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肝豆状核变性又称Wilson氏病,是一种常染色体隐性遗传性铜代谢疾病,我院1982年4月~1984年4月共收住6例,现报告如下: 一、临床表现:儿童患者多以肝损害较为突出,神经精神症状常在肝症状后半年至4年才出现。发病年龄愈小,不出现神经精神症状而只出现肝损害的可能性愈大,其临床表现较复杂,易误诊。(一)肝损本组6例均有不同程度的肝或/和脾肿大,其中4例曾有肝炎史,3例有肝硬化腹水,2例有家族史。由于本病初册出现肝病
Wilson’s disease, also known as Wilson’s disease, is an autosomal recessive inherited copper metabolic disease, our hospital from April 1982 to April 1984 were housed in 6 cases, are as follows: First, the clinical manifestations: Children with liver damage more prominent, neuropsychiatric symptoms often appear in the liver symptoms after six months to 4 years. The smaller the age of onset, neurological symptoms do not appear only the greater the possibility of liver damage, the clinical manifestations of more complex and easy to misdiagnosis. (A) liver damage The group of 6 patients had varying degrees of liver and / or splenomegaly, of which 4 had history of hepatitis, 3 cases of cirrhosis and 2 cases of family history. Because of this disease early liver disease