| 肝豆状核变性八例临床分析 |
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| 引用本文： | 徐建星,邓崇德.肝豆状核变性八例临床分析[J].南京医科大学学报,1986(1). |
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| 作者姓名： | 徐建星 邓崇德 |
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| 作者单位： | 南京医学院第二附属医院儿科
(徐建星)，南京医学院第一附属医院儿科(邓崇德) |
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| 摘 要： | 8例肝豆状核变性中,7例有角膜K-F环,血清铜氧化酶值均低,多数有肝病表现,约半数有神经系统及精神症状。
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| 关 键 词： | 肝窦状核变性 血清铜蓝蛋白 K-F环 |
WILSON'S DISEASE: A RETROSPP TIVE STUDY OF EIGHT CASES |
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| Xu Jianxing,Deng Chongde.WILSON'S DISEASE: A RETROSPP TIVE STUDY OF EIGHT CASES[J].Acta Universitatis Medicinalis Nanjing,1986(1). |
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| Authors: | Xu Jianxing Deng Chongde |
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| Institution: | Xu Jianxing;Deng Chongde |
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| Abstract: | The ages of the eight patients whettheir symptoms developed ranged from 1to 12 years. Delay in diagnosis may stemfrom the varied mode of presentation ofthis disease. Six of the patients had sigor symptoms of liver disease at the tiof diagnosis. Four had neurologic inv(?)vement. The hepatic involvement ofteprecedes the neurologic manifestationby many years, and therefore caremust be taken before the establishmentof diagnosis in any child with signsand symptoms of liver disease. Kayser-Fleischer rings are considered pathog-nomonic for Wilson' disease. However,their absence obviously does not excluoethis diagnosis. A low serum ceruloplasmis confirmative of the diagnosis, but low serumceruloplasmin levels have been reportedin protein-losing enteropathy, protein-calorie malnutrition, certain malabsorption syndromes, nephrotic syndromesand acute necrosis. |
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| Keywords: | Hepatolenticular degene ration ceruloplasmin Kayler-Fleischer rings |
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