VALUE OF URINARY COPPER EXCRETION AFTER PENICILLAMINE CHALLENGE IN THE DIAGNOSIS OF WILSONS-DISEASE
VALUE OF URINARY COPPER EXCRETION AFTER PENICILLAMINE CHALLENGE IN THE DIAGNOSIS OF WILSONS-DISEASE
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DOI：
10.1002/hep.1840150410
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发表时间：
1992-04-01
期刊：
影响因子：
13.5
通讯作者：
MIELIVERGANI, G
中科院分区：
文献类型：
--
作者：
DACOSTA, CM;BALDWIN, D;MIELIVERGANI, G
为了探讨青霉胺激发后 24 小时尿铜排泄测试在诊断 Wilson 病中的诊断价值，我们对 75 名因各种肝脏问题而转诊且已接受铜代谢参数调查的连续儿童进行了回顾性分析。 17 人患有威尔逊病，22 人患有自身免疫性慢性活动性肝炎，6 人患有原发性硬化性胆管炎，12 人患有各种病因的慢性肝病，4 人患有隐源性急性肝衰竭，6 人患有急性肝炎，8 人患有各种肝脏组织学外观正常的疾病。 与所有其他组相比，威尔逊病患者的血清铜蓝蛋白和总铜水平显着较低，但三名威尔逊病儿童的铜蓝蛋白水平正常，七名儿童的总铜水平正常。 威尔逊病患者与其他组之间游离血清铜水平和肝脏铜含量没有显着差异。 与其他患者相比，威尔逊病患者的基线 24 小时尿铜排泄量显着较高，但 6 名威尔逊病儿童的水平略高于正常上限，与 3 名肝衰竭儿童、2 名急性肝炎儿童、2 名自身免疫性慢性活动性肝炎儿童和 3 名原发性硬化性胆管炎儿童获得的值重叠。 青霉胺激发后 24 小时尿铜排泄被证明是最准确的单一诊断测试； 17 名威尔逊氏病患者中，有 15 名患者体内的水平超过 25-mu-mol/24 小时，但在 58 名患有其他疾病的患者中，只有 1 名患有肝功能衰竭。 青霉胺激发试验对于诊断威尔逊氏病非常有价值，特别是对于没有凯撒-弗莱舍环的儿童。
To investigate the diagnostic value of 24-hr urinary copper excretion testing after penicillamine challenge in the diagnosis of Wilson's disease, 75 consecutive children referred for a variety of liver problems and in whom parameters of copper metabolism had been investigated were analyzed retrospectively. Seventeen had Wilson's disease, 22 had autoimmune chronic active hepatitis, 6 had primary sclerosing cholangitis, 12 had chronic liver disease of various etiologies, 4 had cryptogenic acute liver failure, 6 had acute hepatitic illnesses and 8 had a variety of disorders featuring normal liver histological appearance. Serum ceruloplasmin and total copper levels were significantly lower in Wilson's disease patients compared with all other groups, but three children with Wilson's disease had normal ceruloplasmin levels and seven had normal total copper levels. No significant difference was found for free serum copper levels and liver copper content between Wilson's disease patients and the other groups. Baseline 24-hr urinary copper excretion was significantly higher in Wilson's disease patients compared with that of the other patients, but six children with Wilson's disease had levels just above the upper limit of normal, overlapping with values obtained in three children with liver failure, two with acute hepatitis, two with autoimmune chronic active hepatitis and three with primary sclerosing cholangitis. The 24-hr urinary copper excretion after penicillamine challenge proved the most accurate single diagnostic test; levels more than 25-mu-mol/24 hr were present in 15 of 17 patients with Wilson's disease, but in only 1 child with liver failure of the 58 with other disorders. The penicillamine challenge is a valuable aid in the diagnosis of Wilson's disease, particularly in children with no Kaiser-Fleischer rings.