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  • Title: [Analysis of 128 Wilson's disease].
    Author: Gao WS, Liu YW, Chen J.
    Journal: Zhongguo Yi Xue Ke Xue Yuan Xue Bao; 2001 Oct; 23(5):506-8. PubMed ID: 12905873.
    Abstract:
    OBJECTIVE: To investigate the clinical feature and misdiagnosis of 128 patients with Wilson's disease and propose the points of diagnosis for this disease. METHOD: 128 patients with Wilson's disease from Jan. 1983-Apr. 2000 admitted to our hospital were reviewed. Their clinical feature and diagnosis condition, such as the early onset symptoms, misdiagnosis and hypersplenism were studied. RESULTS: 1. The first onset symptoms: 64 were liver dysfunction and 52 were manifestations of CNS (P > 0.05). 2. The average age of patients with liver dysfunction was significantly younger (13 +/- 3.5 years) than that of CNS manifestations (23 +/- 8.3 years, P < 0.05). 3. The positive rate of cornea Kayser-Fleischer ring was 96.8%; the value of copper oxidase absorbency was 0.051 +/- 0.024 (0.01-0.16) and its positive rate was 93%. 4. The overall misdiagnosis rate was 42.9% (55/128) and the predominant mis-diagnostic diseases were hepatitis A and chronic hepatitis. CONCLUSION: The clinical manifestations are diverse, which make the misdiagnosis rate very high. The age of liver dysfunction patients is significantly younger than that of the CNS manifestations patients. Cornea Kayser-Fleischer ring and copper oxidase absorbency are of high positive rate, as it can be carried out conveniently in clinics we recommends to use this diagnostic measure for Wilson's disease.
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