra type [(2.96±2.88) mg/L] and mixed type [(2.34±2.68)mg/L,P﹤0.001],but no difference in serum ceruloplasmin level and 24-h uri-nary copper excretion between the two groups (P﹥0.05). The positive ratio of Kayser-Fleischer rings in patients with liver type (64.9%) was lower than in patients with cerebral type (92.6%) and mixed type (90.1%,P﹤0.05). A logistic regression analysis showed that age (OR 0.922,P=0.014) and serum ceruloplasmin level (OR=35902.1, P=0.015) were related to the presence of Kayser-Fleischer rings. Only 3(9.7%) out of 31 patients with WD with liver type developed into mixed type during the average follow-up of (8.3±5.8) years. Conclusions Mixed and liver type are more common in patients with WD,and liver dysfunction is more severe in patients with liver type than in mixed type,suggesting that the liver is the most common target in WD.